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123 KNP

*Date¡§20200111
*Manager¡§Takahiro Fukuda

1ÆÃħŪ²èÁü½ê¸«¤òÄ褷¡¤Ç¾À¸¸¡¤Ë¤Æ·ì´É±ê¤¬¼¨º¶¤µ¤ì¤¿69ºÐÃËÀ­
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122 KNP

*Date¡§20190824
*Manager¡§Masashi Takanashi

1Åý¹ç¼ºÄ´¾É¤Î·Ð²áÃæ¤Ë¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¤¬½Ð¸½¤·¤¿ 74 ºÐ½÷À­
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121 KNP

*Date¡§20190309
*Manager¡§Kinya Ishikawa

1¤Æ¤ó¤«¤ó¤ò¹çÊ»¤·¤¿ 32 ºÐ½÷À­¤Îº¸Â¦Æ¬ÍÕ¼ðáç
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120 KNP

*Date¡§20181208
*Manager¡§Noriyuki Shibata

1An autopsy case of MAPT intron 10 mutation.
 1 Dementia Research Project, Tokyo Metropolitan Institute of Medical Science, 2 The Brain Bank for Aging Research, Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, 3 Department of Psychiatry, Shimofusa Psychiatric Medical Center, 4 Department of Molecular Genetics¡¦Department of Bioinformatics, Bioresource Science Branch, Center for Bioresources, Brain Research Institute, Niigata University, 5 Yokohama Brain and Spine Center.¡¤
 Ryohei Watanabe1, Ito Kawakami1,2, Mitsumoto Onaya3, Takeshi Ikeuchi4¡¢Shigeo Murayama2¡¢Haruhiko Akiyama1,5¡¢Masato Hasegawa1.
 
2ÂçǾ¿·Èé¼Á¤Ë¥ê¥ó»À²½¥¿¥¦ÃÁÇò¤ÎÃßÀѤ¬ÌÜΩ¤Ã¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î°ì˶¸¡Îã
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119 KNP

*Date¡§20180728
*Manager¡§Taku Homma

1ɸËܶ¡Í÷
 
 
 
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 ÏÂÀôÍ£¿®1)¡¢ºä²¼ÂÙ¹À2)¡¢»³¾å¡¡·½1)¡¢µÜËÜμ²ð1)¡¢Ìî»û͵Ƿ1)¡¢´¤°æ½Ó¹§1)¡¢Â¼»³ÈËͺ2)¡¢³á¡¡Î¶Ñ»1)
 
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 1) °Â˼ÃÏ°è°åÎÅ¥»¥ó¥¿¡¼¡¡Áí¹ç¿ÇÎŲʡ¤2) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÉÂÍý¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤3) °Â˼ÃÏ°è°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê
 Íî¹çºÌ»Ò1¡¢¾¾¸¶Ãι¯2¡¢ÌÚ¼Éð»Ê1¡¢À¾ÌîÍÎ1¡¢Ç½½ÅÊâ3¡¢Ê¿ÅĽ¨¼¤3¡¢Â¼»³ÈËͺ2
 
4Èæ³ÓŪŵ·¿Åª¤Ê·Ð²á¤ò¤¿¤É¤ê¡¢ÉÂÍý³ØŪ¤Ë³¤ÇÏ˵²ó¤Î³¤ÌʾõÊѲ½¤òǧ¤á¤¿¥Ñ¡¼¥­¥ó¥½¥óɤΰìÎã
 1)ÆüËÜÂç³Ø°å³ØÉô Æâ²Ê³Ø·Ï¿À·ÐÆâ²Ê³ØʬÌ2)ÆüËÜÂç³Ø°å³ØÉô ÉÂÂÖÉÂÍý³Ø·Ï¿ÍÂÎÉÂÍý³ØʬÌ
 ¹¾¶¶¡¡Åí»Ò1)¡¢½©ËÜ¡¡¹âµÁ1)¡¢¸¶¡¡À¿1) ¡¢¿¹ÅÄ¡¡¾¼É§1) ¡¢ÃæÅè¡¡½¨¿Í1) ¡¢ÄÔ¼¡¡Î´²ð2) ¡¢ËÜ´Ö¡¡Âö2) ¡¢µµ°æ¡¡Áï1) ¡¤
 
5Ependymoma, RELA fusion-positive: Pathological diagnosis
 Departments of Pathology¡¢Saitama Medical University, Iruma, Japan.
 Atsushi Sasaki
 
6ÆÃħŪ¤Ê¥¢¥ß¥í¥¤¥É¾®Âν¸äȤ¬Çò¼Á¤Ëǧ¤á¤é¤ì¤¿¿·À¸»ù²¾»à¸å°ä¾É¤Î1Îã
 1) ÅìµþÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤2) ÅìµþÅÔΩ¿À·Ðɱ¡ÉÂÍý¡Ê¿À·ÐÉÂÍý¡Ë¡¤3) ÆüËÜÂç³Ø°å³ØÉôÉÂÂÖÉÂÍý³Ø·Ï¿ÍÂÎÉÂÍý³ØʬÌ4) ½ÊÆÁÂç³Ø´Ç¸î±ÉÍܳØÉô´Ç¸î³Ø²Ê¡¤
 Ë¾·îÍÕ»Ò1)3)¡¢ÃÝÆâÀéÀç1)¡¢ËÜ´Ö¡¡Âö2)3)¡¢ÎÓ¡¡²íÀ²4)¡¢¾®¿¹Î´»Ê2)
 
766ºÐ¤Çȯ¾É¤··Ð²á17ǯ¤Î°ÕÌ£À­Ç§Ãξɡ¢83ºÐÃËÀ­Ë¶¸¡Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÉÂÍý¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯, 2) ¤«¤ï¤·¤Þ¿À·ÐÆâ²Ê¥¯¥ê¥Ë¥Ã¥¯
 »³ºê ´´Âç1), ÀîÅè ǵΤ»Ò2), ÀçÀРϣʿ1), ¼»³ ÈËͺ1)
 
8¿ºÌ¤Ê·ÁÂÖ¤ò¼¨¤¹AT8ÍÛÀ­astrocyte¤Î½Ð¸½¤òÂçǾ¤Ë¸«¤ë¿·ÏÅý°à½Ì¾É¤Î°ìÎã
 1) ÅìµþÅÔΩ¿À·Ðɱ¡ ÉÂÍý (¿À·ÐÉÂÍý)¡¤2) ÅìµþÅÔΩ¿À·Ðɱ¡ Ǿ¿À·ÐÆâ²Ê¡¤3) ÆüËÜÂç³Ø°å³ØÉôÉÂÂÖÉÂÍý³Ø·Ï¿ÍÂÎÉÂÍý³ØʬÌ
 ËÜ´Ö¡¡Âö1,3), Èôß·¿¸²ð2), ¾®¿¹Î´»Ê1), °ëùõ ±Ñ¼£2)
 

118 KNP

*Date¡§20180317
*Manager¡§Masashi Takanashi

1¼¯»ùÅ縩°ìÃÏ°è¤Ë¤ª¤±¤ë¥Ñ¡¼¥­¥ó¥½¥óÉ¿ȯ²È·Ï¤Î°ì˶¸¡Îã
 1)½çŷƲÂç³Ø°å³ØÉôÉÕ°½çŷƲ±Ûëɱ¡¿À·ÐÆâ²Ê¡¤2)½çŷƲÂç³Ø°å³ØÉôǾ¿À·ÐÆâ²Ê¡¤3)½çŷƲÂç³Ø°å³ØÉô¿ÍÂÎÉÂÍýÉÂÂֳعֺÂ
 üâÍü²í»Ë1)¡¢Ã«¸ýÂçÍ´2)¡¢À¾²¬·òÌï2)¡¢çÕ»³³Ø2)¡¢ÄÅ»³æÆ3)¡¢ÉþÉô¿®¹§2)
 
2©Àڤ졢°×ÈèÏ«´¶¤Çȯ¾É¤·¡¢µÞ®¤Ë¸ÆµÛº¤Æñ¤¬¿Ê¹Ô¤·¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î°ìÎã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¦Ç¾Â´Ãæ²Ê¡¤
 ¹âÅÄÃ鹬1)¡¢¹­µÈÍ´»Ò2)¡¢ºä²¼ÂÙ¹À1)¡¢¾¾¸¶Ãι¯1)¡¢»³ºê´´Âç1)¡¢ÀçÀÐϣʿ1) 2)¡¢¶â´ÝÏÂÉÙ2)¡¢Â¼»³ÈËͺ1)2)
 
3°¡µÞÀ­¤Ë¿ȯ¤·¤¿¹­ÈƤÊǾ¹¼ºÉ¤Ë¤è¤ê»àµî¤·¤¿Â¦Æ¬Æ°Ì®µðºÙ˦À­Æ°Ì®±ê¤Î°ì˶¸¡Îã
 1)½çŷƲÂç³Ø°å³ØÉôÉÕ°Ǿ¿À·ÐÆâ²Ê¡¤2)½çŷƲÂç³Ø°å³ØÉôÉÕ°½çŷƲ±Ûëɱ¡¿À·ÐÆâ²Ê
 Ã«¸ýÂçÍ´1)¡¢¾åÌîÍ´»Ê1)¡¢¿ÜÅŸ½¼1)¡¢ÃæÌîʸ1)¡¢üâÍü²í»Ë2)¡¢ÉþÉô¿®¹§1)
 
4²­Æì·¿¿À·Ð¸¶À­¶Ú°à½Ì¾É¡ÊHMSN-P¡Ë¤Î°ì˶¸¡Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼ ¿À·ÐÉÂÍý¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2) ¹ñΩɱ¡µ¡¹½²­Æìɱ¡ ¿À·ÐÆâ²Ê¡¤3) ¹ñΩɱ¡µ¡¹½²­Æìɱ¡ ÉÂÍý¿ÇÃDzʡ¤4) ÆÁÅçÂç³Ø ¿À·ÐÆâ²Ê¡¤5) °¦Ãΰå²ÊÂç³Ø²ÃÎð°å²Ê³Ø¸¦µæ½ê
 ¾¾¸¶Ãι¯1), ÃæÃÏμ2), ¿Ûˬ±à½¨¸ã2), Ç®³¤·ÃÍý»Ò3), ÅϲÅÉß¿ò2), ¾ë¸ÍÈþÏ»Ò2), ´¤°æ½Ó¹§4), ÏÂÀôÍ£¿®4), ³áζѻ4), µÈÅÄâÃÍý5), ¼»³ÈËͺ1)¡¤
 
5µÞ®¿Ê¹ÔÀ­¤ÎǧÃε¡Ç½Äã²¼¤òÄ褷¡¢¿ÇÃÇ´ð½à¾åprobable CJD¤òËþ¤¿¤·¤¿¤¬¡¢¥×¥ê¥ª¥óÃÁÇòÌȱÖÀ÷¿§¤ÇÍÛÀ­½ê¸«¤òǧ¤á¤Ê¤«¤Ã¤¿°ìÎã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÄëµþÂç³Ø¿À·ÐÆâ²Ê¡¤3)ÄëµþÂç³ØÉÂÍý³Ø¹ÖºÂ
 ºä²¼ÂÙ¹À1)¡¢Âç·§½¨É§2)¡¢±§±÷ºê¹¨3)¡¢Ê¡Î¤ÍøÉ×3)¡¢±àÀ¸²í¹°2)¡¢Â¼»³ÈËͺ1)¡¤
 
6¹³À¸ºÞǾ¾É¤¬µ¿¤ï¤ì¤¿°Õ¼±¾ã³²¤ò·Àµ¡¤ËÆñ¼£À­áÛÚ»½ÅÀѾõÂ֤Ȥʤꡤ¹­ÈϤÊÂçǾÇò¼ÁÉÂÊѤòÄ褷¤¿88ºÐ½÷À­¤Î˶¸¡Îã
 1)ÅìµþÄþ¿®É±¡ ¿À·ÐÆâ²Ê¡¤2)Ʊ ÉÂÍý¿ÇÃDzʡ¤3)¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼Î×¾²¸¡ººÉô
 ¹ÓÀî¾½1)¡¤´ØÂçÀ®1)¡¤º´Æ£Ã£ºÈ1),¾¡Ëô½ß»Ò1)¡¤Æü½Ð»³Âó¿Í1)¡¤Åļ¹À°ì2)¡¤óîƣʹ»Ò3)¡¤ÄÇÈø¹¯1)
 

117 KNP

*Date¡§20171216
*Manager¡§Takahiro Fukuda

1Forty seven year old male biopsy case with subacute encephalopathy resistant to steroid therapy
 1)Neurol., 2)Pathol., 4)Clin. Pathol., 5)Neuropathol., The Jikei Univ. Schl. of Med., 3)Neurol., Tohoku Univ.
 Komatsu T1), Nakamura M2), Kaneko K3), Kitagawa T1), Sakai K1), Iguchi Y1), Takahashi H4), Fukuda T5)
 
2Èæ³ÓŪ´Ë½ù¤Ë¿Ê¹Ô¤·¤¿¥×¥ê¥ª¥óɵ¿¤¤¤Î84ºÐ½÷À­¡¤
 £±¡Ë¹ñΩɱ¡µ¡¹½ÁêÌϸ¶É±¡¡¡¿À·ÐÆâ²Ê¡¤¡¤£²¡Ë¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Î×¾²¸¡ººÉô¡¤¡¤£³¡ËÅìËÌÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²Ê¡¡ÉÂÂÖ¿À·Ð³ØʬÌî
 Âç¾Âº»¿¥1)¡¢¾®¾¾ÁÕ»Ò2)¡¢¡ûóîƣʹ»Ò2)¡¢ËÌËÜůǷ3)¡¢Ä¹Ã«Àî°ì»Ò1)
 
3¹âÎðȯ¾É¤ÎOptineurin E478GÊÑ°Û¤òǧ¤á¤ë¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î1Îã
 1)ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢3)¹­ÅçÂç³Ø¸¶ÇúÊüÀþ°å²Ê³Ø¸¦µæ½êʬ»Ò±Ö³Ø¸¦µæʬÌî
 ÏÂÀôÍ£¿®1)¡¢¹âÅÄÃ鹬2)¡¢µÜËÜμ²ð1)¡¢Ìî»û͵Ƿ1)¡¢´¤°æ½Ó¹§1)¡¢Àî¾å½¨»Ë3)¡¢¡¤Â¼»³ÈËͺ2)¡¢³á¡¡Î¶Ñ»1)
 
4·Ð²á61ǯ¤ÎÀÔ¿ñÀ­¶Ú°à½Ì¾ÉII·¿ÃËÀ­Îã
 1)Âçºå·Ù»¡É±¡ÉÂÍý¿ÇÃDzʡ¤2)¹ñΩɱ¡µ¡¹½ÅẬ»³É±¡¿À·ÐÆâ²Ê¡¤3)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤
 »³»û¤ß¤µ¤­1)2)¡¢óîÆ£Íøͺ2)¡¢Æ£Â¼À²½Ó2)¡¢Â¼»³ÈËͺ3)
 
5An autopsy case of progressive multifocal leukoencephalopathy in a 66-year-old man with idiopathic CD4+ lymphocytopenia
 1) Department of Neurology, Kameda medical center¡¤2) Department of Neurology, Tokyo metropolitan geriatric hospital
 Tatsuya Fukumoto1), Syun Akaike1), Kentaro Tokumoto1), Masaki Tomura,1), Kazue Tajima1), Ryoko Takeuchi1), Fumiaki Katada1), Susumu Sato1),Hidehiro Shibayama1), Shigeo Murayama2), Toshio Fukutake1).
 66-year-old man who had no specific past medical history first presented with dysarthria and gait disturbance. The diagnosis was cerebellar infarction, but his symptoms were deteriorated afterward and he was admitted to our hospital. The diagnosis of central pontine myelinolysis was made and he took the physical therapy. His symptoms were improved temporary, but eventually deteriorated and became akinetic mutism. Brain MRI showed diffuse expending lesions in the bilateral hemisphere and brainstem. PCR of JC-virus in CSF was positive and he was diagnosed progressive multifocal leukocytopenia. Serum CD4 positive lymphocyte was very low, but HIV studies were negative. Therefore, he also had idiopathic CD4 positive lymphocytopenia. 10 years later, he died for sepsis caused by obstruction of the common bile duct. Pathological study revealed that almost total loss of myeline fibers in the white matters, sparing gray matter. Severe neuronal loss of CA1 of the right hippocampus was also seen, compatible to post epileptic encephalopathy. ¡¤
 
618F-THK5351 PET¤Ç¿íÂÎÏ©¤Ë±è¤Ã¤¿½¸ÀѤòǧ¤á¤¿ç±²ê¼ð¤Î°ì˶¸¡Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼ ¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2) Ʊ ¸¦µæ½ê ¿À·ÐÉÂÍý¡¤3) Ʊ ¿À·ÐÆâ²Ê¡¦Ç¾Â´Ãæ²Ê¡¤4) Ʊ ¿À·Ð²èÁü¸¦µæ¥Á¡¼¥à¡¤
 ¾¾¸¶Ãι¯1,2)¡¤ÇòÀÐÊþ·É3)¡¤ÀçÀÐϣʿ1,3), ¹âÅÄÃ鹬1,2), »³ºê´´Âç1,2), ºä²¼ÂÙ¹À1,2), ¾®ÎÓËü´õ»Ò1,2), Æ£¥öùõ½ã»Ò2), ¿¸ÕůϺ4), Ë­¸¶½á4), Àа渭Æó4), ¶â´ÝÏÂÉÙ3)¡¤Â¼»³ÈËͺ1,2,3)
 
7¹âÅ٤ʱ¿Æ°ÌîÈé¼ÁÊÑÀ­¤È3¥ê¥Ô¡¼¥È¥¿¥¦ÍÛÀ­¥°¥ê¥¢¹½Â¤Êª¤ò¿¿ôȼ¤Ã¤¿¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤Î84ºÐÃËÀ­
 1)½çŷƲÂç³Ø°å³ØÉôǾ¿À·ÐÆâ²Ê¡¢2)½çŷƲÂç³Ø°å³ØÉô¿ÍÂÎÉÂÍýÉÂÂֳء¢3)½çŷƲÂç³Ø°å³ØÉôÉÕ°±Ûëɱ¡¿À·ÐÆâ²Ê
 Ã«¸ýÂçÍ´ 1)¡¢ÇÈÅÄÌîÂö 1)¡¢´ßÀ¤Ä¤­ 2)¡¢üâÍü²í»Ë3)¡¢ÉþÉô¿®¹§ 1)
 

116 KNP

*Date¡§20170701
*Manager¡§Kinya Ishikawa

1̵¾É¸õ¤ÎǾη绡¦Ç¾²ó·ÁÀ®°Û¾ï¤Ë¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤ò¹çÊ»¤·¤¿1Îã
 1) ¥Ó¥Ï¡¼¥é²Ö¤ÎΤɱ¡¡¢2) ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢3) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢4) ¹­ÅçÀ¾°åÎÅ¥»¥ó¥¿¡¼Î×¾²¸¡ºº²Ê¡¢5) ¹­ÅçÀ¾°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¢6) ¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼Î×¾²¸¡ººÉô¡¤
 ÏÂÀôÍ£¿®1,2) ¡¢¹âÅÄÃ鹬3)¡¢¿¥ÅIJíÌé1)¡¢Î©»³µÁϯ4)¡¢ÅÏÊÕÀé¼ï5)¡¢óîƣʹ»Ò6)¡¢Â¼»³ÈËͺ3)
 
2MRI²èÁü¾åsolitary cortical tuber¤¬µ¿¤ï¤ì¤¿Â¦Æ¬ÍդƤ󤫤ó¤Î4ºÐÃË»ùÎã
 1)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡ ¾®»ù¿À·Ð²Ê¡¤2)Ʊɱ¡¡¡Ç¾¿À·Ð³°²Ê¡¤3)Ʊɱ¡¡¡Î×¾²¸¡ººÉôÉÂÍý¡¤4)Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼
 ¾®³Þ¸¶¿¿»Ö1)¡¢º´¡¹ÌÚÀ¬¹Ô1)¡¢´äºê¿¿¼ù2)¡¢º´Ìŵ3)¡¢ÀÆƣʹ»Ò3)¡¢½ÂëÀ¿4)
 
3A case of familial cerebellar ataxia
 Tokyo Medical and Dental University
 Kinya Ishikawa, MD. and Yuka Hirota, MD.
 
4ÈéÉæÀ¸¸¡¤ÇLewy¾®ÂÎÉÂÍý¤òǧ¤á¡¢Ë¶¸¡¤Ç¥Ñ¡¼¥­¥ó¥½¥óɤȳÎÄê¿ÇÃǤ·¤¨¤¿86ºÐÃËÀ­Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤2) Ʊ¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤3) Ʊ¡¡¸¦µæ½ê¿À·ÐÉÂÍý¡¤4) Ʊ¡¡¿ÇÃÇÉÂÍý¡¤¡¤
 ½ÂÀîçýè½1)¡¢ÀçÀÐϣʿ1,2)¡¢¹âÅÄÃ鹬2)¡¢¶â´ÝÏÂÉÙ1)¡¢ºÇ¾¡»û½ã»Ò3)¡¢¿·°æÉÚÀ¸4)¡¢Â¼»³ÈËͺ1)-3)
 
5Ǿ´´ÉÂÊѤÇȯ¾É¤·¡¤Ìó1¥±·î¤Î·Ð²á¤ÇµÞ®¤Ë¿Ê¹Ô¤·»àË´¤·¤¿·ì´ÉÆâÂçºÙ˦·¿BºÙ˦À­¥ê¥ó¥Ñ¼ð¤Î81ÃËÀ­Ë¶¸¡Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤2) Ʊ¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤3) Ʊ¡¡¸¦µæ½ê¡¡¿À·ÐÉÂÍý¡¤4) Ʊ¡¡¿ÇÃÇÉÂÍý¡¤
 ÇòÀÐÊþ·É1)¡¤ÀçÀÐϣʿ1,2)¡¤»³ùõ´´Âç2)¡¤¾®ÎÓËü´õ»Ò2)¡¤Æ£¥öùõ½ã»Ò3)¡¢¿·°æÉÚÀ¸4)¡¢Â¼»³ÈËͺ1,2)
 
6¹­ÈϤʥì¥Ó¡¼ÉÂÍý¤ÈÓ϶äðù㤬½Ð¸½¤·¤¿¤¬¡¢Î×¾²Åª¤ËǧÃε¡Ç½¾ã³²¤¬Ìµ¤«¤Ã¤¿¥Ñ¡¼¥­¥ó¥½¥óÉ¡¢STN-DBS½Ñ»Ü¹Ô¤Î76ºÐÃËÀ­
 1) ½çŷƲÂç³Ø°å³ØÉôÉÕ°±Ûëɱ¡¿À·ÐÆâ²Ê¡¤2) ½çŷƲÂç³Ø°å³ØÉô¿ÍÂÎÉÂÍýÉÂÂֳء¤3) ½çŷƲÂç³Ø°å³ØÉôǾ¿À·ÐÆâ²Ê
 üâÍü²í»Ë1)¡¢´ßÀ¤Ä¤­2)¡¢²¼ÂÙ»Ê3)¡¢ÉþÉô¿®¹§3)
 

115 KNP

*Date¡§20170318
*Manager¡§Noriyuki Shibata

1An autopsy case of 76-year old man with multiple system atrophy and Parkinson¡Çs disease
 1) Department of Neurology, Tokyo Teishin Hospital, 2) Department of Pathology, Tokyo Teishin Hospital, 3) Department of Laboratory Medicine, National Center Hospital, NCNP.
 Miho Matsukawa 1), Takuto Hideyama 1), Risa Maekawa 1), Yukiko Kishida 2), Koichi Tamura 2), Terunori Sano 3), Yuko Saito 3), Yasushi Shiio 1)
 
2¼ó²¼¤¬¤ê¤È¸¸³Ð¤Çȯ¾É¤·¤¿1Îã
 1)ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý
 ÅÚ»ÕÀµÂÀϺ1)¡¢ÏÂÀôÍ£¿®1)¡¢¹âÅÄÃ鹬2)¡¢µÜËÜμ²ð1)¡¢Â¼»³ÈËͺ2)¡¢³áζѻ1)
 
338ºÐ¤Î»þ¡¢¥Ù¡¼¥Á¥§¥Ã¥ÈɤǼºÌÀ¡¢¤½¤Î¸å¡¢²èÁü¾å¤ÇǾ´´°à½Ì¤ò¼¨¤·¤¿¡¤80ºÐ¡¦ÃËÀ­¡¤
 Ê¡»ã¼É±¡¿À·ÐÉÂÍý¸¦µæ½ê¡¡¡¡
 ¶¶µÍÎÉÉ×
 
4½é´üÀÔ¿ñ¾®Ç¾ÊÑÀ­¾É¤Î¿ÇÃǤǤ¢¤Ã¤¿¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤Î°ìÎã - »ë¾²²¼³Ë¤ÎÉÂÊÑʬÉÛ
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)Ʊ¿À·ÐÆâ²ÊŽ¥Ç¾Â´Ãæ²Ê 3) ¸¦µæ½ê¥Æ¡¼¥Þ¿À·ÐÉÂÍý
 ¾®ÎÓËü´õ»Ò1)¡¢¶âÅÄÂçÂÀ1)¡¢2)¡¢¹âÅÄÃ鹬1)¡¢ÃæÌîͺÂÀ1)¡¢Æ£¥öºê½ã»Ò3)¡¢¶â´ÝÏÂÉÙ2)¡¢Â¼»³ÈËͺ1)-3)
 
5MOG antibody positive, steroid responsive, and focal meningoencephalitis
 1)Dept. of Neurology, Kameda Medical Center¡¤2)Dept. of Neurology, Tohoku Univ.¡¤3)Dept. of Neurology, Yonezawa National Hospital¡¤4)Dept. of Neurology and Neuropathology (the Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Medical Center
 Tokumoto Kentaro1), Ryoko Takeuchi1), Fumiaki Katada1), Susumu Sato1), Hidehiro Shibayama1), Kimihiko Kaneko2),Toshiyuki Takahashi2)3), Shigeo Murayama4), Toshio Fukutake 1)
 
6An autopsy case of 55-year old man with Down's syndrome
 1)Dept. of Pathology and Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry¡¤2)Tokyo Medical Examiner's Office¡¤3)Dept. of Psychiatry, National Center Hospital, National Center of Neurology and Psychiatry¡¤4)Dept. of Radiology, National Center Hospital, National Center of Neurology and Psychiatry
 Terunori Sano1), Masahiko Hamamatsu2), Naoki Yoshimura3), Atsuhiko Sugiyama4), Noriko Sato4), Yuko Saito1)
 

114 KNP

*Date¡§20161224
*Manager¡§Taku Homma

1ȯ¾É£²Ç¯¤Ç˶¸¡¤È¤Ê¤Ã¤¿¡¢PSP pure akinesia with gait freezing¤Î£±Îã
 1) ÅìµþÄþ¿®É±¡¿À·ÐÆâ²Ê¡¤2) ÅìµþÄþ¿®É±¡ÉÂÍý¿ÇÃDzʡ¤3) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô
 ´Ø¡¡ÂçÀ®1), ÅÄÃæ¡¡Æĵ­1)¡¤±©Èø¡¡¶Ç¿Í1)¡¤¾¡Ëô¡¡½ß»Ò1)¡¤Á°Àî¡¡Íýº»1)¡¤Æü½Ð»³¡¡Âó¿Í1)¡¤´ßÅÄ¡¡Í³µ¯»Ò2)¡¤Åļ¡¡¹À°ì2)¡¤º´Ìî¡¡µ±Åµ3)¡¤óîÆ£¡¡Í´»Ò3)¡¤ÄÇÈø¡¡¹¯1)
 
2Êâ¹Ô¾ã³²¤ÈǧÃξɤòÄ褷¡¢DAT¥¹¥­¥ã¥ó¼è¤ê¹þ¤ßÄã²¼¤òǧ¤á¡¢ÉÂÍý³ØŪ¤ËÓ϶äðùγÀ­ÊѲ½¤È¿Ê¹ÔÀ­³Ë¾åÀ­ËãáãÍÍÊѲ½¡ÊPSP-like change¡Ë¤òǧ¤á¤¿86ºÐÃËÀ­Ë¶¸¡Î㡤
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¦Ç¾Â´Ãæ²Ê¡¡2)¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢3) ¿À·ÐÉÂÍý³Ø 4)Ʊ¿À·Ð²èÁü¸¦µæ¥Á¡¼¥à¡¢5)ƱÊü¼ÍÀþ¿ÇÃDzʡ¤
 ¶âÅÄÂçÂÀ1)2)¡¢Æ£¥öºê½ã»Ò3)¡¢¹âÅÄÃ鹬2)¡¢ÃæÌîͺÂÀ2)¡¢Àа渭Æó4)¡¢ÆÁ´Ý°¤Ìí5¡Ë ÀçÀÐϣʿ1)2)¡¡Â¼»³ÈËͺ1)2)¡¡¡¤
 
3An autopsy case of Huntington's disease
 ÅÔΩ¿À·Ðɱ¡ 1) ¸¡ºº²Ê, Ʊ 2) Ǿ¿À·ÐÆâ²Ê, 3) ÆüËÜÂç³Ø°å³ØÉô ÉÂÂÖÉÂÍý³Ø·Ï ¿ÍÂÎÉÂÍý³ØʬÌî
 ËÜ´Ö Âö1)3), ÀõÌîͧÍü2), ¾®¿¹Î´»Ê1)
 
4An autopsy case of ALS with family history of MJD
 Department of Neurology, Awa Regional Medical Center 1)¡¤Department of Neurology, Tokyo Metropolitan Institue of Gerontology
 Shuji Hirata 1), Ayumu Nouju 1), Makiko Kobayashi 2), Shigeo Murayama 2)
 The patient was 43 year-old male (at dead). He had weakness of right lower limb at the age of 41, followed by progressive gait disturbance, weakness of upper limbs, and dyspnea, then he was admitted to our hospital at the age of 43. He had many relatives with MJD diagnosed by genetic examination. He had flaccid tetraparesis, fasciculations at limbs, and areflexia of both lower limbs. He didn't have ophthalmoparesis, ataxia, or sensory disturbance. No significant abnormalities in blood examination. CSF examination showed high level of protein. Nerve conduction study of rightside limbs showed no CMAP at lower limb and low amplitude SNAPs at ulnar and sural nerves. Repetitive nerve stimulation test showed waning at right accessory and axillary nerves. EMG showed neuropathic changes at regions of brain stem and cervical, thoracic and lumbar spinal cord. Radiological imaging tests showed no significant abnormalities causing his symptoms.¡¡ALS was suspected but not determined because he had no upper motor neuron signs. Considering inflammatory neuropathies, steroid pulse therapy and IVIg were performed but not succeeded. He died of dyspnea 2.5 months later after admission.
 
5CSF1R¿·µ¬ÊÑ°Û¤òǧ¤á¤¿hereditary diffuse leukoencephalopathy with spheoroid (HDLS)¤Î£±Îã
 1)ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ÏÂÀôÍ£¿®1)¡¢¹âÅÄÃ鹬2)¡¢µÜËÜμ²ð1)¡¢´¤°æ½Ó¹§1)¡¢Â¼»³ÈËͺ2)¡¢³á¡¡Î¶Ñ»1)
 
6º¸Æ⦦ƬÍդμðáçÀ­ÉÂÊѤˤè¤ë¤Æ¤ó¤«¤ó¤òÄ褷¡¢ÀÚ½ü½Ñ¤ò»Ü¹Ô¤µ¤ì¤¿12ºÐÃË»ù
 1. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Î×¾²¸¡ººÉô¡¢2. Åìµþ°åÂçȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼¡¡Î×¾²¸¡ººÉô¡¢3. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡¾®»ù¿À·Ð²Ê¡¢4. Ʊ¡¡Êü¼ÍÀþ¿ÇÎÅÉô¡¢5. Ʊ¡¡Ç¾¿À·Ð³°²Ê
 óîƣʹ»Ò1)¡¢ß§Ã«À¿1)2)¡¢ã·Æ£µ®»Ö3)¡¢º´Ìŵ1)¡¢±Ý±àÈþ¹á»Ò4)¡¢º´Æ£Åµ»Ò4)¡¢º´¡¹ÌÚÀ¬¹Ô3)¡¢´äºê¿¿¼ù5)
 
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 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)Ʊ¿À·ÐÆâ²Ê¡¦Ç¾Â´Ãæ²Ê¡¤3)Ʊ¿À·ÐÉÂÍý¡¤
 ÃæÌîͺÂÀ1)¡¢½ÂÀîçýè½2)¡¢Æ£¥öºê½ã»Ò3)¡¤¾®ÎÓËü´õ»Ò1)¡¢¹âÅÄÃ鹬1)¡¢¶âÅÄÂçÂÀ1,2)¡¢ÀçÀÐϣʿ2)¡¢¶â´ÝÏÂÉÙ2)¡¢Â¼»³ÈËͺ1,2)
 
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 ÆüËÜÂç³Ø°å³ØÉô ÉÂÂÖÉÂÍý³Ø·Ï 1)¿ÍÂÎÉÂÍý³ØʬÌƱ 2) ·ÁÂÖµ¡Ç½ÉÂÍý³ØʬÌƱ 3) ¼ðáçÉÂÍý³ØʬÌÆüËÜÂç³Ø°å³ØÉô 4) Ǿ¿À·Ð³°²Ê³Ø·Ï ¿À·Ð³°²Ê³ØʬÌî
 ËÜ´Ö Âö1)¡¢Âç²ÙÀ¡¹¾2)¡¢»³¼¼½Ó4)¡¢ÆïÈþ²Å¹¸3)¡¢µÈÌîÆƽï4)
 

113 KNP

*Date¡§20160709
*Manager¡§Masashi Takanashi

1ÄÉÅé¡¡¿¹½¨À¸ÀèÀ¸¡¡¤½¤Î¶ÈÀÓ¤ò¿¶¤êÊÖ¤ë
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2¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¡¢Ç§Ãε¡Ç½¾ã³²¤ò¼ç¾É¾õ¤È¤·¤Æ20ǯ¤Î·Ð²á¤Ç¹âÅÙ¤ÎÁ°Æ¬Â¦Æ¬ÍÕ°à½Ì¤òÄ褷¤¿¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼¡¢¥ì¥Ó¡¼¡¢TDP-43º®¹çÉÂÍý¤Î86ºÐ½÷À­Ë¶¸¡Îã
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3ľIJ´âµÚ¤Ó¹üÈ×ÆâÇ¿áç¤Î¼£ÎÅÃæ¤ËµÞ®¿Ê¹ÔÀ­¤ÎÇò¼ÁǾ¾É¤òÄ褷¤¿66ºÐ½÷À­Ë¶¸¡Îã
 1µµÅÄÁí¹çɱ¡¡¡¿À·ÐÆâ²Ê¡¢2ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê
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 1) Åìµþ°å²ÊÂç³Øɱ¡¡¡¿À·ÐÆâ²Ê³ØʬÌ2) Åìµþ°å²ÊÂç³Ø¡¡´ðÁðå³Ø·Ïʬ»ÒÉÂÍý³ØʬÌ3) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¦¥Ð¥¤¥ª¥ê¥½¡¼¥¹¥»¥ó¥¿¡¼¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡Ê¿À·ÐÉÂÍý¡Ë
 ¾®ÎÓËü´õ»Ò1)¡¢óîäÛÃÒ»Ò1)¡¢µÆÌÌÀ1)¡¢°æ¾åʸ1)¡¢ÃæÌîͺÂÀ3)¡¢»³ÅÄÀµ½Ó2)¡¢Ê÷Èø¾¾°ìϺ2)¡¢¹õÅIJíɧ2)¡¢Â¼»³ÈËͺ3)¡¢Áêß·¿Î»Ö1)
 
7RT-QuICË¡ÍÛÀ­¤Ç¸ÉȯÀ­¥¯¥í¥¤¥Ä¥Õ¥§¥ë¥È¡¦¥ä¥³¥ÖÉÂMV2K¤Îµ¿¤ï¤ì¤¿°ì˶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý³Ø¡¤2)Ʊ¿À·ÐÆâ²Ê¡¤3)Ʊ¿À·Ð²èÁü¸¦µæ¥Á¡¼¥à¡¤4)ĹºêÂç³Ø°å»õÌô³ØÁí¹ç¸¦µæ²Ê±¿Æ°¾ã³²¥ê¥Ï¥Ó¥ê¥Æ¡¼¥·¥ç¥óʬÌî¡Ê¿À·ÐÆâ²Ê³Ø¡Ë¡¤5)ÅìËÌÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÉÂÂÖ¿À·Ð³ØʬÌî
 ÃæÌîͺÂÀ1)¡¤ËÜ»³¤ê¤¨2)¡¢Àа渭Æó3)¡¢º´Æ£¹îÌé4)¡¢ËÌËÜůÉ×5)¡¢¹âÅÄÃ鹬1)¡¢¶âÅÄÂçÂÀ1)¡¢Æ£¥öºê½ã»Ò1)¡¤¶â´ÝÏÂÉÙ2)¡¢Â¼»³ÈËͺ1)
 
8Ó̵å¤ËHSV-1¹³ÂÎÍÛÀ­½ê¸«¤È¥ê¥ó»À²½¦Á-synucleinÍÛÀ­½ê¸«¤¬Ç§¤á¤é¤ì¤¿ŽÍŽÙŽÍŽßŽ½Ç¾±ê¤Î°ì˶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý³Ø¡¢2)Ʊ¸ÆµÛ´ïÆâ²Ê¡¢3)Ʊ¿À·ÐÆâ²Ê
 ¹âÅÄÃ鹬1)¡¢º´Äͤޤʤß2)¡¢Å츶¿¿Æà3)¡¢ÃæÌîͺÂÀ1)¡¢¶âÅÄÂçÂÀ1)3)¡¢Æ£¥öºê½ã»Ò1)¡¢¶â´ÝÏÂÉÙ3)¡¢Â¼»³ÈËͺ1)3)
 
9AI¤Çȯ¸«¤µ¤ì¤¿Ä¶µÞÀ­´üǾ¹¼ºÉ
 ¹ñΩ¹ñºÝ°åΟ¦µæ¥»¥ó¥¿¡¼ÉÂÍý¡¦¿À·ÐÆâ²Ê¡¢ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ¹õÅÄμʿ¡¢¿·°æ·û½Ó¡¢Â¼»³ÈËͺ
 

112 KNP

*Date¡§20160326
*Manager¡§Takahiro Fukuda

1Neuropathology of amygdala enlargement accompanying temporal lobe epilepsy
 1)Dept. of Radiology, National Center of Neurology and Psychiatry¡¤2)Dept. of Pathology, the University of Tokyo Hospital¡¤3)Dept. of Pathology and Laboratory Medicine, National Center of Neurology and Psychiatry¡¤4)Dept. of Neurosurgery, the University of Tokyo Hospital¡¤5)Dept. of Neuropsychiatry, the University of Tokyo Hospital
 Daichi Sone, Masako Ikemura, Yuko Saito, Naoto Kunii, Go Taniguchi
 Recently, an increasing number of cases of temporal lobe epilepsy showing ipsilateral amygdala enlargement have been reported. As for the histopathology, in non-neoplastic cases, dysplastic findings such as hypertrophic neurons, vacuolation, and clustering of oligodendroglia-like cells were reported. However, there still have been only a few reports, and no distinct consensus is established. We experienced a case with refractory left temporal lobe epilepsy from the age of 14, who underwent surgery at age 40 years. The preoperative brain MRI revealed left amygdala enlargement, left hippocampal atrophy, and T2WI/FLAIR hyperintensity on them. Regarding the pathology of the enlarged amygdala, we found remarkable clustering of oligodendroglia-like cells in hippocampus amygdala transition area as well as hypertrophic neurons and valuolation as described in a previous report. In the present session, we expect a comprehensive discussion about such neuropathological findings in amygdala enlargement accompanying temporal lobe epilepsy as this case.
 
2Co-occurrence of Alzheimer and TDP-43 pathologies in an 81-year-old man with Alzheimer type dementia
 1)Dept. Neurology, 2)Dept. Pathology 2nd, 3)Dept. Pathology 1st, Tokyo Women's Medical University
 Takahiro Takeda1), Kenji Maruyama1), Hiroshi Yoshizawa1), Saeko Yoshizawa2), Noriyuki Shibata3), Kazuo Kitagawa1)
 An 80-year old man presented to our hospital with complaints of amnesia and irritability. On examination, mini-mental state examination score was 13, and brain MRI demonstrated bilateral severe atrophy of medial temporal regions. Muscle weakness and atrophy were absent. Memory impairments and spatial agnosia were gradually worsened. He was diagnosed as having an Alzheimer type dementia and the treatment was started with donepezil. At the age of 81, he suffered from intestinal obstruction and was admitted to our hospital. The abdominal symptoms were improved with treatment, however, the consciousness deteriorated during a few weeks. Although systemic management could provide temporary improvement of consciousness level, the pleural effusion became obvious. He died from respiratory failure 2 months after the admission. The patient¡Çs brain weighed 1,200 g and macroscopically showed moderate atrophy of anteromedial temporal regions. There was neuronal loss and gliosis restricted to entorhinal cortices and amygdaloid nuclei. Neurofibrillary tangles (NFT) were found in the hippocampal complex and associative cortices (Braak NFT stage IV). Amyloid ¦Â deposition spread to neocortex, limbic area and striatum (Thal phase 3). Moderate amount of cytoplasmic inclusions immunoreactive for phosphorylated TDP-43 were present in the small neurons of entorhinal cortex, dentate gyrus, amygdala and insular cortex. TDP-43-positive neurites were rare (Mackenzie B). Alzheimer pathology and concurrent TDP-43 pathology could contribute to cognitive impairment in this patient. The relationship between tau and TDP-43 deposition remained elucidated.
 
3An autopsy case of ALS with novel TDP-43 mutation
 1) Dept. of Neurol., Kohsei Chuo Hosp.¡¤2) Dept. of Neurol. and Neuropathol., Tokyo Metropolitan Geriatric Hosp. & Inst. of Geronto¡¤logy¡¤3) Dept. of Neurol., Univ. of Kyoto
 Naoyuki Kitagawa 1), Akiko Uchino 2), HIrohumi Yamashita 3), Makoto Urushitani 3), Shigeo Murayama 2)
 
4A case of TDP-43 negative amyotrophic lateral sclerosis with marked lower motor neuron signs.
 1) Department of Neurology, Vihara Hananosato Hospital¡¤2)Department of Neurology, Neuropathology Tokyo Metropolitan Geriatric Hospital.¡¤3)Department of Neurology, Tokushima University
 Matsubara T., Oda M.,Murayama S., Izumi Y.
 
5²È²À­SCA¤È¿ÇÃǤµ¤ì¤Æ¤¤¤¿64ºÐÃËÀ­¤Î¹ÔÀ¯²ò˶Îã
 1)¡¡ÅìµþÅÔ´Æ»¡°å̳±¡¡¡2¡ËÅìµþ»ü·Ã²ñ°å²ÊÂç³Ø¿À·ÐÉÂÍý³Ø
 Ä«ÁÒµ×Èþ»Ò1)¡¢Ê¡ÅÄδ¹À2)¡¢ÎÓµªÇµ1)¡¢Ê¡±ÊζÈË1)
 
6ʪ˺¤ì³°Íè¼õ¿Ç»þ¤Ë·ÚÅÙǧÃξ㳲(MCI)¤òǧ¤á¡¢8¤«·î¸å¤Ë²èÁü¾å¿Ê¹ÔÀ­Ç¾°à½Ì¤òÄ褷Á´¿È¼À´µ¤Ç»àË´¤·¤¿87ºÐÃËÀ­Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤2)Ʊ¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤3)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Î×¾²¸¡ººÉô¡¤
 ½ÂÀîçýè½1)¡¢ÀçÀÐϣʿ1),2)¡¢ÃæÌîͺÂÀ2)¡¢Æ£¥öºê½ã»Ò2)¡¢óîƣʹ»Ò3)¡¢¶â´ÝÏÂÉÙ1)¡¢Â¼»³ÈËͺ1),2)
 
7¹³¥¢¥ß¥í¥¤¥É¦Â¹³Âμ£ÎŸå˶¸¡Îã
 Department of Neurology, Himeji Center Hospital ¡¤Department of Neurology and Neuropathology, TokyoMetropolitan Geriatric Hospital and Institute ofGerontology
 Yasuto Higashi and Shigeo Murayama
 
8Postmortem studies on the first case of mucopolysaccharidosis type I treated with ¦Á-L-iduronidase in Japan
 1Pathol., 2Pediat., and 3Neuropathol., The Jikei Univ. Schl. of Med.
 Fukasawa N1, Kobayashi H2, Takahashi H1, Ikegami M1, Fukuda T3¡¤
 
9Case of vertebrobasilar artery occlusion caused by rupture of atheromatous plaque
 1¡ËDepartment of Neurology, Yokohama Rosai¡¤Hospital¡¥2¡ËDepartment of Diagnostic Pathology, Yokohama Rosai Hospital¡¥3¡ËDepartment of Neurology and Neuropathology (the Brain Bank for Aging Researc¡¤h), Tokyo Metropolitan Geriatric Hospital and Gerontology
  Imafuku I1¡Ë¡¤Nakano Y3¡Ë¡¤Sugiyama Y1¡Ë¡¥Kakuta Y2¡Ë¡¤Matsuda S1¡Ë¡¥Kitamura M1¡Ë¡¤Nakayama T1¡Ë¡¤Murayama S3¡Ë¡¥
 

111 KNP

*Date¡§20151226
*Manager¡§Kinya Ishikawa

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 Ô¢ÅÚ¡¡ÍËÊ¿1¡Ë¡¢ÆâÌî¾´»Ò5)¡¢¹âÅÄ¡¡Ã鹬 2¡Ë¡¢µ×ÊÆ¡¡¹­Âç 1¡Ë¡¢ÃÓÅÄ¡¡ÏÂÂå1¡Ë¡¢³ùÅÄ¡¡Àµµª2¡Ë¡¢½Ð¸ý¡¡°ì»Ö1¡Ë¡¢Æ½¡¡Å¯ÃË3¡Ë¡¢ÀµÌÚ¡¡ÊÙ1¡Ë¡¢ÀéÍÕÍÛ°ì4¡Ë¡¢¾åÌîÀµ¼ù4¡Ë¡¢Â¼»³ÈËͺ5)
 
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 °ÂÉôÅ´Ìé1)¡¢¿åÌîÍ¡»ù1)¡¢º´ÌîÇ1)¡¢Ä¹ÎæÍ£ÅÍ1)¡¢ËÙÆâÍÛ²ð1)¡¢ÂçÊ¿²íÇ·1)¡¢´Ý»³¸µ1)¡¢½Ð¸ý°ìϺ1)¡¢Ê¡²¬ÂîÌé1)¡¢ÎÓ·ò1)¡¢»°Åç°ìɧ2)¡¢ÆâÌ3)¡¢º´¡¹ÌÚÆ×4)¡¢Ãª¶¶µªÉ×1)¡¢¹âÈø¾»¼ù1)
 
5One autopsy case of Globular glial tauopathy(GGT) showing a clinical feature of amyotrophic lateral sclerosis with dementia (ALS-D)
 1) Âçºå»ÔΩÂç³ØÂç³Ø±¡°å³Ø¸¦µæ²Ê ¿À·ÐÆâ²Ê¡¤2) Ʊ ¿ÇÃÇÉÂÍý³Ø¡¤3) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼ ¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý¡¤4) ¿·³ãÂç³ØǾ¸¦µæ½êÉí°À¸Ì¿²Ê³Ø¥ê¥½¡¼¥¹¸¦µæ¥»¥ó¥¿¡¼°äÅÁ»Òµ¡Ç½²òÀϳØʬÌ5) ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½êǧÃξɡ¦¹â¼¡Ç¾µ¡Ç½Ê¬ÌîÉÂÂÖºÙ˦À¸Êª¸¦µæ¼¼
 Ä¹Ã«Àî ¼ù1), °ËÆ£ ÏÂÇî1), ½éÅÄ Íµ¹¬1)3), µ×ÊÝͦµ­2), Âçß· À¯É§2), ÃæÌî ͺÂÀ3), ÃÓÆâ ·ò4), ĹëÀî À®¿Í5), ¼»³ ÈËͺ3), °ËÆ£ µÁ¾´1)¡¤
 
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 1) ÆüËÜÂç³Ø°å³ØÉô ÉÂÂÖÉÂÍý³Ø·Ï¿ÍÂÎÉÂÍý³ØʬÌ2) ÅÔÊÝ·ò°åΟø¼Ò±Á¸¶É±¡¿À·ÐÆâ²Ê¡¤3) ¤¬¤ó¡¦´¶À÷¾É¥»¥ó¥¿¡¼ÅÔΩ¶ð¹þɱ¡ÉÂÍý²Ê¡¤4) ÅÔÊÝ·ò°åΟø¼Ò±Á¸¶É±¡¸¡ºº²ÊÉÂÍý
 ËÜ´Ö¡¡Âö1)¡¢Åĵ×Êݽ¨¼ù2)¡¢Á¥ÅÄ¿®¸²3)¡¢¹â¶¶³Ø4)
 
7Fragile X¾É¸õ·²¤Î£±Ë¶¸¡Îã
 1)ÅìµþÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔΩ¿À·Ðɱ¡¸¡ºº²Ê¡¢3)ÆüËÜÂç³Ø°å³ØÉôÉÂÂÖÉÂÍý³Ø·Ï¿ÍÂÎÉÂÍý³ØʬÌî
 ÃÝÆâÀéÀ硢˾·îÍÕ»Ò
 
8Êä½õ¸ÆµÛ¤Ê¤·¤Ë22ǯÀ¸Â¸¤·¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤ÎÃËÀ­Îã
 1)ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¢3)µþÅÔÂç³Ø¿À·ÐÆâ²Ê
 »³¾å¡¡·½1)¡¢ÏÂÀôÍ£¿®1)¡¢ÆâÌî¾´»Ò2)¡¢ÉðÆ£¹ÀÊ¿1)¡¢³á¡¡À¿Ñ»3)¡¢µÜËÜμ²ð1)¡¢´¤°æ½Ó¹§1)¡¢Â¼»³ÈËͺ2)¡¢³á¡¡Î¶Ñ»1)
 

110 KNP

*Date¡§20150704
*Manager¡§Noriyuki Shibata

1ÌÀ¤é¤«¤Êwearing-off¤ª¤è¤ÓdopaÈ¿±þÀ­¤ÎÓë²¼¾ã³²¤òÄ褷Î×¾²¾å¥Ñ¡¼¥­¥ó¥½¥óɤȿÇÃǤµ¤ì¤Æ¤¤¤¿73ºÐÃËÀ­Ë¶¸¡Îã
 1)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2)Ʊ¿À·ÐÆâ²Ê¡¤3)ƱÊü¼ÍÀþ¿ÇÎÅÉô, 4)Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼Ãæ±û¸¡ººÉô, 5)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½ê/ǧÃξɡ¦¹â¼¡Ç¾µ¡Ç½¸¦µæʬÌîÉÂÂÖºÙ˦À¸Êª¸¦µæ¼¼
 º´Ìŵ1)¡¤¸Åß·²Åɧ2)¡¤²£»³¹¬ÂÀ3)¡¤º´Æ£Åµ»Ò3)¡¤ß§Ã«À¿4), ĹëÀîÀ®¿Í5), ¼ÅÄÈþÊæ2)¡¤óîƣʹ»Ò1)
 
2¼ó²¼¤¬¤ê¤Î¤ß¤òǧ¤á¤¿MSA¤Î°ì˶¸¡Îã
 1)¿À¹Ýµ­Ç°É±¡¡¢2¡Ë·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼
 ¹â¶¶ÀµÇ¯1)¡¢¾¾ËÜ¿¿°ì1)¡¢ÆâÌî¾´»Ò2)¡¢Â¼»³ÈËͺ2)
 
3Êâ¹Ô¾ã³²¡¢¿¶Àï¤Î¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¤òǧ¤á¡¢·Ð²áÃæǧÃε¡Ç½Äã²¼¤¬¿Ê¹Ô¤·¤¿Á´·Ð²á4ǯ¤ÎPallidonigroluysian atrophy (PNLA)˶¸¡Îã
 1) ½çŷƲÂç³Ø°å³ØÉôǾ¿À·ÐÆâ²Ê¡¤2) ½çŷƲÂç³Ø°å³ØÉôÉÕ°½çŷƲÅìµþ¹¾Åì¹âÎð¼Ô°åÎÅ¥»¥ó¥¿¡¼Ç¾¿À·ÐÆâ²Ê
 ¹âÍü²í»Ë 1)¡¢Ã渶Åлּù 2)¡¢¸ÅÀî˧ÌÀ 2)¡¢ÉþÉô¿®¹§ 1)
 
4A brain biopsy case of 22 y.o. female
 1 Div Neuropathol., 2Dept Clin. Pathol., 3Dept. Neurosurg., The Jikei Univ. Schl. of Med.
 Fukuda T1, Nomura K2, Watanabe N3, Arai T3
 
5Ĺ´ü´Ö¤ÎÎ×¾²·Ð²á¤ò´Ñ»¡¤·ÆÀ¤¿81ºÐÃËÀ­¥Ñ¡¼¥­¥ó¥½¥óɤΰì˶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý³Ø¡¤2)Ʊ¿À·ÐÆâ²Ê¡¤3)ƱÊü¼ÍÀþ¿ÇÃDzÊ
 ÃæÌîͺÂÀ1)¡¤¶â´ÝÏÂÉÙ2)¡¤ÆâÌî¾´»Ò1)¡¤ÆÁ´Ý°¤Ìí3)¡¤Æ£¥öºê½ã»Ò1)¡¤Â¼»³ÈËͺ1)
 
6µÞ®¤Ê·Ð²á¤ò¤¿¤É¤Ã¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î°ìÎã
 
 ÏÂÀôÍ£¿®1)¡¢ÆâÌî¾´»Ò2)¡¢ÉðÆ£¹ÀÊ¿1)¡¢³á¡¡À¿Ñ»3)¡¢Â¼»³ÈËͺ2)¡¢³á¡¡Î¶Ñ»1)¡¤1) ÆÁÅçÂç³Ø¿À·ÐÆâ²Ê¡¢2) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¢3) µþÅÔÂç³Ø¿À·ÐÆâ²Ê¡¤
 

109 KNP

*Date¡§20150307
*Manager¡§Hideo Mori

1Ê£»ë¤ÈËö¾¿À­´éÌÌËãáã¤Çȯ¾É¤·°¡µÞÀ­¤Ë°Õ¼±¾ã³²¡¢¸ÆµÛÍÞÀ©¤¬¿Ê¹Ô¤·¤¿81ºÐÃËÀ­Ë¶¸¡Îã
 1)½çŷƲÂç³ØǾ¿À·ÐÆâ²Ê¡¢2)½çŷƲÎýÇÏɱ¡Ç¾¿À·ÐÆâ²Ê¡¢3)½çŷƲÎýÇÏɱ¡ÉÂÍý¿ÇÃDzʡ¢4)½çŷƲ±Ûëɱ¡Ç¾¿À·ÐÆâ²Ê
 ¹âÍü²í»Ë 1)¡¢Ä®ÅÄ͵ 2)¡¢Æ£´¬´ðµª 2)¡¢¾¾ËܽӼ£ 3)¡¢¾®ÁÒ²ÃÆà»Ò 3)¡¢ºä¸ý°¡¼÷Èþ 3)¡¢»°ÎØ±Ñ¿Í 2)¡¢¿¹½¨À¸ 4)¡¢ÉþÉô¿®¹§ 1)
 
2BetzºÙ˦¡¢·Û¿ñÁ°³ÑºÙ˦¤Ë¥¿¥¦ÃßÀѤòǧ¤á¤¿¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤Î1Îã
 1)¹áÀîÂç³Ø¾Ã²½´ï¿À·ÐÆâ²Ê¡¡¡¤2)Ʊ¡¡¿À·ÐÆñɹֺ¡¡¡¤3)Ʊ¡¡·ò¹¯²Ê³Ø¡¡¡¤4)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý³Ø¸¦µæ¡¤
 µ×Êƹ­Âç1)¡¢ÃæÌîͺÂÀ4)¡¢Ô¢ÅÚÍËÊ¿1)¡¢¹âÅÄÃ鹬1)¡¢ÃÓÅÄÏÂÂå2)¡¢³ùÅÄÀµµª2)¡¢½Ð¸ý°ì»Ö1)¡¢ÀµÌÚ¡¡ÊÙ1)¡¢Æ½ ůÃË3)¡¢Â¼»³ÈËͺ4)¡¤¡¤
 
3ǧÃξÉȯ¾É¸å¤ËPIB-PET¤Ç·Ð»þŪ¤Ë¥¢¥ß¥í¥¤¥É¦Â¤ÎÃßÀѤ¬´Ñ»¡¤µ¤ì¤¿97ºÐÃËÀ­Ë¶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý³Ø¡¤2)ƱPET²èÁü¿ÇÃǸ¦µæ¡¤3)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½êǧÃξɡ¦¹â¼¡Ç¾µ¡Ç½¸¦µæʬÌ
 ÃæÌîͺÂÀ1)¡¤Àа渭Æó2)¡¤Ä¹Ã«ÀîÀ®¿Í3)¡¤¶ù¢Â繬1)¡¤ÆâÌî¾´»Ò1)¡¤Â¼»³ÈËͺ1)
 
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572 y.o. male biopsy case of brain abscess
 1Div. of Neuropathol., The Jikei Univ. Schl. of Med. 2Dept. of Neurosurgery, Sato daiichi Hosp., 3Dept. of Neurosurgery, The Jikei Univ. Schl. of Med.
 Fukuda T, Yamada K, Sasaki Y, Nakahara N
 
6¿´¶Ú¥·¥ó¥Á¥°¥é¥Õ¥£¤ÇMIBG½¸ÀÑÄã²¼¤òǧ¤á¤¿Â¿·ÏÅý°à½Ì¾É¤Î67ºÐÃËÀ­Ë¶¸¡Îã
 1) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¿À·ÐÆâ²Ê¡¤3) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼¿À·Ð¸¦µæ½ê¼Àɸ¦µæÂè°ìÉô¡¤4) Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼ÉÂÍý¿ÇÃDzÊ
 º´Ìî¡¡µ±Åµ1),2), ±öë¡¡ºÌ»Ò1), À¾Ìî¡¡°ì»°3), ߧë¡¡À¿4), ÎÓ¡¡ÍÄ°Î2), ¼ÅÄ¡¡ÈþÊæ2), óîÆ£¡¡Í´»Ò1)
 
7¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼É´ØÏ¢ÉÂÍý¤Ë²Ã¤¨¤ÆÓ̵塢Íü¾õÍÕÈé¼Á¡¢Ù¨Åí³Ë¤Ë¥ê¥ó»À²½¦Á¥·¥Ì¥¯¥ì¥¤¥óÍÛÀ­½ê¸«¤òǧ¤á¤¿88ºÐÃËÀ­Ë¶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤2)Ʊ¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¦¿À·ÐÉÂÍý³Ø¡¤
 ½ÂÀîçýè½1)¡¤ÃæÌîͺÂÀ2)¡¤¶ù¢Â繬2)¡¤ÆâÌî¾´»Ò2)¡¤Â¼»³ÈËͺ2)
 

108 KNP

*Date¡§20141220
*Manager¡§Takahiro Fukuda

1ÊÕ±ï·Ï¤òÃæ¿´¤ËRD3+RD4ÍÛÀ­¤ÎPickµå¤òǧ¤á¤¿Pickɤΰì˶¸¡Îã
 ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý¡¢¿À·ÐÆâ²Ê
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2An autopsy case of Parkinson¡Çs disease with VPS35 p.D620N mutation.
 1 Dept. of Neurology,NHO Tokyo National Hospital¡¤2 Dept. of Neuropathology, Tokyo Metropolitan Institute of Gerontology. The Brain Bank for Aging Research3 Dept.of Clinical research, Tokyo National Hospital
 Ishizu N1,Sumikura H2,Hebisawa A3,Yomono H1,¡¤Kurisaki H1,Murayama S2¡¤
 
3An autopsy case of 'multisystemic type' ALS with TDP43- and cyctatin c-immunoreactive large oval cytoplasmic inclusions
 1ÂçºåÀÖ½½»úɱ¡ÉÂÍý¿ÇÃDzʡ¢2Ʊ¿À·ÐÆâ²Ê¡¢3¿®½£Âç³Ø°å³ØÉô¿À·ÐÆñɳØ
 Shintaku M1, Kaneda H2, Oyanagi K3
 
4Á´¿È·ì´É¤Î¶¹ºõ¤ÈÊĺɤòȼ¤¦º¸±ä¿ñ³°Â¦¾É¸õ·²¤Î77ºÐÃËÀ­¤Î˶¸¡Îã
 1) ÅìµþÂç³Ø°å³ØÉôÉí°É±¡¿À·ÐÆâ²Ê¡¤2) ¹ñ²È¸ø̳°÷¶¦ºÑÁȹçÏ¢¹ç²ñ¸×¤ÎÌçɱ¡¿À·ÐÆâ²Ê, 3) ¹ñ²È¸ø̳°÷¶¦ºÑÁȹçÏ¢¹ç²ñ¸×¤ÎÌçɱ¡ÉÂÍý¿ÇÃDzÊ, 4) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯, 5) ¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô
 ±­¾Â¡¡ÆØ 1) 2), Èõ¸ý¡¡Ï¼÷ 2),¡¡µÜÆ⠽ʻË2), Á°ÅÄ¡¡ÌÀ»Ò 2), ¾åºä¡¡µÁÏ 2), °ËÆ£¡¡¿µ¼£3),¡¡Æ£°æ ¾æ»Î3),¡¡Â¼»³ ÈËͺ4), óîÆ£¡¡Í´»Ò 5)
 
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 1Åìµþ»ü·Ã²ñ°å²ÊÂç³Ø¿À·ÐÆâ²Ê¡¤2Åìµþ»ü·Ã²ñ°å²ÊÂç³Ø¿À·ÐÉÂÍý¡¤
 Ç߸¶¡¡½ß1¡¢ ±ö߷ͧµ¬1¡¢²¬¡¡¾°¾Ê1¡¢ Ê¡ÅÄδ¹À2
 
6ÀÔ¿ñ¾®Ç¾ÊÑÀ­¾É¤¬µ¿¤ï¤ì¤¿¤¬°äÅÁ»ÒÊÑ°Û¤òƱÄê¤Ç¤­¤Ê¤«¤Ã¤¿61ºÐÃËÀ­Ë¶¸¡Îã
 µµÅÄ¥á¥Ç¥£¥«¥ë¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê
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107 KNP

*Date¡§20140726
*Manager¡§Kinya Ishikawa

1¿Ê¹ÔÀ­¤Î°Õ¼±¾ã³²¤È»ý³Ū¤ÊDWI¹â¿®¹æ¤òȼ¤¦¹­ÈƤÊÇò¼ÁǾ¾É¤òÄ褷¤¿81ºÐ½÷À­¤Î˶¸¡Îã
 1) ÅìµþÄþ¿®É±¡¿À·ÐÆâ²Ê, 2) ÅìµþÂç³Ø°å³ØÉôÉí°É±¡¿À·ÐÆâ²Ê, 3)ÅìµþÄþ¿®É±¡ÉÂÍý¿ÇÃDzÊ, 4) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯, 5)¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô
 º´¡¹ÌÚ¡¡ÂóÌé 1) 2), Á°Àî¡¡Íýº» 1),¡¡ÆÁ½Å ¿¿°ì1) 2), Æü½Ð»³¡¡Âó¿Í 1), ÄÇÈø¡¡¹¯ 1),´ßÅÄ Í³µª»Ò3) ,Åļ ¹À°ì3), ¶ù¢ Â繬4) , ¼»³ ÈËͺ4) , óîÆ£¡¡Í´»Ò 5)¡¤
 
2·Ð²á8ǯ¤Ç¡¤pTDP43ÍÛÀ­½ê¸«¤¬·ÚÅ٤Ǥ¢¤Ã¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¡¡64ºÐÃËÀ­¤Î˶¸¡Îã
 1) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¿À·ÐÆâ²Ê¡¤3) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼¿À·Ð¸¦µæ½ê¼Àɸ¦µæÂè°ìÉô
 º´Ìî¡¡µ±Åµ1) 2), ±öë¡¡ºÌ»Ò1), ¾¾ËÜ¡¡Àé¿Ò2), À¾Ìî¡¡°ì»°3), »³ËÜ¡¡ÉÒÇ·2), ¼ÅÄ¡¡ÈþÊæ2), óîÆ£¡¡Í´»Ò1)
 
3²ò˶»þ¤Ë¶öȯŪ¤ËƬ³¸¹üÉôʬ´ÙËפòǧ¤á¤¿Marfan¾É¸õ·²54ºÐ½÷À­Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý³Ø¸¦µæ¡¤2)Ʊ¿´Â¡³°²Ê¡¤3)ƱÉÂÍý¿ÇÃDzÊ
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4°Û¾ï¸ÀÆ°¡¢×Ñ×ˤÇȯ¾É¤·Á´·Ð²á6ǯ¡¢59ºÐ¤Ç»àË´¤·¤¿¼ãǯÀ­Ç§ÃξÉ
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 ÆÁÅçÂç³Øɱ¡¿À·ÐÆâ²Ê
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6À¸Á°¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¤òǧ¤á¡¢¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼É¤ιçÊ»¤È¿ÇÃǤµ¤ì¤Æ¤¤¤¿Ó϶äðùγÀ­Ç§Ãξɤΰì˶¸¡Îã
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106 KNP

*Date¡§20140322
*Manager¡§Noriyuki Shibata

1¤·¤Ó¤ì¤Çȯ¾É¤·¡¢¶¶¾®Ç¾Í¥°Ì¤ÇǾÀÔ¿ñ¤Ë¿ȯÀ­°Û¾ïÁý¶¯²èÁüÉÂÊѤòÄ褷¤¿62ºÐÃËÀ­¤ÎǾÀ¸¸¡Îã
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5ƬÉôMRI T2¶¯Ä´²èÁü¤Ë¤ª¤¤¤ÆÃæ¿´Á°²ó¤¬Äã¿®¹æ¤ò¡¢¤½¤Îľ²¼Çò¼Á¤¬¹â¿®¹æ¤òÄ褷¤¿Â¿·ÏÅý°à½Ì¾É¤Î£·£°ºÐ½÷À­¤Î°ì˶¸¡Îã
 1. ÆÈΩ¹ÔÀ¯Ë¡¿Í¹ñΩɱ¡µ¡¹½Åìµþɱ¡¿À·ÐÆâ²Ê¡¢2. ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢3.¹ñΩɱ¡µ¡¹½Åìµþɱ¡Î×¾²¸¦µæÉô
 ÀÐÄÅĪδ1¡¢½éÅÄ͵¹¬2¡¢ÃæÌîͺÂÀ2¡¢¶ù¢Â繬2¡¢¡¤¼Øß·¾½3¡¢Ãæ¼Èþ·Ã1¡¢·ªºêÇî»Ê1¡¢Â¼»³ÈËͺ2
 
6TDP-43 proteinopathy¤òȼ¤Ã¤¿ À®¿Í·¿AlexanderɤΠ72ºÐ½÷À­Ë¶¸¡Îã
 1) ÅìµþÂç³Ø°å³ØÉôÉí°É±¡ ¿À·ÐÆâ²Ê¡¤2) ÅìµþÂç³Ø°å³ØÉôÉí°É±¡ ÉÂÍýÉô¡¤3) ÅìµþÂç³Ø°å³ØÉôÉí°É±¡ Êü¼ÍÀþ²Ê¡¤4) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¿À·ÐÉÂÍý
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 1)²£ÉÍÏ«ºÒɱ¡¡¡¿À·ÐÆâ²Ê¡¢2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢3)²£ÉÍÏ«ºÒɱ¡¡¡ÉÂÍý¿ÇÃDzʡ¢4)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê
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105 KNP

*Date¡§20131207
*Manager¡§Hideo Mori

1ƬÉôMRI¤Î°Û¾ï½ê¸«¤òǧ¤á¤¿È¾Ç¯¸å¤«¤éǧÃξɤ¬´Ë½ù¤Ë¿Ê¹Ô¤·, ˶¸¡¤Ë¤Æ¸ÉȯÀ­Creutzfeldt-JakobÉÂMV·¿¤È¿ÇÃǤµ¤ì¤¿°ìÎã
 1) NHO ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤2) ÃÏÊýÆÈΩ¹ÔÀ¯Ë¡¿Í ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¦¿À·ÐÉÂÍý¡¤3) ÅìËÌÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²Ê¡¡ÉÂÂÖ¿À·Ð³ØʬÌ
 ¹ÓÌÚˮɧ
 
2½é´ü¤è¤ê¾®Ç¾À­±¿Æ°¼ºÄ´¤¬ÌÜΩ¤Á, ˶¸¡¤Ë¤Æ¥ì¥Ó¡¼¾®ÂΤò¹­ÈƤËǧ¤á¤¿¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤Î°ìÎã
 1) NHO ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤2) ÃÏÊýÆÈΩ¹ÔÀ¯Ë¡¿Í ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¦¿À·ÐÉÂÍý¡¤
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3Ó̵塦ÂçǾÊÕ±ï·Ï¤ª¤è¤Ó¦ƬÍÕ¿·Èé¼Á¤ËLewy¾®ÂÎÉÂÍý¤òȼ¤¦PARK2¤Î1˶¸¡Îã
 1) ÅÔΩ¿À·Ðɱ¡ ¸¡ºº²ÊÉÂÍý¡¤2) ÅÔΩ¿À·Ðɱ¡ Ǿ¿À·ÐÆâ²Ê¡¤3) ÆüËÜÂç³Ø°å³ØÉô ÉÂÂÖÉÂÍý³Ø·ÏÉÂÍý³ØʬÌ4) ÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼ ¿À·ÐÆâ²Ê
 ËÜ´Ö¡¡Âö1,3)¡¤²£ÃÏ˼»Ò2)¡¤Ë¾·îÍÕ»Ò1,4)¡¤¾®¿¹Î´»Ê1)
 
4PINK1°äÅÁ»Ò¥Û¥âÀܹ緿ÊѰۤˤè¤ë²È²À­¥Ñ¡¼¥­¥ó¥½¥óÉ (PARK6)¤Î°ì˶¸¡Î㡧Åö±¡¤Ç¤ÎPARK2˶¸¡Îã¤È¤ÎÈæ³Ó
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 ¹âÍü²í»Ë (1)¡¢½®ÊÕ¤µ¤ä¤« (2)¡¢¿¹¡¡½¨À¸(3)¡¢ÉþÉô¿®¹§ (1)
 
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6»ÑÀª»þ¿¶À±¿Æ°¼ºÄ´¤ò¼çħ¤È¤·¡¤MRI¤Ë¤Æξ¦Ã澮ǾµÓ¤Î°Û¾ï¤òÄ褷¡¤ÊÕ±ï·Ï¤Ë¿¿ô¤Î³ËÆâÉõÆþÂΤòǧ¤á¤¿ÀȼåX´ØÏ¢¿¶Àï/±¿Æ°¼ºÄ´¾É¸õ·²¤Î74ºÐÃËÀ­Îã
 1)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2)ÃÞÇÈÂç³Ø°å³Ø°åÎÅ·Ï¿À·ÐÆâ²Ê³Ø¡¤3)ÃÞÇÈÂç³Ø°å³Ø°åÎÅ·ÏÉÂÍý³Ø¡¤
 ±öëºÌ»Ò1)2)¡¤Àаæ°ì¹°2)¡¤º´Æ£ÂÙ¼ù3)¡¤Ìî¸ý²íÇ·3)¡¤¶Ì²¬¹¸2)¡¤óîƣʹ»Ò1)
 
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8ParkinsonɤÈÉû¿ÕÇò¼Á¥¸¥¹¥È¥í¥Õ¥£¡¼¤Î¹çÊ»Îã
 £±¡ËÅìµþ°å²ÊÂç³Øɱ¡¡¡¿À·ÐÆâ²Ê¡¡£²¡ËÅìµþ°å²ÊÂç³Øɱ¡¡¡Ê¬»ÒÉÂÍý³Ø¡¡£³¡ËÃÏÊýÆÈΩ¹ÔÀ¯Ë¡¿Í ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¦¿À·ÐÉÂÍý
 °æ¾å¡¡Ê¸£±¡Ë¡¢ÆâÌî¾´»Ò£³¡Ë¡¢µÆÌÌÀ£±¡Ë¡¢ÀмÍÎÊ¿£±¡Ë¡¢²ÃÆ£ÍÛµ×£±¡Ë¡¢³Ò¡¡´²Íº£±¡Ë¡¢ÁýÅÄâÃÇ·£±¡Ë¡¢ÃçÌð¾æͺ£²¡Ë¡¢¹õÅIJíɧ£²¡Ë¡¢Â¼»³ÈËͺ£³¡Ë¡¢Áêß·¿Î»Ö£±¡Ë
 

104 KNP

*Date¡§20130720
*Manager¡§Takahiro Fukuda

1An autopsy case that showed severe vacuolar changes in the white matter in the three months from radiation to the thoracic spine metastasis of prostate cancer.
 Tokyo metropolitan geriatric hospital¡¤1)Neuropathology¡¤2)Urology
 Hiroyuki Sumikura1), Takushi Nagata2), Yuta Nakano1), Akiko Uchino1), Akane Nogami1), Hiroyuki Hatsuta1), Masaki Takao1), Shigeo Murayama1)
 
2astrocytoma¤Èoligodendrocytoma¤ÎÀ®Ê¬¤ò´Þ¤ó¤ÀǾ¼ðá磳¾ÉÎã
 Div. of Neuropathol., Dept. of Pathol., The Jikei Univ. Schl. of Med.
 Fukuda T
 
3WernickeǾ¾É¤òÄ褷¤¿80ºÐÃËÀ­Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¸¦µæ½ê¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢2)¹ñºÝ°åÎÅÊ¡»ãÂç³Ø»°ÅÄɱ¡¡¡¿À·ÐÆâ²Ê
 Ìî¾å°«1)¡¢´äÅÄ¿®·Ã2)¡¢ÃæÌîͺÂÀ1)¡¢¶ù¢Â繬1)¡¢ÆâÌî¾´»Ò1)¡¢½éÅÄ͵¹¬1)¡¢¹âÈø¾»¼ù1)¡¢ÉðÅĹîɧ2)¡¢Â¼»³ÈËͺ1)
 
4Ìó16ǯ¤Î·Ð²á¤Çº¸±¦ÈóÂоÎÀ­¤Î¿Ê¹ÔÀ­¾å»è¶ÚÎÏÄã²¼¤òÄ褷¤¿°ìÎã
 ²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê¡¡1¡Ë¡¢Åìµþ»ü·Ã²ñ°å²ÊÂç³Ø¿À·ÐÆâ²Ê¡¡2¡Ë¡¢ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡3¡Ë
 Ë·Ìî·Ã»Ò2)¡¢º£Ê¡°ìϺ1)¡¢ÆâÌî¾´»Ò3)¡¢ÃæÌîͺÂÀ3) ¡¢Ìî¾å°«3) ¡¢½éÅÄ͵¹¬3) ¡¢¹âÈø¾»¼ù3) ¡¢Â¼»³ÈËͺ3)
 
5An autopsy case of 95-yo male with hippocampal sclerosis and NFTD
 1Dept. of Neurol., and 2Div. of Neuropathol., The Jikei Univ. Schl. of Med.
 Yamazaki M1, Sengoku R1, Iguchi Y1, Fukuda T2
 

103 KNP

*Date¡§20130223
*Manager¡§Noriyuki Shibata

1An autopsy case of primary central nervous system T-cell lymphoma
 Department of Pathology, Saitama Medical University International Medical Center
 Akiko Masaoka
 Primary T-cell lymphomas are quite rare in the CNS. Here, we report a case of primary CNS T-cell lymphoma that could be pathologically diagnosed in autopsy. A 40-year-old man was presented with right-sided hemiparesis. MRI revealed a lesion with ring-like enhancement in the left thalamus. The radiographic diagnosis was glioblastoma. The pathologic diagnosis of a brain biopsy was acute demyelinating lesion. The patient received steroid treatment. However, 3 months after the diagnosis, he died. At autopsy, a large, necrotic lesion in the bilateral cerebral hemisphere, focusing on the left thalamus, was noted. Histologically, perivascular infiltration of small- to medium-sized, atypical lymphoid cells was observed. The atypical lymphoid cells stained positive for CD3, CD8, granzyme B, perforin, but negative for CD4, CD20. T cell monoclonality was present in TCR gene rearrangement study by PCR. Thus, the final diagnosis was primary CNS T-cell lymphoma, peripheral T-cell lymphoma, unspecified. The present case indicates that the prognosis of primary CNS T-cell lymphoma might be fatal rapidly, and it might be difficult to determine the diagnosis in small biopsy specimen.
 
2Ë㿾Ǿ±êØí´µ¤«¤é±¦ÂçǾȾµå¤Î¹­ÈƤʾ㳲¤òÍ褷¡¢¸òºµÀ­¾®Ç¾°à½Ì¤òÄ褷¤¿°ìÎã
  1)µµÅÄÁí¹çɱ¡ ¿À·ÐÆâ²Ê¡¤2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯ ¡¤
 ³á À¿Ñ»1)¡¤ÊÒ¿ »ËÌÀ1)¡¤º´Æ£ ¿Ê1)¡¤¼Æ»³ ½¨Çî1)¡¤Ê¡Éð ÉÒÉ×1)¡¤°ËÆ£ ¿µ¼£2)¡¤Â¼»³ ÈËͺ2)
 
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 ¿·°æ¿®Î´1)4)¡¢±Ê°æÂÙµ±2)¡¢ÆîÆÁÌÀ2)¡¢±§ÅÄÉð¹°2)¡¢¿¹ÌîÆ»À²2)¡¢Ìø²¼¾Ï3)¡¢¾®¿¹Î´»Ê4)1)
 
4°­À­¿À·Ðç±¼ð¤ÈPNET¤¬º®ºß¤·¤¿À®¿ÍÂçǾȾµå¼ðáç¤Î1Îã
 1¡ËÅÔΩ¿À·Ðɱ¡ÉÂÍý¡¢2¡ËÅìµþ½÷»Ò°å²ÊÂç³ØǾ¿À·Ð³°²Ê¡¢3¡ËÈĶ¶Ãæ±ûÁí¹çɱ¡Ç¾¿À·Ð³°²Ê¡¢4¡ËÅìµþ½÷»Ò°åÂçÂè°ìÉÂÍý
 ¾®¿¹Î´»Ê1)4)¡¢¿·ÅIJíÇ·2)¡¢Â¼³ÀÁ±¹À2)¡¢´Ý»³Î´»Ö2)¡¢»°±ºÄ¾µ×3)¡¢ÁðÌîÎÉ3)¡¢¸üÃÏÀµ»Ò3)¡¢ºÙÌî½ã¿Î3)¡¢²¬ÅÄ˧ÏÂ2)¡¢ß·ÅÄãÃË4)¡¢¼ÆÅÄμ¹Ô4)
 
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 £±¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¢£²¡ËËÌΤÂç³Øɱ¡¿À·ÐÆâ²Ê
 ÆâÌî¾´»Ò£±¡Ë£²¡Ë¡¢²®ÌîÈþ·Ã»Ò£²¡Ë¡¢µÜÀ¿¥£²¡Ë¡¢À¾»³ÏÂÍø£²¡Ë¡¢¹âÈø¾»¼ù£±¡Ë¡¢Â¼»³ÈËͺ£±¡Ë¡¢
 
8Î×¾²Åª¤Ë¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼·¿Ç§Ãξɤ¬µ¿¤ï¤ì¤¿¡¢³¤ÇϹŲ½¾ÉÀ­Ç§ÃξɤÎ1˶¸¡Îã
 1) ÅìµþÂç³Ø°å³Ø·Ï¸¦µæ²ÊÉ°ø¡¦ÉÂÍý³ØÀ칶¡¡¿ÍÂÎÉÂÍý³Ø¡¦ÉÂÍý¿ÇÃdzء¤2) ÅìµþÂç³Ø°å³Ø·Ï¸¦µæ²ÊÀ¸¿£¡¦È¯Ã£¡¦²ÃÎð°å³ØÀ칶²ÃÎð°å³Ø¹ÖºÂ¡¡Ï·Ç¯É³ء¤3) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¤
 ÅÄÃæËãÍý»Ò1)¡¢ÃÓ¼²í»Ò1)¡¢¿·Ã«Íµ²Ã»Ò1)¡¢»³¸ýÂÙ¹°2)¡¢¿¼»³Àµµ×1)¡¢Â¼»³ÈËͺ3)
 
9Multiple system atrophy, accompanying swelling of medulla oblongata
 Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
 Yuko Hiroyoshi, Shinji Ito, Masaki Takao, Kazutomi Kanemaru, Aya Tokumaru, Shigeo Murayama
 
10ÀÔ¿ñ»ë¾²Ï©¤äÀÔ¿ñ¾®Ç¾Ï©¤ËÉÂÊѤòȼ¤Ã¤¿µåÀÔ¿ñÀ­¶Ú°à½Ì¾É¤Î51ºÐÃËÀ­Îã
 1¡Ë¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡ Î×¾²¸¡ººÉô¡¤2¡ËƱ ¿À·ÐÆâ²Ê¡¤3¡ËƱ Àº¿À²Ê¡¤4) Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼ Ãæ±û¸¡ººÉô
 ±öëºÌ»Ò1¡Ë¡¤ÀîźνÌé2¡Ë¡¤¶â°æ²í͵2¡Ë¡¤¿¹¤Þ¤É¤«2¡Ë¡¤¾®Àî²íʸ2¡Ë¡¤Â¼ÅÄÈþÊæ2¡Ë¡¤Í­ÇÏË®Àµ3¡Ë¡¤½ÂëÀ¿4¡Ë¡¤óîƣʹ»Ò1¡Ë
 

102 KNP

*Date¡§20121222
*Manager¡§Kinya Ishikawa

1¥Ñ¡¼¥­¥ó¥½¥ó¾É¾õ¤¬Àè¹Ô¤·¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤¬Â³È¯¤·¤¿68ºÐ½÷À­Îã
 1)ÆÁÅçÂç³Øɱ¡¿À·ÐÆâ²Ê¡¤2)¹áÀîÂç³Ø°å³ØÉô¿À·ÐÆñɹֺ¡¤3)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¡¡¡
 ÏÂÀôÍ£¿®1)¡¢Æ£ÅĹÀ»Ê1)¡¢¾®Àô±Ñµ®1)¡¢³ùÅÄÀµµª2)¡¢¶ù¢Â繬3)¡¢ ½éÅÄ͵¹¬3)¡¢Â¼»³ÈËͺ3)¡¢³áζѻ1)¡¤¡¡¡¤¡¤
 
2Neuropathological findings of a case with FTLD-TDP
 Department of Neurology, Department of Pathology, National Hospital Organization, Tokyo National Hospital
 Hitoshi Aizawa, Mie Nakamura, Harumi Shiroyama,¡¡Hiroshi Kurisaki, Akira Hebisawa
 A 58-year-old man developed difficulty in speaking. After 1 year, he visited our hospital. He had difficulty in naming and writing, and impaired executive function. At the age of 60, he showed echoralia, and continuation of using same items such as scissors. At the age of 61, he exhibited urinary incontinence, and selfish behavior. At the age of 63, He died of advanced lung cancer.The brain weighed 1,290g. Anterior part of temporal lobe was atrophic. There were numerous TDP-43 positive dystrophic neurites and a few cytoplasmic inclusions with neuronal loss and astrocytosis in the anterior part of temporal cortex. TDP-43 cytoplasmic inclusions were more prominent than dystrophic neurites in¡¤hippocampal dentate nucleus and neostriatum. Mild TDP-43 pathology was observed in the parietal and occipital cortices without neuronal loss or astrocytosis. TDP-43 pathology was observed more widely in the brain such as neostriatum, parietal and occipital cortices than anterior temporal cortex, suggesting TDP-43 pathology exceed neuronal loss and astrocytosis.
 
3Argyrophilic grain dsease clinically presenting with parkinsonim in the oledest old
 Fukushimura Brain Bank
 Hiroyasu Akatsu, Yoshio Hashidume
 
4¸ÆµÛÉÔÁ´¤Çȯ¾É¤·¤¿¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î°ìÎã
 ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê1¡Ë¡¤ÅìµþÅÔ·ò¹¯Ä¹¼÷¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯2¡Ë
 È¬ÌÚÀëÂÙ1¡Ë ¡¢¾®ÈøÃÒ°ì1¡Ë ¡¢¹ÓÌÚˮɧ1¡Ë ¡¢¿ù±ºÌÀ1¡Ë ¡¢»³ºê¸øÌé1¡Ë ¡¢ÆâÌî¾´»Ò2¡Ë¡¢½éÅÄ͵¹¬2¡Ë¡¢¹âÈø¾»¼ù2¡Ë¡¢Â¼»³ÈËͺ2¡Ë ¡¢¹Â¸ý¸ù°ì1¡Ë¡¤
 
5ÁжËÀ­´¶¾ð¾ã³²¤Î·Ð²áÃæ¤Ë¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¡¤¸¸³Ð¤¬½Ð¸½¤·¡¤Ë¶¸¡¤Ë¤Æ¥ì¥Ó¡¼¾®ÂÎÉÂÍý¤òÄ褷¤¿58ºÐÃËÀ­Îã
 1¡Ë¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡ Î×¾²¸¡ººÉô¡¤2¡ËÃÞÇÈÂç³Ø°å³Ø°åÎÅ·Ï¿À·ÐÆâ²Ê³Ø¡¤3¡Ë¹ñΩɱ¡µ¡¹½ÈîÁ°Àº¿À°åÎÅ¥»¥ó¥¿¡¼ ¡¡¡¤4¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤
 ±öëºÌ»Ò1¡Ë2¡Ë¡¤Ûº³Ùʸ3¡Ë¡¤¶Ì²¬¹¸2¡Ë¡¤Â¼»³ÈËͺ4¡Ë¡¤óîƣʹ»Ò1¡Ë
 
6¾ÇÅÀÀÚ½ü½Ñ¤ÏÁÕ¸ù¤»¤º¡¢±¦È¾µåÎ¥ÃǽѤˤè¤ê¤Æ¤ó¤«¤óȯºî¤¬¾Ã¼º¤·¤¿¡¤Rasmussen¾É¸õ·²¤Î6ºÐ½÷»ù¡£¡ÁÈé¼Á·ÁÀ®°Û¾ï¤Îʻ¸¡ÊFCD type IIIdÁêÅö¤ÎÉÂÍý¡Ë¤Î¡¤Í­Ìµ¤Ë¤Ä¤¤¤Æ¡Á¡¤
 1) ¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2) ¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¾®»ù¿À·Ð²Ê¡¤3) ¹ñΩÀº¿À¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Ç¾¿À·Ð³°²Ê¡¤4) ¿·³ãÂç³ØǾ¸¦µæ½êÉÂÂÖ¿À·Ð²Ê³ØÉôÌçÉÂÍý³ØʬÌî
 ßÀÊݱѼù 1)2)¡¢ËÜÅÄÎÃ»Ò 2)¡¢¿Ü³­¸¦»Ê 2)¡¢º´¡¹ÌÚÀ¬¹Ô 2)¡¢¹â¶¶¾ÏÉ× 3)¡¢ÂçÄÐÂÙ²ð 3)¡¢±öëºÌ»Ò 1)¡¢³ÁÅÄÌÀÈþ 4)¡¢óîƣʹ»Ò 1)
 
7¸òºµÀ­¾®Ç¾µ¡Ç½²òÎ¥¤Ë¤è¤ëÂЦ¾®Ç¾¤Î°à½Ì¡Êcrossed cerebellar atorophy)¤òǧ¤á¤¿ÊÒ¦ͥ°ÌáÛÚ»¸åǾ¾É¤Î°ìÎã
 1)µµÅÄÁí¹çɱ¡ ¿À·ÐÆâ²Ê¡¤2)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ³á À¿Ñ»1)¡¤ÊÒ¿ »ËÌÀ1)¡¤º´Æ£ ¿Ê1)¡¤¼Æ»³ ½¨Çî1)¡¤Ê¡Éð ÉÒÉ×1)¡¤°ËÆ£ ¿µ¼£2)¡¤Â¼»³ ÈËͺ2)
 
8Neuropathological study of an autopsy case of FALS- SOD1 A4D
 Departments of Neurology and Pathology, Postraduate School of Medicine, the University of Tokyo;¡¤Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Insitute of Gerontology
 Hiroya Naruse, Atsushi Iwata, Shoji Tsuji, Masako Ikemura, Masahisa Fuakayama, Shigeo Murayama
 
9¥°¥ê¥ª¡¼¥Þ¤Î°äÅÁ»Ò°Û¾ï
 Åìµþ°åÂç°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¿ÇÃÇÉô
 ß§Ã«¡¡À¿
 

101 KNP

*Date¡§20120728
*Manager¡§Hideo Mori

1çÁÂÓ·ì°Ü¿¢¸å¡¢GVHD¤ò¹çÊ»¤·¡¢ ÊÕ±ï·Ï¤Ë¸Â¶É¤·¤¿°Û¾ï¤òǧ¤á¤¿ HHV6´ØϢǾ±ê¤Î65ºÐ½÷À­Îã
 £±¡ËµµÅÄÁí¹çɱ¡¡¡¿À·ÐÆâ²Ê¡¤£²¡ËƱ¡¡·ì±ÕÆâ²Ê¡¤£³¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤¡¤
 ¹â¶¶ÀµÇ¯1),¡¡°ËÆ£¿µ¼£3), Ê¡ÉðÉÒÉ×1), À¾ÅÄÍ­µ£2).¡¡Â¼»³ÈËͺ 3)
 
2An autopsy case of a 70-year-old woman with encephalomyelitis after cord blood transplantation
 Department of Neuropahology 1), Pathology 2) and Hematology 3), Tokyo metropolitan Geriatric Hospital & Institute of Gerontology
 H Hatsuta 1)2), M Takao 1), A Uchino 1), H Sumikura 1), A Nogami 1), S Itoh 1), J Miyakoshi 3), T Arai 2), S Murayama 1)
 
3¸åº÷³Ë¤ÎÉÂÍý³ØŪÊѲ½¤òȼ¤Ã¤¿¼ãǯ·¿DRPLA¤Î49ºÐ½÷À­
 1. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¾®»ù¿À·Ð²Ê, 2. Åìµþ°å²ÊÂç³Ø°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼Î×¾²ÉÂÍý¡¤3. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤
 ã·Æ£¡¡µ®»Ö1¡¢¿Ü³­¡¡¸¦»Ê1¡¢º´¡¹ÌÚ¡¡À¬¹Ô1¡¢½Âë¡¡À¿2¡¢óîƣʹ»Ò
 
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 (1)½çŷƲÂç³ØǾ¿À·ÐÆâ²Ê¡¢(2)ÅÔΩ¾¾Âôɱ¡¿À·ÐÆâ²Ê¡¢(3)¿à»ÒÀÄÌÚɱ¡¡¢(4)½çŷƲÂç³ØÉÕ°±Ûëɱ¡Ç¾¿À·ÐÆâ²Ê
 ¹âÍü²í»Ë (1)¡¢½®ÊÕ¤µ¤ä¤« (1)¡¢°ÂÌî¤ß¤É¤ê (2)¡¢³ÑÌîÄ÷»Ò (3)¡¢¿¹¡¡½¨À¸ (4)
 
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7An autopsy case of 80-y.o. Japanese-Brazilian man, presenting conscious disturbance, leukoencephalopathy, and megacolon
 1) Metabolic and General medicine, Fuji City General Hospital, 2) Pathology and 3) Neuropathology, The Jikei University School of Medicine
 Shiozaki M1, Iuchi H1, Yamashiro H1, Fujii T1, Chiba S2, Fukuda T3¡¤
 
8FUSÍÛÀ­¤Î¹¥±ö´ðÀ­ÉõÆþÂΤòǧ¤á¤¿ALS¤Î62ºÐ½÷À­¤Î˶¸¡Îã
 1) ²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê 2) ƱÉÂÍý¿ÇÃÇ²Ê 3)ÅìµþÂç³Ø°å³ØÉôÉí°É±¡¿À·ÐÆâ²Ê 4) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤¡¤
 ÆÁ½Å¿¿°ì1) 3), º´¡¹ÌÚÂóÌé1), º£Ê¡°ìϺ1), ³ÑÅĹ¬Íº2), ÈîÅĤ¢¤æ¤ß3), À¶¿å½á3), ÄԾʼ¡3),°ËÆ£¿µ¼£4)¡¤¶ù¢Â繬4), Ìî¾å°«4)¡¤ÆâÌî¾´»Ò4)¡¤½éÅÄ͵¹¬4)¡¤¹âÈø¾»¼ù4)¡¤Â¼»³ÈËͺ4)¡¤
 
9Á´·Ð²á5ǯ¤Ç»àË´¤·¤¿²¼¿âÂθåÍÕ¥ê¥ó»À²½¦Á¥·¥Ì¥¯¥ì¥¤¥óÃßÀѤòȼ¤ï¤Ê¤¤ParkinsonÉ£·£¸ºÐÃËÀ­Îã
 1) ÅÔΩ¿À·Ðɱ¡ ¸¡ºº²Ê ÉÂÍý(¿À·ÐÉÂÍý) 2) Ʊ Ǿ¿À·ÐÆâ²Ê¡¤3) ºë¶Ì°å²ÊÂç³Ø ÉÂÍý³Ø¡¤4) ÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤5) ÅÔΩÉÜÃæÎŰ饻¥ó¥¿¡¼
 Taku Homma 1,3), Ryoichi Okiyama 2), Yoko Mochizuki 1,4), Toshio Mizutani 1,5), Takashi Komori 1), Shirou Matubara 2)
 
10Î×¾²Åª¤ËÁ°Æ¬Â¦Æ¬·¿Ç§ÃξÉ(FTD)/¶Ú°à½ÌÀ­Â¦ºö¹Å²½¾É¤òȼ¤¦FTD (ALSD/MND with FTD)¤¬µ¿¤ï¤ì¤¿58ºÐÃËÀ­
 Ê¡»ã¼É±¡¿À·ÐÉÂÍý¸¦µæ½ê£±¡Ë¡¢°¦Ãΰå²ÊÂç³Ø²ÃÎð°å³Ø¸¦µæ½ê¡¡¿À·ÐÉÂÍýÉôÌ磲¡Ë¡¢Ì¾¸Å²°»ÔÁí¹ç¥ê¥Ï¥Ó¥ê¥Æ¡¼¥·¥ç¥ó¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê£³¡Ë¡¢ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯£´¡Ë
 ÀÖÄÅ͵¹¯£±¡Ë¡¢£²¡Ë¡¢ËÙËܲÂɧ£³¡Ë¡¢µÈÅÄâÃÍý£²¡Ë¡¢Â¼»³ÈËͺ£´¡Ë¡¢¶¶µÍÎÉÉ×£±¡Ë
 

100 KNP

*Date¡§20120324
*Manager¡§Takahiro Fukuda

1SOD1ÊÑ°Û¤òȼ¤¦²È²À­¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É(ALS1)¤Î°ìÎã
 1) Department of Neurology, Shizuoka Institute of Epilepsy and Neurological Disorders¡¤2) Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
 Nobuyasu Yagi, Tomokazu Obi, Akira Sugiura, Kinya Yamazaki, Shigeo Murayama, Kouichi Mizoguchi
 
2¶¶³Ë¤Î¿À·ÐºÙ˦¤âæÍ¡¢FUSÍÛÀ­¹½Â¤Êª¤¬¹­ÈϰϤÊÊÑÀ­Éô°Ì¤Ë½Ð¸½¤·¤¿¤¬¡¢FUS/TLS°äÅÁ»ÒÊÑ°Û¤¬Ç§¤á¤é¤ì¤Ê¤«¤Ã¤¿²È²À­ALS¤Î£±Îã
 ÅÔΩ¿À·Ðɱ¡¡¡£±¡Ë¸¡ºº²ÊÉÂÍý¡¢£²¡ËǾ¿À·ÐÆâ²Ê¡£¡¤ÅÔ°å³Ø¸¦¡¡£³¡ËÅý¹ç¼ºÄ´¡¦¤¦¤ÄÉÂ¥×¥í¥¸¥§¥¯¥È¡¢¡¡£´¡Ë¿À·ÐÊÑÀ­ÉÂÍý¥×¥í¥¸¥§¥¯¥È¡¢£µ¡ËǧÃÎ¾É¥×¥í¥¸¥§¥¯¥È¡¢£¶¡Ë¹­ÅçÂ縶ÇúÊü¼ÍÀþ°å³Ø¸¦µæ½êʬ»Ò±Ö³Ø¸¦µæʬÌî¡¢£·¡ËÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¢£¸¡Ëºë¶Ì°åÂçÉÂÍý¡¢£¹¡ËÅÔΩÉÜÃæÎŰ饻¥ó¥¿¡¼
 Mochizuki Y 1,7),
 
3ÃøÌÀ¤Ê¿À·ÐºÙ˦æÍî¤ÈFUSÍÛÀ­¹½Â¤Êª¤ò¹­ÈϰϤËǧ¤á¤¿FTLD¤ª¤è¤ÓALS¤Î¹çÊ»Îã
 1)ÅìÂçɱ¡¿À·ÐÆâ²Ê¡¤2)ÅìÂçɱ¡ÉÂÍýÉô¡¤3)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 Ã渵¤Õ¤ß»Ò1)¡¢¾¾Àî·É»Ö1)¡¢ÎÓ½Ó¹¨1)¡¢À¶¿å½á1)¡¢Á°ÅÄÂçÃÏ2)¡¢ÃÓ¼²í»Ò2)¡¤°ËÆ£¿µ¼£2)3)¡¢¹âÈø¾»¼ù3)¡¢Â¼»³ÈËͺ3)¡¤ÄԾʼ¡1)
 
4autopsy case of Leigh encephalopathy with a mutation of Mt8993T>G
 1) Division of Child Neurology, 2) Division of Pathology, Kanagawa Children's Medical Center
 Akiko Oshiro1), Mitsuko Okuda1), Kyouko Takano1), Takahito Wada1), Mizue Iai1), Hitosi Osaka1), Sumimasa Yamashita1), Yukichi Tanaka2)
 
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 ·²ÇÏÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÉÂÂÖÉÂÍý³ØʬÌî
 ÃæΤÍΰì
 
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 1)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½ê ǧÃÎ¾É¥×¥í¥¸¥§¥¯¥È¡¢2)²£ÉÍ»ÔΩÂç³Ø°å³ØÉôÀº¿À°å³Ø¡¢3)ÅìµþÅÔΩ¾¾Âôɱ¡Àº¿À²Ê
 ²Ï¾å½ï1,2)¡¢ÂçÅç·ò°ì1,3)¡¢¿·Î¤Ï¹°1,3)¡¢Å쿸Æó1)¡¢ÀÄÌÚľºÈ1,2)¡¢¾¡À¥Â糤2)¡¢ÅԹÿò2)¡¢¿·°æůÌÀ1)¡¢Ê¿°ÂÎÉͺ2)¡¢½©»³¼£É§1)
 
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 £±¡ËÊ¡»ã¼É±¡¿À·ÐÉÂÍý¸¦µæ½ê¡¤£²¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤£³¡Ë°¦ÃΰåÂç²ÃÎð°å³Ø¸¦µæ½ê¡¤
 ÀÖÄÅ͵¹¯£±¡Ë¡¢Â¼»³ÈËͺ£²¡Ë¡¢µÈÅÄâÃÍý£³¡Ë¡¢¶¶µÍÎÉÉ×£±¡Ë
 
8II·¿¸ÆµÛÉÔÁ´¤Ç»àË´¤·¡¤Á°³ÑºÙ˦¤ËTDP43ÃßÀѤòǧ¤á¤¿¡¤·Ð²á8ǯ¤ÎÂçǾÈé¼Á´ðÄì³ËÊÑÀ­¾É¤Î63ºÐ½÷À­Îã¡¥
 *²£ÉÍÏ«ºÒɱ¡¡¡¿À·ÐÆâ²Ê¡¤**ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¸¦µæ½ê¡¦Ï·Ç¯ÉÂÍý³Ø¸¦µæ¥Á¡¼¥à¡¦¿À·ÐÉÂÍý³Ø¡¡¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ±óÆ£¡¡¹À¿®*¡¤Ã滳¡¡µ®Çî*¡¤½®ÊÕ¡¡¤µ¤ä¤«**¡¤º£Ê¡¡¡°ìϺ*¡¤Â¼»³¡¡ÈËͺ**¡¤
 
9È¿Éü¾ïƱ¹ÔÆ° (punding)¤òǧ¤á¡¤Ç®Ãæ¾É¤Ë¤Æ»àË´¤·¤¿¥Ñ¡¼¥­¥ó¥½¥óÉÂYahr 2ÅÙ¤Î59ºÐÃËÀ­Îã
 £±¡Ë¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¿À·ÐÆâ²Ê¡¤£²¡Ë·¼¿®²ñµþÅԻ;òɱ¡¥Ñ¡¼¥­¥ó¥½¥óÉ¡¦¿À·ÐÆñÉÂ¥»¥ó¥¿¡¼¡¤£³¡Ë¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô
 ¡ûÃÓÅĸ¬Ê壱¡Ë¡¤¸Åß·²Åɧ£±¡Ë¡¤µ×ÌîÄç»Ò£²¡Ë¡¤Â¼ÅÄÈþÊ棱¡Ë¡¤óîƣʹ»Ò£³¡Ë
 
10¤Æ¤ó¤«¤ó³°²ÊÉÂÍý¿ÇÃÇ¥Õ¥©¡¼¥é¥à¡Ê£²¡Ë:¡¤¸Â¶ÉÀ­Èé¼Á°Û·ÁÀ®¤ÈÊÒ¦µðǾ¾É¤Î»ÐÄïÎã
 1) ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡¤Æ¤ó¤«¤ó¥»¥ó¥¿¡¼¡¢2) ¿·³ãÂç³ØǾ¸¦¡¡¿À·ÐÉÂÍý3) °Â¾ë¹¹À¸É±¡¡¡¾®»ù²Ê
 óîƣʹ»Ò1)¡¢³ÁÅÄÌÀÈþ2)¡¢´Ý»³¿µ²ð1)¡¢ËÜÅÄÎûÒ1)¡¢°ËÆ£²íÇ·1)¡¢¿¼ÂôãÌé3)¡¢µ×ÊÝÅÄůÉ×3)¡¢¡¡º¬Íè̱»Ò3)¡¢¿Ü³­¸¦»Ê1)¡¢ÂçÄÐÂÙ²ð1)
 
11Neuropathology of Demyelinating disorders
 Neuropathology, UNC
 Kinuko Suzuki, MD
 Myelin is a membranous structure formed by an extended and modified plasma membrane of the myelin forming cells; oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS). A predominant component of the white matter in the CNS is the myelin and thus, diseases affecting myelin are diseases, largely involving the white matter of the brain. The molecular composition of the CNS myelin differs significantly from that of the PNS. Therefore, most acquired myelin disorders affecting the CNS myelin do not involve the PNS. For the histological study of the myelin diseases, many myelin specific stains are necessary, since with Hematoxylin and Eosin stain, myelin appears pink or lightly eosinophilic and myelin pathology may not be easily appreciated¡¤Myelin disorders are defined as disorders caused by the degeneration of myelin sheath, either as a primary event or as a result of the degeneration or dysfunction of the myelin forming cells. Traditionally, myelin disorders are classified into two large groups: demyelinating disorders (acquired myelin diseases) and leukodystrophies (genetic myelin disease). Demyelinating disorders are further sub classified into immune-mediated (or inflammatory), viral and toxic type of demyelination. ¡¤Today¡Çs talk is focused on the pathology of the demyelinating disorders and review of the recent progress in the investigation on the pathogenesis of these disorders.¡¤ ¡¤References¡¤¡¤Lucchinetti, et.al. Heterogeneity¡¡of¡¡multiple¡¡sclerosis¡¡lesion:¡¡implication¡¡for¡¡the¡¡pathogenesis¡¡of¡¡demyelination.¡¡Ann Neurol 2000; 47:707-717¡¤Barnett and Prineas. Relapsing and remitting multiple sclerosis: pathology of newly formed lesion. Ann Neurol 2004; 55:458-466¡¤Lucchinetti, et.al. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 2008:131:1759-1775¡¤Lucchinetti, et. al. Inflammatory cortical demyelination in early multiple sclerosis. NEJM 2011; 365: 2188-2197¡¤Lennon, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004; 364:2106-2112.¡¤Misu et al.Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. Brain 2007; 130: 1224-1234¡¤Misu T Pathology of neuromyelitis optica¡¥Rinsho Shinkeigaku 2009 49:896-869¡¤Roemer, et.al.¡¢Pattern specific loss of aquaporin-£´ immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain 2007; 130:1194-1205.¡¤Kleinschmidt-DeMasters and Tayler, Progressive multifocal leukoencephalopathy complicating treatment with natalizumad and interferon beta-Ia for multiple sclerosis. NEJM 2005;. 353:369-374.¡¤Langer-Goud, et. al., Progressive multifocal leukoencephalopathy in a patient treated with natalizamab. NEJM 2005;:353:375-381¡¤Kelleher et al., Progressive multifocal leukoencephalopathy in a patient with Alzheimer disease. Diag Mol Path 1994; 3:105-113.¡¤
 

99 KNP

*Date¡§20120107
*Manager¡§Noriyuki Shibata

1¹âÅÙ¤ÊÇò¼ÁÉÂÊѤòȼ¤Ã¤¿µª°Ë¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¡¦¥Ñ¡¼¥­¥ó¥½¥óǧÃξÉÊ£¹ç¤Î°ì˶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)»°½ÅÂç³Ø°å³ØÉô¿À·ÐÆâ²Ê¡¤3)Î뼯°åÎŲʳØÂç³Ø ÊÝ·ò±ÒÀ¸³ØÉô °åÎÅÊ¡»ã³Ø²Ê
 ¿¹Ëܸç1),½éÅÄ͵¹¬1),¾®µ×Êݹ¯¾»2),³ë¸¶Ìмù3),¼»³ÈËͺ1)
 
2¿Ê¹ÔÀ­ÈóήĪÀ¸¼º¸ì¤òÄ褷¤¿ÂçǾÈé¼Á´ðÄì³ËÊÑÀ­¾É¤Î87ºÐ½÷À­Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢2)ƱÉÂÍý¿ÇÃDzʡ¢3)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½êǧÃÎ¾É¥×¥í¥¸¥§¥¯¥ÈÉÂÂÖºÙ˦À¸Êª³Ø¸¦µæ¼¼
 Ìî¾å°«1)¡¢¹âÈø¾»¼ù1)¡¢°ËÆ£¿µ¼£1)¡¢½®ÊÕ¤µ¤ä¤«1)¡¢½éÅÄ͵¹¬1)¡¢¿·°æÉÚÀ¸2)¡¢Ä¹Ã«ÀîÀ®¿Í3)¡¢Â¼»³ÈËͺ1)
 
3Points of intraoperative consultation of central nervous system lesions.
 Department of Pathology, Saitama Medical University, Saitama
 Atsushi Sasaki
 
4¤Æ¤ó¤«¤ó³°²ÊÉÂÍý¿ÇÃÇ¥Õ¥©¡¼¥é¥à
 1)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½ê¡¦Ç¾ÉÂÍýɸËܥꥵ¡¼¥Á¥»¥ó¥¿¡¼¡¤2)¿·³ãÂç³ØǾ¸¦µæ½ê¡¦À¸Ì¿²Ê³Ø¥ê¥½¡¼¥¹¸¦µæ¥»¥ó¥¿¡¼¡¤3)½©Åĸ©Î©Ç¾·ì´É¸¦µæ¥»¥ó¥¿¡¼¡¦Ç¾¿À·ÐÉÂÍý³Ø¡¤4)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼¡¦Î×¾²¸¡ººÉô¡¤5)ÅìµþÅÔΩ¿À·Ðɱ¡¡¦¸¡ºº²Ê
 ¿·°æ¿®Î´1)¡¢³ÁÅÄÌÀÈþ2)¡¢µÜÅĸµ3)¡¢óîƣʹ»Ò4)¡¢¾®¿¹Î´»Ê5)
 
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10Èæ³ÓŪ¹­ÈϰϤËPickµå¤Î¹­¤¬¤ê¤òǧ¤á¤¿PickɤΰìÎã
 1)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¤2)¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Àº¿À²Ê¡¤3)Åìµþ°å²ÊÂç³Ø°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý³Ø¡¤4)ÅìµþÅÔ°å³ØÁí¹ç¸¦µæ½êǧÃÎ¾É¥×¥í¥¸¥§¥¯¥ÈÉÂÂÖºÙ˦À¸Êª³Ø¸¦µæ¼¼ ¡¤
 Shioya A1)¡¤Arima K2)¡¤Ikeda K1)¡¤Shibuya M3)¡¤Hasegawa M4)¡¤Saito Y1)
 

98 KNP

*Date¡§20110806
*Manager¡§Kinya Ishikawa

1Æñ¼£À­¤Æ¤ó¤«¤ó¤òÄ褷¤¿angiocentric glioma¤Î1Îã
 Åìµþ°å²Ê»õ²ÊÂç³Ø¡¡Ç¾¿À·Ð³°²Ê1)¡¢ÉÂÍýÉô2)¡¢¤Ê¤á¤¬¤¿ÃÏ°èÁí¹çɱ¡3)
 ¾®È¨²Âµ±1)¡¢Á°¸¶·ò¼÷1)¡¢¾®ÎÓÂçÊå2)¡¢ÀÄÌø·æ1)¡¢¹õ´äÉÒɧ3)¡¢ÂçÌî´îµ×Ϻ1)¡¢
 
2STN-DBS»Ü¹Ô¸å¤ËlevodopaÈ¿±þÀ­MSA-P¤ÈȽÌÀ¤·¤¿71ºÐÃËÀ­Îã
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 Æ⻳ÃÒÇ·1)¡¢ËÒÌîδ¹¨1)¡¢¾®Åç°ìÊâ1)¡¢ÛÆÅÄÂçÀ®1)¡¢¶â°æ¿ôÌÀ1)¡¢Èõ¸ý²Â§2)¡¢ÀÄÌøµþ»Ò2)¡¢ÊÆÀ¹ÍÕ»Ò3) ¡¢Âç½Ðµ®Éð3) ¡¢Ãæë¹Ôͺ3)¡¢¿·°æ¸ø¿Í1)¡¢·¬¸¶¡¡Áï1)
 
3¿Ê¹ÔÀ­¤ÎǧÃΡ¦±¿Æ°¾ã³²¤òÄ褷¡¤¹­ÈϤÊÂçǾÇò¼ÁÉÂÊѤò¼¨¤·¤¿°ì˶¸¡Îã
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4Èé¼Á²¼¹¼ºÉ¤ÈÇò¼ÁǾ¾É¤òȼ¤¦¾ïÀ÷¿§ÂÎÎôÀ­Ç¾Æ°Ì®¾É(CARASIL)¤Î54ºÐÃËÀ­Ë¶¸¡Îã
 1) ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢2) Ê¡»ã¼É±¡¿À·ÐÆâ²Ê¡¢3) ¼¯ÅçÏ«ºÒɱ¡¿À·ÐÆâ²Ê¡¢4)µµÅÄ¥á¥Ç¥£¥«¥ë¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê
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 Ìî¾å°«1)¡¢ÄÇ̾ÌÁ»Ò2)¡¢¹âÈø¾»¼ù1)¡¢°ËÆ£¿µ¼£1)¡¢½®ÊÕ¤µ¤ä¤«1)¡¢½éÅÄ͵¹¬1)¡¢ÂôÊÕ¸µ»Ê3)¡¢Â¼»³ÈËͺ1)
 
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 ²Ï¾å½ï1,2)¡¢¾¡À¥Â糤1)¡¢ÀÄÌÚľºÈ1,2)¡¢Å쿸Æó2)¡¢ÎëÌÚµþ»Ò1)¡¢ÅԹÿò1)¡¢¿·°æ¿®Î´3)¡¢½©»³¼£É§2)¡¢Ê¿°ÂÎÉͺ1)¡¤
 
7¡Ö°×žÅÝÀ­¡¢È¯Ïäθº¾¯¤Çȯ¾É¤·¡¢¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤È¿ÇÃǤµ¤ì¤Æ¤¤¤¿78ºÐÃËÀ­Îã¡×
 1. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡Î×¾²¸¡ººÉô¡¢2. ¼«¼£°å²ÊÂç³ØÉí°É±¡¤µ¤¤¤¿¤Þ°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¢3. ¸×¥ÎÌçɱ¡Ê¬±¡¡¡¿À·ÐÆâ²Ê¡¢4.ÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢5. Åìµþ°åÂç°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¡¢8. ¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Àº¿À²Ê
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8ÎöǾ¾É¡¢Dandy-Walker¾É¸õ·²¤Î°ì˶¸¡Îã
 1) ÅìµþÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÉ°øÉÂÍý³ØÀ칶¿ÍÂÎÉÂÍý³Ø¡¦ÉÂÍý¿ÇÃdzØʬÌ2) ÅìµþÂç³Ø°å³ØÉôÉհɱ¡ÉÂÍýÉô¡¤3)Ʊ¾®»ù²Ê¡¤4)ÅìµþÂç³ØÂç³Ø±¡ °å³Ø·Ï¸¦µæ²Ê¹ñºÝÊÝ·ò³ØÀ칶 ȯã°å²Ê³ØʬÌ5)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ¿¹ÅÄÌмù1)¡¢ÃÓ¼²í»Ò2)¡¢°Â°æ¹§ÆóϺ3)¡¢¿å¸ý²í4)¡¢Â¼»³ÈËͺ5)¡¢¿¼»³Àµµ×1,2).
 

97 KNP

*Date¡§20101204
*Manager¡§Hideo Mori

1Áؾõ¤Îæ¿ñ¤òÄ褷¤¿43ºÐÃËÀ­Îã
 1)Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerountology (TMGHIG), Tokyo, Japan¡¤2)Department of Pathology, TMGHIG¡¤3) Department of Neurology, TMGHIG, 4) Department of Radiology, TMGHIG
 H Hatsuta 1)2), Y Hiroyoshi 3), K Kanemaru 3), S Ito 1), M Sugiyama 1), S Funabe 1), M Takao 1), T Arai 2), A Tokumaru 4), K Suzuki 1) and S Murayama 1)
 
2¿Ê¹ÔÀ­ÈóήĪÀ­¼º¸ì¤òÄ褷¤¿TDP-43ÍÛÀ­ÉõÆþÂΤòȼ¤¦Á°Æ¬Â¦Æ¬ÍÕÊÑÀ­¾É¤Î£±Îã
 1)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¡Ï·Ç¯´üÀº¿À¼À´µ¸¦µæ¥Á¡¼¥à2)²£ÉÍ»ÔΩÂç³Ø°å³ØÉôÀº¿À°å³Ø¶µ¼¼
 1)2)ÀÄÌÚľºÈ¡¢1)ÅÚëˮ½©¡¢1)¾®ÎÓÁµ¡¢£±)¿·°æůÌÀ¡¢2)ÅԹÿò¡¢2)¾¡À¥Â糤¡¢£²)ÆâÌçÂç¾æ¡¢2)²Ï¾å½ï¡¢1)½©»³¼£É§¡¢2)Ê¿°ÂÎÉͺ
 
3A case of tumefactive multiple sclerosis, initially diagnosed as astrocytoma
 Second Department of Internal Medicine, Division of Neurology, University of Fukui¡¤Department of Neuropathology, and Brain Bank for Aging Research, Tokyo Metropolitan Geriatric Hospital and Gerontology¡¤Department of Neuropathology, North Carolina University
 Nobuhiko Ohashi 1, Ryo Nakachi 1, Masamichi Ikawa 1, Toru Kishitani 1, Tadanori Hamano 1, Makoto Yoneda 1,Shinji Ito 2, Shigeo Murayama 2, Kinuko Suzuki 3
 A 37-years-old woman presented disturbance of speech, psychiatric symptoms, and difficulty in moving upper and lower limbs. She was initially diagnosed as dissociative disorder. MRI study showed mass lesion accompanying edema with Gd-enhancement in the left frontal lobe. She underwent brain biopsy. Hisologically, atypical glial cells were observed. So, she was diagnosed as low grade astrocytoma. She was planned to undergo operation. Before operation, she presented tonic seizure. At that time, MRI study showed size reduction of the lesion. So, the possibility of multiple sclerosis (MS) was suspected out, and operation was not performed. One year later, she presented right visual disturbances. As the MRI study showed Gd-enhancement of right optic nerve, she was diagnosed as optic neuritis. Two years later, she presented dysarthria, and truncal ataxia. Follow up brain MRI disclosed mass lesion accompanying edema in the right cerebellum. After steroid pulse therapy, the size of the lesion got smaller, and the degree of enhancement was also attenuated. Initially, it was not easy to rule out the possibility of brain tumor. But, she was finally diagnosed as MS by clinical course, neuroradiological findings, and therapeutic effect of steroid. Histopathologically, myelin stain was re-evaluated¡¡to detect demyelinating lesions. We will discuss the correlation of clinical courses, neuroradiological findings and neuropathological findings.
 
4Autopsy findings of Sanfilippo syndrome type A
 1)Kanagawa Children's Medical Center, Institute of Severe Motor and Intelligent Disabilities, 2)Dept. of Pathology¡¤
 1)Sumimasa Yamashita, Michiko Yamada,2)Yukici Tanaka, Yoshiro Sasaki
 
5¶µ°é¹Ö±é
Application of molecular markers in diagnostic pathology of gliomas
 Department of Pathology, Saitama Medical University
 Atsushi Sasaki
 ¶áǯ¤ÎǾ¼ðáç°äÅÁ»Ò²òÀϤ«¤éǾ¼ðá硤Æä˥°¥ê¥ª¡¼¥Þ¤Î¿ÇÃÇ¡¦¼£ÎŤ˽ÅÍפǤ¢¤ë¤³¤È¤¬È½ÌÀ¤·¤¿Ê¬»Ò¥Þ¡¼¥«¡¼¤¬¤¢¤ë¡¥º£²ó¤Ï¡¤¤½¤ì¤é¤Î¥Þ¡¼¥«¡¼¤ÎÃ椫¤é¡¤O6-methyl-guanine-methyltransferase (O6-MGMT)¤Èisocitrate dehydrogenase (IDH)1¤ò¼è¤ê¾å¤²¡¤¿ÇÃÇÉÂÍý±þÍѤǤÎÍ­¸úÀ­¡¤ÌäÂêÅÀ¤ò½Ò¤Ù¤¿¤¤¡¥
 
6¾å°ÌÍ¥°Ì¤Î±¿Æ°¥Ë¥å¡¼¥í¥óÊÑÀ­¤ò¹âÅÙ¤Ëǧ¤á¤¿Èé¼Á´ðÄì³ËÊÑÀ­¾É¤Î74ºÐ½÷À­
 1.¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Î×¾²¸¡ººÉô¡¤2.Åìµþ°å²ÊÂç³Ø°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¿ÇÃÇÉô¡¤3.¹ñΩÀº¿À¡¦¿À·Ð°åΟ¦µæ¥»¥ó¥¿¡¼É±¡¡¡Àº¿À²Ê¡¤4.½ÕÆüÉôÁí¹çɱ¡¡¡Æâ²Ê¡¤5.ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¡Êü¼ÍÀþ²Ê
 óîƣʹ»Ò1¡¢½ÂëÀ¿1¡¢2¡¢Æ£°æ¤µ¤ä¤«3¡¢¸Í°æ§É×4¡¢ùþ´Ý°¤Ìí5, Í­ÇÏË®Àµ3
 
7An autopsy case of 37 y.o. male with Dejerin syndrome
 1 Neurology and 2Neuropathology, The Jikei Univ. Schl. of Med.
 Umehara A1, Kinosita K1, Sengoku R1, Kouno Y1, Morita M1, Mochio S1, Fukuda T2
 
8MRIT2¶¯Ä´²èÁü¤ÇÂçǾÇò¼Á¤Ë¹­ÈϰϤ˹⿮¹æ°è¤ò¼¨¤·¡¢ÉÂÍý¤Ç¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼É¤òÄ褷¤¿73ºÐ½÷À­
 ½çŷƲÂç³ØǾ¿À·ÐÆâ²Ê¡¡
 ½®ÊÕ¤µ¤ä¤«
 

96 KNP

*Date¡§20100717
*Manager¡§Takahiro Fukuda

1Tumefactiveº¸Á°Æ¬ÍÕÉÂÊѤΰìÎã
 ÅìËÌÂç³Ø¿À·ÐÆâ²Ê¡¤ÅìËÌÂç³ØÉÂÍýÉô
 ²ÃÆ£Î̹­, °æÀô ÎÜÈþ»Ò, ¿ûÌîľ¿Í, ĹëÀî δʸ, ÃæÅç°ìϺ, ÉðÅÄ ÆÆ, Æ£¸¶°ìÃË, ÅÏÊդߤ«
 
2¥¢¥À¥ê¥à¥Þ¥ÖÅêÍ¿¸å¤Ë¼ðáçÍÍÃæ¿õÀ­Ã¦¿ñÀ­ÉÂÊѤòǧ¤á¤¿72ºÐ½÷À­Îã
 Åìµþ°å²Ê»õ²ÊÂç³Ø°å³ØÉôÉհɱ¡¿À·ÐÆâ²Ê
 °¤Éô·½Êå
 
3Ǿɽ¥Ø¥â¥·¥Ç¥í¡¼¥·¥¹¡Ê¥Ø¥â¥·¥Ç¥ê¥óÄÀÃå¾É¡Ë¤Î1˶¸¡Îã
 ºâÃÄË¡¿ÍǾ·ì´É¸¦µæ½ê¡¡Èþ¸¶µ­Ç°É±¡¡¡¿À·ÐÆâ²Ê¡¤¿À·ÐÆñÉ¡¦Ç§ÃξÉÉôÌç
 ¹âÈø¾»¼ù¡¤ÌÚ¼¹À¹¸¡¤Èþ¸¶¡¡Èס¤µÈÅÄÍÎÆó
 
4Minimal cytological changes of progressive supranuclear palsy (PSP) type in clinically possible PSP
 Department of Neurology, Nakano Sogo Hospital 1, Department of Neurology, Tokyo Metropolitan Inst for Neurosci 2, Department of Pathology, Tokyo Medical and Dental University 3
 Toru S 1, Uchihara T 2, Akashi T 3, Kobayashi T 1
 69-yo male patient died of aspiration pneumonia after two years of clinical course resembling PSP. At autopsy, a very small number of tau-positive glial lesions were identified in restricted parts of the brain, where similar lesions appear in full-blown PSP. Quality rather than quantiity of lesions represents neuropathology of PSP.
 
5Neuropathology of epilepsy - Sturge-Weber syndrome -
 Epilepsy Pathology Center, Tokyo Metropolitan Institute for Neuroscience
 Nobutaka ARAI1)2), Akira YAGISHITA3), Shigeki SUNAGA4)
 
6¿¿©¡¢¼«È¯À­Äã²¼¡¢¹ÔÆ°°Û¾ï¤¬¤ß¤é¤ì¡¢Á´·Ð²áÌó3ǯ4¥ö·î¤ÇµÞ»à¤·¤¿FTLD-FUS¤Î»àË´»þ47ºÐ½÷À­Îã
 1)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¡Ï·Ç¯´üÀº¿À¼À´µ¸¦µæ¥Á¡¼¥à¡¢2)Åìµþ°å²Ê»õ²ÊÂç³ØÂç³Ø±¡¡¡Ç¾¿À·ÐÉÂÂֳء¢3)ÃÞÇÈÂç³ØÂç³Ø±¡¡¡¿Í´ÖÁí¹ç²Ê³Ø¸¦µæ²ÊÀº¿ÀÉÂÂÖ°å³Ø¡¤
 1)2)¾®ÎÓÁµ¡¢1)½©»³¼£É§¡¢1)ÅÚëˮ½©¡¢1)¶áÆ£¤Ò¤í¤ß¡¢1)3)¿·°æůÌÀ¡¢2)¿åß·±ÑÍÎ
 
7Case of 77 y.o. male with motor neuron disease and glioneuronal intranuclear polyglutamin inclusion
 Neurology and Neuropathology, The Jikei University School of Medicine
 Sengoku R, Kouno Y, Suzuki K, Fukuda T, Mochio S
 
8<³°²ÊÉÂÍý¾ÉÎã¡ä¡¡Â¦Æ¬ÍÕÆâ¦ÉôǾ±ê¤È¹Í¤¨¤é¤ì¤ë°ìÎã
 Åìµþ°å²ÊÂç³Ø¡¡°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¿ÇÃÇÉô¡¤Åìµþ°å²ÊÂç³Ø¡¡Ç¾¿À·Ð³°²Ê¡¥Åìµþ°å²ÊÂç³Ø¡¡¿ÍÂÎÉÂÍý³Ø¡¤
 ß§Ã«¡¡À¿¡¤½©¸µ¼£Ï¯¡¤Ä¹Èø½Ó¹§
 
9Æá¿Ü-HakolaɤÎ1˶¸¡Îã
 ²£ÉÍ»ÔΩÂç³Ø°å³ØÉôÀº¿À°å³Ø¶µ¼¼
 ÀÄÌÚľºÈ¡¢¾¡À¥Â糤¡¢²Ï¾å½ï¡¢ÎëÌÚµþ»Ò¡¢ÆâÌçÂç¾æ¡¢ÅԹÿò
 
10LRRK2¤Ë¤Þ¤ì¤ÊSNPÊÑ°Û¤òǧ¤á¡¢²È²À­¤¬µ¿¤ï¤ì¤¿Lewy¾®ÂΤòȼ¤¦¥Ñ¡¼¥­¥ó¥½¥óɤΰì˶¸¡Îã¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡¡
 £±¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¡2) ½çŷƲ°å±¡Ç¾¿À·ÐÆâ²Ê¡¡3)µµÅÄÁí¹çɱ¡¿À·ÐÆâ²Ê¡¤
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95 KNP

*Date¡§20100313
*Manager¡§Makoto Shibuya

1radiological quize
 £±¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯ ¡ÊBBAR¡Ë£²¡ËƱÉÂÍý¿ÇÃÇ²Ê £³¡ËƱÊü¼ÍÀþ²Ê £´¡ËƱ¥ê¥Ï¥Ó¥ê¥Æ¡¼¥·¥ç¥ó²Ê £µ¡ËƱǾ³°²Ê £¶¡ËƱ¸ÆµÛ´ïÆâ²Ê¡¤
 Â­Î©Àµ£±¡Ë¡¢½éÅÄ͵¹¬£±¡Ë£²¡Ë¡¢¿ù»³Èþµª»Ò£±¡Ë¡¢½®ÊÕ¤µ¤ä¤«£±¡Ë¡¢ùþ´Ý°¤Ìí£³¡Ë¡¢²ÃÆ£µ®¹Ô£´¡Ë¡¢ÃÞ°æ·ÃÈþ»Ò£µ¡Ë¡¢Ë¾·î±ÑÌÀ£¶¡Ë¡¢ÂôÊÕ¸µ»Ê£²¡Ë¡¢Â¼»³ÈËͺ£±¡Ë
 
2µðǾ¾É¡¢ÀèÅ·À­¿åƬ¾É¤òÄ褷¤¿2ºÐÃË»ù˶¸¡Îã
 £±¡ËÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯ £²¡ËƱÉÂÍý¿ÇÃÇ²Ê ¡¤£³¡ËË­Åçɱ¡¾®»ù²Ê £´¡ËË­Åçɱ¡¸¡ºº²Ê¡¤
 Â­Î©Àµ£±¡Ë¡¢½éÅÄ͵¹¬£±¡Ë£²¡Ë¡¢ÎëÌÚ°á»Ò£±¡Ë¡¢ÁÒÈËÊþ»Ò£³¡Ë¡¢Å¢»Òʸ£´¡Ë¡¢Â¼»³ÈËͺ£±¡Ë
 
3Ã×»àŪǾ±ê¤òÄ褷¤¿ÆÃȯÀ­¹¥»ÀµåÁý¿¾É¤Î34ºÐÃËÀ­Îã
 1)Åìµþ°å²Ê»õ²ÊÂç³ØÂç³Ø±¡Ç¾¿À·ÐÉÂÂֳء¢2)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¢3)ÅìµþÅÔΩ¾¾Âôɱ¡¸¡ºº²Ê¡¢4)Áí¹çɱ¡¼è¼ê¶¨Æ±É±¡
 ¾®ÎÓÁµ1)2)¡¢ÅÚëˮ½©2)3)¡¢»°ÌÚ°ìÆÁ4)¡¢½©»³¼£É§2)
 
4ALSD with tauopathy
 Neurol1) and Neuropathol2), The Jikei Univ. Schl. of Med.
 Yoshioka M1), Hashimoto M1), Murakami M1), Kawasaki K1), Suzuki M1), Fukuda T2)
 
5An autopsy case of dementia with Lewy bodies with marked brain atrophy.
 National center hospital of neurology and psychiatry
 Yoshihiko Furusawa, Yoshiyuki Kondo, Miho Murata, Yuko Saito
 
6MM2 cortical + thalamic form¤Î¥¯¥í¥¤¥Ä¥Õ¥§¥ë¥È¡¦¥ä¥³¥ÖɤÎ1˶¸¡Îã
 £±¡ËÅìµþ°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤£²¡ËÈþ¸¶µ­Ç°É±¡¡¡¿À·ÐÆâ²Ê¡¤£³¡ËÅìµþ°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¡¤£´¡ËÅìËÌÂç³ØÉÂÂÖ¿À·Ð³ØʬÌ£µ¡ËÈþ¸¶µ­Ç°É±¡¡¡¿À·ÐÆñÉ¡¦Ç§ÃξÉÉôÌ硤£¶¡ËÅìµþÂç³Ø¿À·ÐÆâ²Ê¡¤
 ¿¹ÅÄÍÛ»Ò£±¡Ë¡¤¹âÈø¾»¼ù£²¡¤£µ¡Ë¡¤ÁÒ»ý¡¡ÌУ³¡Ë¡¤ËÌËÜůǷ£´¡Ë¡¤Èþ¸¶È×£²¡Ë¡¤µÈÅÄÍÎÆ󣵡ˡ¤ÂåÅÄͪ°ìϺ£¶¡Ë
 
7¸ÆµÛ¶ÚËãáã¤ÎµÞ®¤Ê¿Ê¹Ô¤ò¼¨¤·¤¿¡¢Î×¾²·Ð²áȾǯ¤Î¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î1˶¸¡Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤3)ÀŲ¬»ÔΩÀ¶¿åɱ¡¡¡¿À·ÐÆâ²Ê
 ¿ù»³Èþµª»Ò1)¡¢¿ù±ºÌÀ2)¡¢¾®ÈøÃÒ°ì2)¡¢Â­Î©Àµ1)¡¢½éÅÄ͵¹¬1)¡¢½®ÊÕ¤µ¤ä¤«1)¡¢ÈªÎ´»Ö3)¡¢Â¼»³ÈËͺ1)¡¢ ¹Â¸ý¸ù°ì2)
 
8A 42-year-old female with left-sided hemiparesis and multiple brain lesions
 Dept. of Pathology, Saitama Medical University, International Medical Center
 Atsushi Sasaki
 

94 KNP

*Date¡§20091212
*Manager¡§Kinya Ishikawa

1°¡µÞÀ­¿Ê¹ÔÀ­Î¾¾å»èËãáã¤òÄ褷¡¢¸Â¶ÉÀ­ÂоÎÀ­²¼°Ì·Û¿ñÁ°³ÑÃæ¿´ÉÂÊѤòǧ¤á¤¿¡¢86ºÐÃËÀ­Îã
 1)ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÉÂÍý¡¢2)Ʊ¿À·ÐÆâ²Ê¡¤3)ƱÉÂÍý¿ÇÃDzʡ¤4)ƱÊü¼ÍÀþ²Ê
 ¿ù»³Èþµª»Ò1)¡¢¿Î²Ê͵»Ë2)¡¢ËÜ´Ö¾°»Ò3)¡¢Â­Î©Àµ1)¡¢½®ÊÕ¤µ¤ä¤«1)¡¢½éÅÄ͵¹¬1)¡¢ÆÁ´Ý°¡Ìí4)¡¢Â¼»³ÈËͺ1)¡¡
 
2Á´Ç¾·¿ÉÂÊѤò¤­¤¿¤·¤¿¡¢¥×¥ê¥ª¥ó°äÅÁ»ÒE200KÊÑ°Û¤òȼ¤¦¡¢¥¯¥í¥¤¥Ä¥Õ¥§¥ë¥È¥ä¥³¥Öɤΰì˶¸¡Îã
 1) Department of Neurology, Shizuoka Institute of Epilepsy and Neurological Disorders¡¤2) Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
 Akira Sugiura, Tomokazu Obi, Kinya Yamazaki, Takeo shishido, Shigeo Murayama, Kouichi Mizoguchi
 
3ÉÔ¼ÂÌÑÁÛ¤òÆÃħ¤È¤·¡¢È¯¾É¸åÌó2ǯ¤Î·Ð²á¤Ç»àË´¤·¤¿Ç§Ãξɤòȼ¤¦¶Ú°à½ÌÀ­¹Å²½¾É¤Î57ºÐ½÷À­Îã
 1. ¹ñΩÀº¿À¿À·Ð¥»¥ó¥¿¡¼É±¡¡¡¿À·ÐÆâ²Ê¡¢2. ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¡Ê¬»Ò¿À·ÐÀ¸Êª³Ø¸¦µæ¥Á¡¼¥à3. ¹ñΩÀº¿À¿À·Ð¥»¥ó¥¿¡¼É±¡¡¡Î×¾²¸¡ººÉô¡¢*¸½¡¡¹ñΩÀº¿À¿À·Ð¥»¥ó¥¿¡¼¡¡¿À·Ð¸¦µæ½ê¡¤
 Â¼ÅIJ»Ò1¡¢À鸶ŵÉ×1*¡¢ÄÍËÜÃé1¡¢Â¼ÅÄÈþÊæ1¡¢Ä¹Ã«ÀîÀ®¿Í2¡¢óîƣʹ»Ò3
 
4¶µ°é¹Ö±é
³°½ýÀ­¤Æ¤ó¤«¤ó¤Î¿À·ÐÉÂÍý
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¦¤Æ¤ó¤«¤ó¥Ñ¥½¥í¥¸¡¼¥»¥ó¥¿¡¼
 ¿·°æ¿®Î´
 
5An autopsy case of leukodystrophy presenting with early-onset dementia
 Department of Neurology, Showa University School of Medicine 1), Department of Neurology, Ushioda General Hospital 2), Department of Neurology, Jichi Medical School 3)
 Ishihara K 1), Ohno H 1), Sugie M 1),¡¤Kawamura M 1), Shiota J 2), Nakano I 3)
 
6Lessons from observation on spinal cord with vertebra in an autopsy case with post-operative state of ossification of posterior longitudinal ligament at the cervical
 1)Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Departments of 2)Laboratory Medicine and 3)Neurosurgery, Tokyo Metropolitan Neurological Hospital, 4)Department of Neurology, Tokyo Metropolitan Kita Medical and Rehabilitation Center for the Disabled, 5)Department of Laboratory Medicine, Tokyo Medical University Ibaraki Medical Center
 Kiyomitsu Oyanagi1,2), Liyuan Sun1), Jun Nakauchi3), Yoko Mochizuki2,4), Makoto Shibuya2,5), Toshio Mizutani2)
 An autopsy case with post-operative state of ossification of posterior longitudinal ligament (OPLL) at the cervical was examined neuropathologically. Observation on the spinal cord with vertebra elucidated transverse myelopathy (TM) at the 6th cervical vertebra and ascending and descending Wallerian degeneration. Protrusion of OPLL lesion (2mm to back side) was observed at the TM level, but enough room was found in the spinal canal. Pathomechanisms of the lesions were discussed.
 
7Autopsy case of subacute sclerosing panencephalitis
 Kanagawa Children's Medical Center¡¤1)Division of Child Neurology, 2)Dept of Pathology
 1)Sumimasa Yamashita, 2)Mio Tanaka, Yuukiti Tanaka
 
8¶µ°é¹Ö±é
Ǿ¼ðá綵°é±éÂꡧÃæƬ³¸ãݵðÂç·ì´ÉÀ­¼ðáç¤Î£³Îã
 Åìµþ°å²ÊÂç³Ø¡¡°ñ¾ë°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¿ÇÃÇÉô
 ß§Ã«¡¡À¿
 

93 KNP

*Date¡§20090718
*Manager¡§Tomohiko Mizutani

1A case with hypertrophy of the left lumbosacral nerve roots and ganglia, ¡¤presenting progressive muscle weakness and sensory disturbances of the left leg¡¤
 1) National Center Hospital of Neurology and Psychiatry, Department Neurology¡¤2) National Center Hospital of Neurology and Psychiatry, Department of Laboratory Medicine¡¤
 Tomoko Okamoto1), Yoshiyuki Kondo1), Yasushi Oya1), Yuko Saito2) , Yoshihiko Furusawa1), ¡¤Harumasa Nakamura1), Miho Murata1)¡¤
 
2An autopsy case of sporadic amyotrophic lateral sclerosis(TDP-43 proteinopathy) with localized degeneration in corpus callosum.
 1)Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology¡¤2)Department of Neurology, Kameda medical center¡¤3)Department of Neurology, Yokohama city university
 Mikiko Sugiyama1)3)¡¢Masatoshi Takahashi2)¡¢Tadashi Adachi1)¡¢Hiroyuki Hatsuta1)¡¢Sayaka Funabe1)¡¢Toshio Fukutake2)¡¢Shigeo Murayama1)
 
3An autopsied case of upper motor neuron dominant amyotrophic lateral sclerosis with dementia presenting with agraphia in the early stage
 1) Dep. Neurol., Showa Univ. Sch. Med. 2) Dep. Neurol.,Ushioda Gen. Hosp. 3) Dep. Neurol., Jichi Med. Sch.
 Ishihara K1) Kawamura M1) Suzuki Y2) Shiota J2) Nakano I3)
 
4²¼°Ì±¿Æ°¥Ë¥å¡¼¥í¥óħ¸õ¤Î¤ß¤òÄ褷¤¿Á´·Ð²á19ǯ¤Î¸ÉȯÀ­ALS¤Î61ºÐ¡Ê»àË´»þ¡Ë½÷À­Î㡤
 1)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¢2)Åìµþ°å²Ê»õ²ÊÂç³ØÂç³Ø±¡Ç¾¿À·ÐÉÂÂֳء¢3)ÅìµþÅÔΩ¾¾Âôɱ¡¸¡ºº²Ê¡¢4)¥Þ¥ó¥Á¥§¥¹¥¿¡¼Âç³ØÎ×¾²¿À·Ð²Ê³Ø¸¦µæ¥°¥ë¡¼¥×
 ¾®ÎÓÁµ1)2)¡¢ÅÚëˮ½©3)¡¢¿·°æůÌÀ1)¡¢²£ÅĽ¤4)¡¢½©»³¼£É§1)¡¢¿åß·±ÑÍÎ2)
 
5¶µ°é±éÂꡧNeimann-Pick É Type C
 ÅìµþÅÔ·ò¹¯Ä¹¼÷°åÎÅ¥»¥ó¥¿¡¼¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ÎëÌÚ¡¡°á»Ò
 
6µÙ·Æ
 
 
 
7²èÁü¥Ö¥ê¡¼¥Õ¥ì¥Ó¥å¡¼
 
 
 
8Pathology of Gliomatosis Cerebri¡¡
 Department of Pathology, Saitama Medical University, International Medical Center, Saitama, Gunma, Japan¡¤¡¤
 Atsushi Sasaki
 
9An autopsy case of sarcoidosis with ossification of the posterior longitudinal ligament (OPLL) and remote cerebellar hemorrhage
 1)Department of Pathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerountology, Tokyo, Japan¡¤2)Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerountology, Tokyo, Japan¡¤3) Department of Neurology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerountology, Japan, 4) Department of Radiology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerountology, Japan
 Hiroyuki HATSUTA 1)2), Hiroshi NISHINA 3), Masako SUNAGAWA 3), Mikiko SUGIYAMA 2), Sayaka FUNABE 2), Tadashi ADACHI 1), Aya TOKUMARU 4), Tomio ARAI 2) and Shigeo MURAYAMA 1)
 
10A case of diffuse leukoencephalopathy with cortical laminar necrosis
 1)Dept. of Neurology, Jikei Univ Sch Med, 2)Dept. of Neurosurgery, Jikei Univ Sch Med, 3)Dept. of Neuropathology, Jikei Univ Sch Med
 Yohei Tamura1), Shinji Miyagawa1), Toshiaki Hirai1), Akira Kurita1), Toshihide Tanaka2), Takahiro Fukuda3)
 
11A case of middle-aged man with a frontal lobe brain tumor presenting with convulsion
 1) Department of Pathology, Nihon University, School of Medicine, Laboratory Department 2), Department of Neurosurgery 3) and Department of Radiation4), Tokyo Metropolitan Health and Medical Treatment Corporation, Ebara Hospital
 Taku Homma 1), 2) Hitoshi Tokunaga 3) Masahiro Ida 4) Hiroshi Doi 3) Manabu Takahashi 2)
 

92 KNP

*Date¡§20090314
*Manager¡§Takahiro Fukuda

1¾ÉÎ㣱
ȯ¸ì¼º¹Ô¤òÄ褷¡¢Ë¶¸¡¤Ë¤ÆWernickeǾ¾É¡¢pellagraǾ¾É¡¢Morel¡Çs laminar sclerosis¤ÎÉÂÍý½ê¸«¤òǧ¤á¤¿57ºÐÃËÀ­Îã
 1)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¢2)Åìµþ°å²Ê»õ²ÊÂç³ØÂç³Ø±¡Ç¾¿À·ÐÉÂÂֳء¢3)ÅìµþÅÔΩ¾¾Âôɱ¡¸¡ºº²Ê
 ¾®ÎÓÁµ1)2)¡¢ÅÚëˮ½©3)¡¢²£ÅĽ¤1)¡¢¿·°æůÌÀ1)¡¢½©»³¼£É§1)¡¢¿åß·±ÑÍÎ2)
 
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57ºÐȯ¾É¤ÎRasmussenǾ±ê¡Êµ¿¤¤¡ËÎã
 1)ÀŲ¬ÀÖ½½»úɱ¡¿À·ÐÆâ²Ê¡¤2)ÀŲ¬ÀÖ½½»úɱ¡ÉÂÍýÉô¡¤3)Ǿ·ì´É¸¦µæ½êÈþ¸¶µ­Ç°É±¡¿À·ÐÆñÉ¡¦Ç§ÃξÉÉôÌç
 1)¾®À¾¹â»Ö¡¡1)ȬÌÚÀëÂÙ¡¡1)º£°æ¾º¡¡1)¶Üß·ÀµÇî¡¡1)¾®Ä¥¾»¹¨¡¤2)³Þ¸¶ÀµÃË¡¤3)¹âÈø¾»¼ù
 
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Degeneration of spinal lateral funiculi in a 71-year-old female with large cell carcinoma of lung
 1)Department of Neurology, Hiratsuka Municipal Hospital¡¤2)Department of Internal Medicine, Hiratsuka Municipal Hospital¡¤3)Department of Pathology, School of Medicine, Keio University¡¤4)Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience
 1)Koide T, 2)Sakaguchi T, 3)To B, 4)Hashimoto T, 4)Oyanagi K
 Neuropathological findings of a 71-year-old female with metastatic lung cancer of brain were described. She suffered from diabetes and hyperlipidemia, and had gingival cancer in the past history. High CEA value was pointed 7 months before her death. MRI indicated multiple tumor masses in the brain. She ¡¤died of pneumonia. Autopsy revealed large cell carcinoma in the left lung, ¡¤immunopositive for synaptophysin and CD56, and multiple metastases in the cer¡¤ebrum and cerebellum. The medulla oblongata and spinal cord showed severer degeneration in the distal portions of the lateral corticospinal tracts, dorsal spinocereballar tracts and fasciculus gracilis on both sides. A certain malnutrition state or toxic situation remained as the pathogenesis of the tracts degeneration. However, since there were some reports indicating a degeneration of the lateral corticospinal and ascending tracts in the spinal cord as a paraneoplastic neuropathy, the present case should be precisely examined and reviewed in that light.
 
4¾ÉÎ㣴
ÀÔ¿ñ¥µ¥ë¥³¥¤¥É¡¼¥·¥¹¤Î¹çÊ»¤¬µ¿¤ï¤ì¤¿¥·¥§¡¼¥°¥ì¥ó¾É¸õ·²¤Î51ºÐ½÷À­
 1Dept. of Neurology, and 2Div. of Neuropathology, The Jikei Univ. Schl. of Med.
 Takagi S1,Murakami Y1, Umehara T1, Kouno Y1, Yaguchi H1, Mochio S1, Fukuda T2
 
5¾ÉÎ㣵
·Ð²á58ǯ¡¢»ë³Ð¾ã³²¡¦°Û¾ï´¶³Ð¡¦Êâ¹Ôº¤Æñ¤Çȯ¾É¤·¡¢ºÇ¶áÌó20ǯ´Ö¤ÏÌÀ¤é¤«¤ÊºÆȯ¤Î¥¨¥Ô¥½¡¼¥É¤òǧ¤á¤Ê¤«¤Ã¤¿¡¢Â¿È¯À­¹Å²½¾É¤Î76ºÐ½÷À­Ë¶¸¡Îã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê ϷǯÉÂ¥²¥Î¥à¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÅìµþÂç³Ø°å³ØÉôÉí°É±¡¡¡¿À·ÐÆâ²Ê
 ÅÄÃæ¿¿À¸1)2)¡¢½®ÊÕ¤µ¤ä¤«1)¡¢Â­Î©Àµ1)¡¢½éÅÄ͵¹¬1)¡¢óîƣʹ»Ò1)¡¢Â¼»³ÈËͺ1)
 
6µÙ·Æ
 
 
 
7²èÁü¥×¥ì¥Ó¥å¡¼
 
 
 
8¾ÉÎ㣶
An autopsy case of parkinsonism with dementia, caused by degenerated substantia nigra and locus ceruleus, alpha-synuclein positive neurons in amygdala, and many senile plaques
 ÆüËÜÂç³Ø°å³ØÉô ÉÂÍý³ØʬÌ¡Ë¡¢Æ±¡¡¿À·ÐÆâ²Ê³ØʬÌî2)¡¢Ä¹²¬À¾É±¡3)¡¢Àî¸ý»Ô̱°åÎÅ¥»¥ó¥¿¡¼4)
 Taku Homma 1), Makoto Sano 1), You Kanno 2), Yukari Obana 1), Kenji Miki 4), Naoki Ishihara 2), Masato Tamura 3), Satoshi Kamei 2), Norimichi Nemoto 1), Tomohiko Mizutani 2)
 
9¾ÉÎ㣷
An autopsy case of diffuse neurofibrillary tangles with calcification and numerous senile plaques
 1)Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology,¡¤Tokyo, Japan¡¤2)Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤3) Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤4)Department of Radiology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤
 Hiroyuki HATSUTA 1), Yuko SAITO 1)2), Tadashi ADACHI 1), Sayaka FUNABE 1), Meiko SHIINA 3), Aya TOKUMARU 4), Tomio ARAI 2) and Shigeo MURAYAMA 1)
 
10¾ÉÎ㣸
An autopsy case with widespread phosphorylated TDP43 immunoreactive structures
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology¡¤2) Department of Pathology, Tokyo Metropolitan General Hospital (TMGH)¡¤3) Department of Radiology, TMGH¡¤4) Department of Neurology, TMGH¡¤
 Tadashi Adachi1), Yuko Saito1)2), Hiroyuki Hatsuta1), Sayaka Funabe1), Aya Tokumaru3), Kazutomi Kanemaru4), Motoji Sawabe2), Shigeo Murayama1)
 Case: The patient was 96 years old at the time of her death. She was diagnosed Alzheimer type senile dementia at 82 years old, but there is no information of her dementia. She lived in nursing home. At the age 91, she was admitted our hospital because of aspiration pneumonia. She could not walk and lived with wheel chair. At the age 92, MMSE score was 0/30 and she was bedridden. She died at the age of 96. Twelve days before her death, she presented repeated general convulsion.¡¤General pathology: The cause of her death was malnutrition and pleural effusion.¡¤Neuropathology: Brain weighted 1138 gram prior to fixation. Left side- dominant atrophy around precentral gyrus was present. Substantia nigra was pale and hipoccampus was atrophic and ¡¤subiculum was atrophic and grayish discoloration. Dentate nucleus and subthalamic nucleus were also grayish discoloration. Micorscopically, there were wide spread neuronal loss and gliosis in the ¡¤dentate nucleus, pallidum, inferior olivary nucleus, subthalamic nucleus, nigra, hippocampus, and cerebral cortex. Phosphorylated TDP43 positeve structures are also present in the degenerative area. Some of them were detected with ubiquitin immunostain, but with non- phosphorylated TDP43, there is small number of positive structures. In the anterior horn of spinal cord, there was little TDP43 structures. Braak NFT stage was 2, and amyloid stage was 0.¡¤Discussion: What category should we adopt this case ?¡¤
 
11¾ÉÎ㣹
An autopsy case of collodion baby
 1Dept. of Pediatrics, Jikei Univ. Schl. of Med., 2Dept. of New Born Baby, Ctr for Perinatal Care, Tokyo Metropolitan Bokutoh hp, 3Dept. of Pathology, Ctr for Perinatal Care, Tokyo Metropolitan Bokutoh hp, 4Div. of Neuropathology, Jikei Univ. Schl. of Med.
 Kobayashi M1, Kimura Y2, Warabi M3, Fukuda T4
 
12¾ÉÎ㣱£°
An autopsy case of Mucolipidosis III
 »ü·Ã°åÂç1°äÅÁ»Ò¼£ÎŸ¦µæÉô¡¢2Ʊ¾®»ù²Ê¡¢3Ʊ¿À·ÐÉÂÍý³Ø¡¢4Ʊ°äÅÁÉ¡ʥ饤¥½¥¾¡¼¥àÉ¡˸¦µæ¹ÖºÂ¡¢5ÂçºåÂç³ØÂç³Ø±¡¾®»ùȯã°å³Ø¹ÖºÂ
 Kobayashi H1,2, Sakurai K1,2, Ohashi T1,2, Ida H1,2, Fujigasaki J3, Etoh Y4, Sakai N5, Ohtomo T5
 

91 KNP

*Date¡§20080110
*Manager¡§Hitoshi Okazawa

1¹âÎðȯ¾É¡¢¿Ê¹ÔÀ­¸ÆµÛÉÔÁ´¤ò¼çħ¤È¤·¤¿¡¢¹³Hu¹³ÂÎÍÛÀ­Ëµ¼ðáç¾É¸õ·²Ë¶¸¡Îã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¡¦ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¡¡¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¢£²¡ËÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê
 óîƣʹ»Ò
 
247ºÐ¤Çȯ¾É¤·¤¿Ç§ÃξɤÎ1¾ÉÎã¡ÊºÆ¹Í¡Ë
 1)Èþ¸¶µ­Ç°É±¡¿À·ÐÆñÉ¡¦Ç§ÃξÉÉôÌ硤2)Ʊ¡¡¿À·ÐÆâ²Ê¡¤3)ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¤4)ºë¶Ì°å²ÊÂç³Ø¹ñºÝ°åÎÅŽ¾ŽÝŽÀŽ°Ç¾Â´Ã掾ŽÝŽÀŽ°¡¤5)¤è¤ß¤¦¤ê¥é¥ó¥É·Äͧɱ¡
 ¹âÈø¡¡¾»¼ù
 
3ðô¿ñ¹ÅËì²¼·ì¼ð¤Î°ìÎã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¡¡¿À·ÐÉÂÍý¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯ 2)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¡¡Ë¶¸¡ÉÂÍý²Ê¡¡3)Ʊ¡¡Êü¼ÍÀþ²Ê
 ½®ÊÕ¤µ¤ä¤«1) óîƣʹ»Ò1)2)¡¡Â­Î©Àµ1)¡¡½éÅÄ͵¹¬1)¡¡ÆÁ´Ý°¡Ìí3)¡¡¿·°æÉÚÀ¸2) ¼»³ÈËͺ1)
 
4Three cases of Langerhans cell histiocytosis involving skull or hypothalamus
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma; Department of 3Neurosurgery, Tatebayashi Municipal Hospital, Gunma
 Atsushi Sasaki1, Shogo Ishiuchi2, Keishi Horiguchi3, Yoichi Nakazato1
 
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6²èÁü¥×¥ì¥Ó¥å¡¼
 
 
 
7What is your diagnosis
 Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology
 Emeritus Professor Kinuko Suzuki
 
8Autopsy case of Joubert syndrome
 Kanagawa Children's Medical Center 1)Division of Child Neurology, 2)General Pediatrics, 3)Nephrology and 4)Pathology
 1)Sumimasa Yamashita, Kiyoko Samejima, 2)Kiyoshi Matsui, 3)Eihiko Takahashi, 4)Mio Tanaka
 
9̤ʬ²½°­À­¼ðáç¤òȼ¤¤¡¢Á°³Ñ¡¦Ç¾´´¤òÃæ¿´¤È¤·¤¿congulomerate inclusion¤Î¿ȯ¤òǧ¤á¤¿°ì˶¸¡Îã
 1) ÅìµþÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²Ê¿À·ÐÆâ²Ê¡¤2) ÅìµþÂç³Ø°å³ØÉôÉհɱ¡ÉÂÍýÉô¡¤3) ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤
 ¶¶ËÜÌÀ»Ò1)¡¢¿¹ÅÄÌмù2)¡¢¸Åϵ×Êþ1)¡¢´äÅÄ¡¡½ß1)¡¢À¶¿å¡¡½á1)¡¢ÄÔ¡¡¾Ê¼¡1)¡¢¡¤Ê¡Åè·Éµ¹2)¡¢¿¼»³Àµµ×2)¡¢Â¼»³ÈËͺ3)
 
10·Ð²á18ǯ¤ÎSOD1ÊÑ°Û¤òȼ¤¦²È²À­¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É74ºÐ˶¸¡Îã
 1)Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology,¡¤Tokyo, Japan¡¤2)Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤3)Department of Neurology, Kameda Medical Center, Chiba, Japan
 Hiroyuki HATSUTA 1), Yuko SAITO 2), Susumu SATO 3), Toshio FUKUTAKE 3) and Shigeo MURAYAMA 1)
 
11amyotrophic lateral sclerosis with palidolysienne degeneration
 1) Department of Neurology & 2) Pathology, Yokohama Rosai Hospital; 3) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology
 Shigeo Murayama, Shinya Oominami, Ichiro Imafuku
 

90 KNP

*Date¡§20080719
*Manager¡§Hideo Mori

1¾å°Ìðô¿ñ¡¦±ä¿ñ¤Ë¼çÉÂÊѤòÍ­¤¹¤ëdural AVF¤È¹Í¤¨¤é¤ì¤ë63ºÐÃËÀ­Ë¶¸¡Îã
 1¡ËÄëµþÂç³Ø°å³ØÉôÉÂÍý¡¤2¡Ë¡¡¡·¡¡¡¡¡¡¡¡¿À·ÐÆâ²Ê¡¤3¡Ë¼«¼£°åÂç¿À·ÐÆâ²Ê¡¤
 ¡ûÃÓ¼²í»Ò1¡Ë¡¢¡ûȪÃæ͵¸Ê2¡Ë¡¢°ËÆ£±Ñ°ì2¡Ë¡¢¿·°æÍ´»Ò2¡Ë¡¢À¶¿åµ±É×2¡Ë¡¢ÃæÌ¼£3¡Ë¡¢Ê¡Î¤ÍøÉ×1¡Ë
 
2Á´¿ÈËã¿ì½Ñ¸åÓë²¼¾ã³²¤¬½Ð¸½¤·¡¢Â¿·ÏÅý°à½Ì¾ÉÁ°¶î¾õÂÖ¤¬µ¿¤ï¤ì¤ë77ºÐÃËÀ­Ë¶¸¡Îã
 1) Department of Neuropathology(The Brain Bank for Aging Research), Tokyo Metropolitan Institute of Gerontology, 2) Department of Pathology, Tokyo Metropolitan Geriatric Hospital, 3) Department of Neurology, Tokyo Metropolitan Geriatric Hospital, 4) Department of Radiology, Tokyo Metropolitan Geriatric Hospital
 Hatsuta H 1), Saito Y 1)2), Shiina M 3), Adachi T 1), Funabe S 1), Tokumaru A 4), Sawabe M 2) and Murayama S 1)
 
3»àË´»þ63ºÐ¡¢·ì´ÉÆâ¥ê¥ó¥Ñ¼ð¥¢¥¸¥¢°¡·¿ÃËÀ­Îã
 1)ÆÁÅçÂç³Øɱ¡ ¿À·ÐÆâ²Ê¡¡¡¤2)ÆÁÅçÂç³Ø¡¡´ï´±ÉÂÂÖ½¤Éü°å³Ø¹ÖºÂ¡¡¡¤3)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤¡¤
 ³ùÅÄÀµµª1)¡¢ÏÂÀôÍ£¿®1)¡¢Àõ¾Â¸÷ÂÀϺ1)¡¢³áζѻ1)¡¡¡¤¹©Æ£±Ñ¼£2)¡¤Â¼»³ÈËͺ3)¡¤
 
4What's your diagnosis? ¡¼ÎëÌÚ°á»ÒÀèÀ¸¤ÎÆÃÊ̥쥯¥Á¥ã¡¼(1)
An autopsy case of an infant with severe microcephaly
 
 Kinuko Suzuki
 Clinical presentation¡¤A 39-year-old female with no significant past medical history delivered a female infant via an uncomplicated C-section at 39 weeks gestation. The prenatal history was unremarkable. Chorionic villus sampling performed during the second trimester revealed a normal karyotype. At birth, the infant failed to exhibit initial cry and Apgars were 6 at one minute and 7 at five minutes. She had a poor respiratory effort and a heart rate less than 100 beats per minute. Her heart rate improved with positive pressure ventilation, while respiratory efforts remained depressed, leading to intubation. The patient also began to exhibit seizure-like activity immediately after birth with jerking movements involving all four extremities. She was loaded with Phenobarbital, and antibiotics were started. The infant was noted to be hypertonic, jittery, and microcephalic with a down-sloping forehead and overriding coronal sutures. Septic work-up was initiated but cultures were negative. An ultrasound of the head revealed bilateral extra-axial fluid collections and possible mild prominence of the cisterna magna. An MRI of the brain showed generalized hypoplasia of the cerebral hemispheres and cerebellum. There was diffuse abundance of the extra-axial CSF spaces and minimal hydrocephalus. EEG revealed a pattern consistent with severe bihemispheric dysfunction without evidence of seizure activity. The parents were advised of the infant¡Çs poor prognosis, and they elected to withdraw support. The infant died on day two of life. ¡¤¡¤¡¤Postmortem Examination¡¤External examination of the infant revealed a female infant consistent with 39 weeks gestational age (3100 grams; expected 2789 +/- 520 grams). The head exhibited overlapping cranial bones with no appreciable fontanels and a down-sloping forehead with a head circumference of 31 cm. No other external anomalies were identified. ¡¤On internal examination, the organs were normally positioned and grossly unremarkable. The brain was small, firm, and well-formed (110 grams; expected 362 grams) with a developmental delay and a gyral pattern consistent with an estimated gestational age of 32-35 weeks. ¡¤
 
5¶µ°é¹Ö±é-Ǿ¼ðáçÉÂÍý¥·¥ê¡¼¥º
Glioblastoma with oligodendroglioma component¡¤grade IV (WHO 2007)¤ÎÉÂÍý¿ÇÃÇ¡¤¡¤
 Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼ÉÂÍý¿ÇÃÇÉô1) ¡¤ÅÔΩÉÜÃæɱ¡Ç¾¿À·Ð³°²Ê2)¡¤ÅÔΩ¿À¡¤·Ðɱ¡¸¡ºº²Ê3)¡¤Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼Ç¾¿À·Ð³°²Ê4)¡¤
 ß§Ã«¡¡À¿1,3)¡¤¿åë¡¡Å°2)¡¤¿åë½Óͺ3)¡¤ÃÓÅŬÊæ4)¡¤¡¤
 
6ǧÃξɡ¦¿íÂγ°Ï©¾ã³²¡¦¼«Î§¿À·Ð¾ã³²¤òÄ褷¡¢¹­ÈϤÊÇò¼ÁÉÂÊѤòǧ¤á¤¿73ºÐ½÷À­
 £±¡Ë½çŷƲÂç³Ø°å³ØÉôǾ¿À·ÐÆâ²Ê¡¤£²¡Ë½çŷƲ±Ûëɱ¡Ç¾¿À·ÐÆâ²Ê
 ½®ÊÕ¡¡¤µ¤ä¤«
 
7Ǿ¼¼¼þ°ÏÇżï¤òÄ褷¤¿°­À­¥ê¥ó¥Ñ¼ð¤Î70ºÐÃËÀ­Ë¶¸¡Îã
 1)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê Ϸǯ´üÀº¿À¼À´µ¸¦µæ¥Á¡¼¥à 2)ÅìµþÅÔΩ¾¾Âôɱ¡¸¡ºº²Ê 3)ÃæÌîÁí¹çɱ¡¿À·ÐÆâ²Ê 4)Éð¢ÌîÀÖ½½»úɱ¡¿À·ÐÆâ²Ê 5)Éð¢Ìî·ò¹¯³«È¯»ö¶ÈÃÄ 6)Éð¢ÌîÀÖ½½»úɱ¡ÉÂÍýÉô 7)ÅìµþÅÔΩ¾¾Âôɱ¡Æâ²Ê
 ¾®ÎÓÁµ1)¡¢ÅÚëˮ½©2)¡¢Ä®ÅÄÌÀ3)¡¢»°ÌÀ͵ÃÎ4)¡¢ÌÊ°úÄêÀ¶5)¡¢ÂíÏÂÇî6)¡¢±©²ìÀé·Ã1)¡¢²£ÅĽ¤1)¡¢¸åÆ£½á7)¡¢¿·°æůÌÀ1)¡¢½©»³¼£É§1)
 
8ÉñƧÉ¡¤¾®Ç¾¼ºÄ´¡¤Ç§Ãξ㳲¤òǧ¤á¡¤¹õ¼Á¡¦²¼¥ª¥ê¡¼¥Ö³Ë¤ËÊÑÀ­¤òǧ¤á¤¿1Îã
 £±¡ËÈþ¸¶µ­Ç°É±¡¡¡¿À·ÐÆñÉ¡¦Ç§ÃξÉÉô¡¤Ì硤£²¡ËƱ¡¡¿À·ÐÆâ²Ê¡¤£³¡ËÅÔΩ¾¾Âôɱ¡¡¡¸¡ºº²Ê¡¤£´¡ËÅìµþÅÔÀº¿À²Ê³ØÁí¹ç¸¦µæ½ê¡¤£µ¡Ë·ÄØæµÁ½ÎÂç³Ø¿À·ÐÆâ²Ê¡¤£¶¡Ë¤µ¤¤¤¿¤Þ»ÔΩɱ¡¡¡¿À·ÐÆâ²Ê¡¤£·¡Ë·ÄØæµÁ½ÎÂç³ØÉÂÍý³Ø¶µ¼¼¡¤
 ¹âÈø¾»¼ù
 

89 KNP

*Date¡§20080315
*Manager¡§Tomohiko Mizutani

1¿Ê¹ÔÀ­¤ÎÁ°Æ¬Â¦Æ¬·¿Ç¾°à½Ì¤òȼ¤Ã¤¿ ¤Ó¤Þ¤óÀ­¥ì¥Ó¡¼¾®ÂÎÉ¤Σ±Îã
 £±¡ËÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êϷǯÉÂ¥²¥Î¥à¡¦¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤£²¡ËÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼Ë¶¸¡ÉÂÍý²Ê¡¤£³¡Ë²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê¡¤£´¡Ë²£ÉÍÏ«ºÒɱ¡ÉÂÍý²Ê
 Â­Î©Àµ£±¡Ë¡¢óîƣʹ»Ò£±¡Ë£²¡Ë¡¢´Ø¹±·Ä£³¡Ë ¡¢¸Þ½½À»Ö£³¡Ë£´) ¡¢º£Ê¡°ìϺ£³¡Ë¡¢³ÑÅĹ¬Íº£´¡Ë¡¢Â¼»³ÈËͺ£±¡Ë¡¤¡¤
 
2An autopsied case of ¡Èneuro-Behcet disease¡É clinically masquerading frontotemporal dementia
 Department of Neurology, Showa University1¡¤Department of Neurology, Jichi Medical School2
 K. Ishihara1, Y. Horibe1, M. Sugie1, J Shiota1, M. Kawamura1, I Nakano2
 
3An 88-years old female autopsy case of encephalopathy with demyelination, necrosis and ischemic change
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, 2) Department of pathology, Tokyo Metropolitan Geriatric Hospital, 3) Department of nephrology, Tokyo Metropolitan Geriatric Hospital, 4) Department of radiology, Tokyo Metropolitan Geriatric Hospital
 Hatsuta H 1), Saito Y 1)2), Kurashige T 3), Sakiyama Y 1)2), Tokumaru A 4), Sawabe M 2), Murayama S 1)
 
4Spindle cell oncocytoma of the adenohypophysis
 Department of Human Pathology, Gunma University Graduate School of Medicine
 Atsushi Sasaki
 
5Pathology of the epileptogenic focus around the cavernous hemangiomas
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¤¿À·Ðȯ㡦ºÆÀ¸¸¦µæʬÌî (Î×¾²¿À·ÐÉÂÍýÉôÌç)¡¤¤Æ¤ó¤«¤ó¥×¥í¥¸¥§¥¯¥È¡¦¥Ñ¥½¥í¥¸¡¼¥»¥ó¥¿¡¼¡¤
 ¿·°æ¡¡¿®Î´
 
6¼ãǯÀ­²È²À­¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤Î1Îã
 ÅìµþÅÔΩ¿À·Ðɱ¡¸¡ºº²ÊÉÂÍý¿ÇÃÇÉôÌ磱¡¢Ç¾¿À·ÐÆâ²Ê£²¡¢ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¿À·ÐÉÂÍý³Ø£³¡¢Åìµþ°å²ÊÂç³ØȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼ÉÂÍý¿ÇÃÇÉô4
 Ë¾·îÍÕ»Ò
 
7FAMILIAL PARKINSONISM AND DEMENTIA WITH BALLOONED NEURONS, ARGYROPHILIC NEURONAL INCLUSIONS, ATYPICAL NEUROFIBRILLARY TANGLES, TAU-NEGATIVE ASTROCYTIC FIBRILLARY TANGLES, AND LEWY BODIES¡¡-FURTHER OBSERVATIONS-
 Departments of Neurology, Pathology and Anatomy¡¤Nihon University School of Medicine,
 Tomohiko Mizutani, Taku Homma, Kenji Uehara, Satoshi Kamei, Yutaka Suzuki, Motohiko Hara¡¢Shigeo Kakimi
 
8A case of multiple cerebral infarction caused by non-bacterial thrombotic endocarditis (NBTE) which was diagnosed at autopsy.
 Departments of Pathology£±), Neurology2), and Hematology and Rheumatology£³, Nihon University School of Medicine
 Taku Homma£±), Masayuki Minami 2), Kazuhiro Takei£³), Makoto Sano£±), Norimichi Nemoto£±), Tomohiko Mizutani2)
 We present a case of multiple cerebral infarction (CI) caused by NBTE which was diagnosed at autopsy. ¡¤Clinical course: Sixty six year-old man was diagnosed as having essential thrombocytosis which had been treated with hydroxyurea successfully. Three years after the diagnosis, he suddenly developed left hemiplegia. Brain CT showed a large low density area in the right middle cerebral artery (MCA) territory. Then he suffered from another cerebral infarct in the left MCA territory. His general condition deteriorated rapidly, and he died on the 8th hospital day. The thrombocytosis was controlled on admission.¡¤Autopsy: NBTE was observed in the mitral valve. Thromboemboli and thrombosis were observed in the systemic organs. Neuropathologically, the brain showed severe swelling with uncal and tonsillar herniations. On coronal section, the cerebrum revealed massive softenings in the MCA (Rt>Lt) and anterior cerebral artery territories. Microscopically, fresh thrombi were detected in the lumens of the both MCA and right ACA. Furthermore, scattered microthromboemboli were observed in the cerebrum. ¡¤Conclusion: Although the cause of the CIs was not determined ante-mortem, autopsy showed NBTE in the mitral valve, which was considered to be responsible for the multiple CIs in this patient. This case indicated the importance of autopsy in patients with CIs of undetermined cause.
 

88 KNP

*Date¡§20071208
*Manager¡§Takahiro Fukuda

1¾ÉÎã
¿Ê¹ÔÀ­Ç§Ãε¡Ç½¾ã³²¤ÈPSDÍÍǾÇȤòÄ褷¤¿beta-amyloid angiopathy¤Î70ºÐÃËÀ­
 1)¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼Éð¢É±¡Î×¾²¸¡ººÉô¡¤2)Ʊ¿À·ÐÆâ²Ê
 Í­ÇÏË®Àµ1) ¡¤¾®Àî²íʸ2)¡¤ÂçÌð¡¡Ç«2)¡¤Â¼ÅÄÈþÊæ2)
 
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°öƬ°ãÏ´¶¤Çȯ¾É¤·¤¿»àË´»þ55ºÐ¤ÎÃ×»àÀ­²È²À­ÉÔ̲¾É
 1)¡¡¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼Éð¢É±¡¿À·ÐÆâ²Ê¡¤2)¡¡Æ±Î×¾²¸¡ººÉô, 3) ÅìËÌÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÁÏÀ¸±þÍÑ°å³Ø¸¦µæ¥»¥ó¥¿¡¼¥×¥ê¥ª¥óÃÁÇò¸¦µæÉôÌç
 ºØƣͦÆó1)¡¤Í­ÇÏË®Àµ2)¡¤ÂçÌð¡¡Ç«1)¡¤ÅÄ¥±Ã«¹ÀË®2)¡¤ËÌËÜůǷ3)¡¤¾®Àî²íʸ1)¡¤Â¼ÅÄÈþÊæ1)
 
3¶µ°é¸ý±é
Neuropathology of mitochondria encephalomyopathy
 Division of Child Neurology¡¤Kanagawa Children's Medical Center
 Sumimasa¡¡Yamashita
 
4¾ÉÎã
¹³·ìÀòÎÅË¡Äñ¹³À­¤Ç¡¤Ç¾À¸¸¡¸å¤ËƳÆþ¤·¤¿¥¹¥Æ¥í¥¤¥É¡ÝÌȱÖÍÞÀ©ºÞÊ»ÍÑÎÅË¡¤Ë¤Æ´²²ò¤·¤¿isolated angiitis of central nervous system (IACNS) µ¿¤¤¤Î61ºÐ½÷À­Îã
 ¼«¼£°å²ÊÂç³ØÉí°É±¡Ç¾¿À·Ð¥»¥ó¥¿¡¼¡¤£±¡Ë¿À·ÐÆâ²Ê¡¤£²¡ËǾ¿À·Ð³°²Ê
 À¶¿å°½»Ò£±¡Ë¡¤²ÏËôÀéÄᣱ¡Ë¡¤³êÀîÆ»¿Í£±¡Ë¡¤ÃæÌ¼££±¡Ë¡¤µÜÅĸ޷¡Ë¡¤±×»ÒÉÒ¹°£²¡Ë
 
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A case of central neurocytoma
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan
 Atsushi Sasaki1, Shogo Ishiuchi2, Takuma Oishi1, Yoichi Nakazato1
 
6¾ÉÎã
Distribution of cerebral lesions in lymphomatoid granulomatosis - an autopsy case
 1)Department of Neurology, Tsuchiura Kyodo Hospital, 2)Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 3)Department of Neurology, Nakano Sogo Hospital, 4)Department of Neuropathology, Tokyo Institute of Psychiatry
 Zen Kobayashi1), Kuniaki Tsuchiya2), Makoto Takahashi3), Minoru Kotera1), Osamu Yokota4), Tetsuaki Arai4), Haruhiko Akiyama4)
 A 27-year-old Japanese man developed recurrent respiratory symptoms, neurological symptoms and hemophagocytic syndromes with a clinical course of 6 years. CT demonstrated bilateral pulmonary infiltrates and Trans-bronchial lung biopsy (TBLB) disclosed angiocenric lymphocytic proliferations without malignant cells or granulomas. MRI revealed multiple abnormal intensity areas in the brain and spinal cord. Cerebrospinal fluid (CSF) examinations disclosed mild pleocytosis and a presence of Epstein-Barr virus (EBV)-DNA by polymerase chain reaction (PCR). He died of a hemorrhagic shock associated with thrombocytopenia and coagulopathy. A postmortem study revealed multifocal lymphocytic proliferations or necrotic foci in the cerebrum, cerebellum, brainstem and spinal cord, predominantly in the posterior fossa. Involvement of the lung and central nervous system (CNS), detection of EBV-DNA in CSF, coexistence of a hemophagocytic syndrome, and multifocal lymphocytic proliferations without malignant cells made us consider lymphomatoid granulomatosis.
 
7¶µ°é¸ý±é
diagnostic imaging of Neurodegenerative diseases
 Neuroradiology, Tokyo Metropolitan Neurological Hospital
 Akira Yagishita
 
8¾ÉÎã
An Alzheimer disease with dementia with Lewy bodies (limbic form) patient presenting progressive supranuclear palsy like symptom and radiological findings: hummingbird sign.
 1) Department of Neurology, Makita General Hospital¡¤2) Department of Clinical Neuropathology, Tokyo Metropolitan Institute of Neuroscience¡¤3) Department of Interanal Medicine, Makita General Hospital¡¤4) Department of Pathology, Makita General Hospital
 Naoki Kasahata 1), Nobutaka Arai 2), Asako Kageyama 3), Takanobu Hidaka 1), Ikuyo Satou 3), Tomobumi Kotani 3), Yoshihisa Makita 3), 4)
 
9¾ÉÎã
Dynamic neuropathology of argyrophilic grain disease; clinical, radiological, and neuropathological study of an autopsy case
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology¡¤2) Department of Pathology, Tokyo Metropolitan Geriatric Hospital¡¤3) Department of Radiology, Tokyo Metropolitan Geriatric Hospital¡¤4) Positron Medical Center, Tokyo Metropolitan Institute of Gerontology¡¤5) Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Department of Neurology, Tama Hokubu Medical Center¡¤
 Tadashi Adachi1), Yuko Saito1)2), Aya Tokumaru3), Kenji Ishii4) , Ichiro Kasahara2) , ¡¤Kazuko Mitani5), Shigeo Murayama1)¡¤
 ¡ÚCase¡Û¡¤The patient was a Japanese man who was 86 years old at the time of death. Since 40s, he was jealousy for his wife, and had a persecution mania. He was divorced from her wife. At the age of 65years old, he married again but soon lived apart at the age of 73 because of his persecution mania. He lived alone, got on badly with his neighbors, and he did not pay living expenses for his wife. At the age of 80 years old, he had traffic accident while he was driving a car. Head CT revealed subdural hematoma(SDH).From this point, he was demented gradually, and he could not come home on his familial way. He could not live alone, and lived with his wife and his eldest son. At the age of 81 years old, he was admitted to Neurosurgery at the A University hospital. Brain CT revealed SDH was naturally absorbed, no brain contusion. MRI revealed bilateral atrophy of the temporal gyrus, and he was diagnosed Alzheimer¡Çs disease. He began to take donepezil. Gradually his gait became brady , and he complained of stammering and difficulty of swallowing. At the age of 82 years old, he was admitted to our hospital. Neurological examination revealed small monotonous speech, dementia, irritated, extrapyramidal sign(masked face, oral dyskinesia), bradykinesia. Muscle tonus was normotonic, and no limbs ataxia, no Babisnki sign. Retropulsion test was positive. MRI demonstrated atrophy of the amygdale and hippocampus, and FDG PET demonstrated left- side dominant hypometabolism of the anterior temporal lobe .Clinical diagnosis was frontotemporal dementia. At the age of 83, he was admitted to a nursing home. He could operate wheelchairs. He could communicate only with his family, but he was soon got tired. He died of ileus at the age of 86 years old. He was preserved at 4oC from one hour after of his death. Autopsy was performed with a postmortem interval of 29hrs and 32 minutes. Autopsy examination revealed ischemic change and bleeding tendency of the stomach, ileum, colon, and rectus. The cause of his death was ischemic enteritis.¡¤¡¤¡ÚMethods¡Û¡¤¡¡Neuropsychological examination, only MMSE was performed because of his going my way behavior.¡¤¡¡Neuroradiological examination, two neuroradiological specialists blindly diagnosed two MRI (at the time of A University Hospital, and our hospital), respectively. For FDG-PET investigation, after routine ¡¤imaging, we chose decreased area(p<0.01 value) using SPM analysis.¡¤Neuropathological examination followed the BBAR protocol, 6-micrometer- thick serial sections were studied by hematoxilin and eosin, and Kluver-Barrera method. The representative sections were examined with Gallyas- Braak, modified Methenamine Silver staining. For the immunocytochemical studies, AT8, Abeta 11-24, psyn #64, Anti-4 repeat (R) tau¡ÊRD4¡Ë, anti-3R tau¡ÊRD3¡Ëantibodies were used.( using a Ventana NX20 autostainer (Ventana, Tucson,AZ)).¡¤¡ÚResults¡Û¡¤Neuropsychological¡§persecution mania, irritable, not quantitative evaluated. ¡¤MMSE score was 26/30(remote memory 0/3, he couldn¡Çt answer floor name.).¡¤Neuroradiological¡§MRI¡§left- side dominant dilatation of the anterior horn of the lateral ventricle and atrophy of the amygdale and hippocampus, moderate atrophy of cerebral cortex of frontotemporal lobe, and mild periventricular leukoaraiosis. The atrophy of ambient gyrus and amygdale progress slowly, slightly left side dominant , and the atrophy reaches posterior hippocampus between 2001 and 2003.¡¤FDG- PET: At the inspection, left- side dominant hypometabolism of the anterior temporal lobe and orbital surface, SPM(p<0.01) analysis revealed left- side dominant hypometabolism of the anterior temporal lobe and orbital surface, anterior cingulate, white matter of medial frontal gyrus.¡¤Neuropathological findings¡§¡¤General neuropathological findings¡§The brain weight was 1339g. Gross examination confirmed fronto-temporal atrophy. Serial coronal sections demonstrated bilateral severe atrophy of ambient gyrus, and dilatation of the lateral ventricle especially left side, and hippocampal atrophy with posterior gradient.¡¤Microscopically, left- side dominant abundant argyrophilic grains (AGs) accompanied with atrophy in the anterior- dominant medial temporal lobe. In spite of relatively spared cortical architecture, superficial spongyosis in the atrophic cortex with abundant AGs. Ballooned neurons were seen moderately in neocortex with abundant AGs, and scattered in the amygdale especially left side.¡¤Immnocytochemistory: Majority is RD4 positive pretangles and RD4/ GB positive grains and pregrains. Relatively abundant AT8/ RD4 immunoreactive bush-like astrocytes and oligodendroglial coiled bodies. AGs and pretangles widespread in the dentate gyrus, CA4, CA2, CA1, entorhinal cortex and transentorhinal cortex and neocortex (around T2) although small amount in the neocortex. RD3 positive structures are limited in the entorhinal cortex.AT8-IR ballooned neuron, especially in the left amygdale, accompanied with abundant AGs including in the neocortex. Abundant senile plaques, mainly diffuse plaques (DPs), in the occipital lobe, parietal lobe, frontal lobe temporal lobe, hippocampal proper and precentral gyrus (Braak C.). Neurofibrillary tangles(NFTs), small amount of NFTs in the hippocampal proper, abundant in the entorhinal cortex and transentorhinal cortex. (Braak NFT Stage II(by Gallyas- Braak stain), Stage II(by AT8)).No definite Lewy body related ¦Á-synuclein immunoreactive lesion.¡¤¡ÚDiscussion¡Û¡¤A case of ¡Ègeneralized type¡É dementia with grains. Clinical diagnosis was ¡È frontotemporal dementia¡É with apparent disinhibition. MRI demonstrated left- side dominant atrophy of the amygdale and hippocampus. FDG PET demonstrated left- side dominant hypometabolism of the anterior temporal lobe and orbital surface, which is compatible with the severe type of the dementia with grains. These findings on the neuroimages were caused by pathological changes with AGs accentuated in the orbital surface and medial temporal lobe. This case is the first case of ¡Ègeneralized type of the DG¡É with intensive cliniconeuropathological study. Widespread grains might be related with widespread diffuse plaques in the neocortex. Much more study is needed in the prospective study¡¤
 

87 KNP

*Date¡§20070721
*Manager¡§Hitoshi Okazawa

1¥µ¥¤¥Õ¥©¥óÉô¤Ë¤ª¤±¤ëξ¦Æâ·ÛÆ°Ì®Êĺɡ¦¹âÅÙ¶¹ºõ¤Î77ºÐÃËÀ­Ë¶¸¡Îã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê ¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ ˶¸¡ÉÂÍý²Ê¡¤3)Ʊ ¿À·ÐÆâ²Ê¡¤4)Ʊ Êü¼ÍÀþ²Ê
 ½éÅÄ͵¹¬1)¡¢óîƣʹ»Ò1)2)¡¢º½Àî¾»»Ò3)¡¢¾®µÜÀµ3)¡¢ÆÁ´Ý°¤Ìí4)¡¢³Þ¸¶°ìϺ2)¡¢Â¼»³ÈËͺ1)
 
221ǯ¤Î·Ð²á¤Ç¶ËÅÙ¤ÎÂçǾ°à½Ì¤òÄ褷¤¿¥æ¥Ó¥­¥Á¥ó²½ÉõÆþÂΤòȼ¤¦¸¶È¯À­Â¦º÷¹Å²½¾É79ºÐÃËÀ­Îã
 £±¡ËÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êϷǯÉÂ¥²¥Î¥à¡¦¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤£²¡ËÆÈΩ¹ÔÀ¯Ë¡¿Í¹ñΩɱ¡µ¡¹½Åìµþɱ¡¿À·ÐÆâ²Ê¡¤£³¡ËÆÈΩ¹ÔÀ¯Ë¡¿Í¹ñΩɱ¡µ¡¹½Åìµþɱ¡ÉÂÍýÉô¡¤
 ºê»³²÷É×£±¡Ë¡¢»ÍÌÐÌî¤Ï¤ë¤ß£²¡Ë¡¢¼Øß·¾½£³¡Ë¡¢·ªºêÇî»Ê£²¡Ë¡¢óîƣʹ»Ò£±¡Ë¡¢Â¼»³ÈËͺ£±¡Ë¡¤
 
3A biopsy case of 62-year-old male with multiple mass lesions
 1Neuropathology, The Jikei University School of Medicine, 2Neurosurgery and 3 Pathology, Atsugi City Hospital
 Fukuda T1, Kaku S2, Tokuda T3
 
4A case of cerebellar hemangioblastoma
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan
 Atsushi Sasaki1, Shogo Ishiuchi2, Takuma Oishi1, Junko Hirato1, Yoichi Nakazato1
 
5¤Æ¤ó¤«¤ó¸¶À­Ç¾·ÁÀ®°Û¾ï¤Î³°²ÊÉÂÍý¿ÇÃǾå¤ÎÌäÂêÅÀ¡¡¡¼Palmini¤ÎʬÎà¤Ë¤Ä¤¤¤Æ
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¦¤Æ¤ó¤«¤ó¥Ñ¥½¥í¥¸¡¼¥»¥ó¥¿¡¼
 ¿·°æ¡¡¿®Î´
 
6¦Ã¥Ê¥¤¥Õ¾È¼ÍÆóÆü¸å¤Ë»àË´¤·¤¿Å¾°ÜÀ­Ç¾¼ðáç
 ²£ÉÍÏ«ºÒɱ¡¡¡ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê
 ´Ø¹±·Ä¡¡¸Þ½½À»Ö¡¡¼þÆ£¹â¡¡Á°¸¶¹§¸÷¡¡³ÑÅĹ¬Íº¡¡ÀçÀÐϣʿ¡¡Â¼»³ÈËͺ
 
7ÂçƬ¾É¤È¿¾®Ç¾²ó¤òǧ¤á¤¿4q-¾É¸õ·²ÃË»ù¤Î°ì˶¸¡Îã
 1¡ËÅìµþÅÔΩ¿À·Ðɱ¡¡¡¿À·Ð¾®»ù²Ê¡¢2¡ËÅìµþÅÔΩ¿À·Ðɱ¡¡¡¸¡ºº²ÊÉÂÍý¿ÇÃÇÉôÌç¡¢¡¤3¡ËÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡Î×¾²¿À·ÐÉÂÍý¡¤
 ÉÚÅÄ¡¡Ä¾1¡Ë¡¢Ë¾·î¡¡ÍÕ»Ò2¡Ë¡¢·§ÅÄ¡¡Áï»Ò1¡Ë¡¢²Ö˼¡¡Í³µ¨»Ò1¡Ë¡¢·ª¸¶¡¡±ÉÆó1¡Ë¡¢¡¤ÎÓ¡¡²íÀ²3¡Ë¡¢¿åë¡¡½Óͺ2¡Ë¡¤
 
8Spinocerebellar ataxia 2. A case report.
 £±¡Ë·ÄØæµÁ½ÎÂç³Ø°å³ØÉôË¡°å³Ø¡¤£²¡ËºâÃÄË¡¿ÍǾ·ì´É¸¦µæ½êÈþ¸¶µ­Ç°É±¡¡¤£³¡ËÀÄ»³Æâ²Ê¾®»ù²Ê°å±¡¡¤£´¡ËÅìµþ°å²Ê»õ²ÊÂç³Ø¿À·ÐÆâ²Ê¡¤£µ¡ËÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê¡¤£¶¡ËÅÔΩ¾¾Âôɱ¡¸¡ºº²Ê
 ¹âÈø¾»¼ù£±¡¤£²¡Ë¡¤ÀÄ»³ÀµÍΣ³¡Ë¡¤±ÊÅç佨£²¡Ë¡¤ÉÚÅÄ͵£²¡Ë¡¤ÀÐÀî¶ÖÌ飴¡Ë¡¤½©»³¼£É§£µ¡Ë¡¤ÅÚëˮ½©£¶¡Ë¡¤µÈÅÄ¡¡ÍÎÆ󣲡ˡ¤Èþ¸¶¡¡È×£²¡Ë
 

86 KNP

*Date¡§20070317
*Manager¡§Hideo Mori

1Severe involvement of the emotional limbic circuit in amyotrophic lateral sclerosis with dementia
 Departments of Neuropathology1) and Clinical Neuropathology2), Tokyo Metropolitan Institute for Neuroscience¡¢Department of Neurology, Tokyo Women¡Çs Medical University3)¡¢Department of Pathology, Faculty of Medicine, Kinki University4)¡¢Department of Neurology, Baba Memorial Hospital5)¡¤
 Takahiro Takeda1)3) , Toshiki Uchihara1), Taka-aki Chikugo4), Teiichi Hiraga5), Masataka Kitaguchi5) and Hideaki Kojima2)
 A woman first presented with progressive abnormal behaviors at the age of 46, followed by loss of social awareness, perseveration, dysarthria, dysphagia and muscle weakness a few years after the onset, all of which progressed until her death at the age of 53. Brain weight was 1,260g. Spongiosis and gliosis were moderate to severe in the fronto-temporal cortex and extended to the orbital gyrus and temporal tip in addition to the involvement of motor neurons. The medio-dorsal thalamic nuclei (MD) and amygdala were also involved. Ubiquitin-positive inclusions (UI) present in the fronto-temporal cortical neurons were also found in the MD and striatum. These inclusions in the striatum were eosinophilic on H&E staining. Moreover, preferential involvement of the orbital gyrus, temporal tip, amygdala and MD, each associated with UI, has not yet been well recognized in ALSD either. Because the complex of these structures is known as basolateral limbic circuit, its preferential involvement as we demonstrated in this patient will be helpful in interpreting the clinical picture of this condition. It is necessary to clarify whether these novel findings are shared with other patients with ALSD.
 
2Sialidase°äÅÁ»Ò¤ËÊÑ°Û¤ò¤â¤Äsialidosis type 1¤Î˶¸¡Îã
 1)ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡¿À·ÐÉÂÍý¡¤2)ÃæÌîÁí¹çɱ¡¡¡ÉÂÍý¡¤3)Åìµþ°å²Ê»õ²ÊÂç³Ø¡¡ÉÂÍý¡¤4)ÃæÌîÁí¹çɱ¡¡¡¿À·ÐÆâ²Ê
 Æ⸶½Óµ­1), Â綶·ò°ì2,3), ËÌÀî¾»¿­2,3), ÁÒÅÄÀ¹¿Í2,3), Ãæ¼°½»Ò1)¡¤¾®ÎÓ¹âµÁ4)
 
3Neuropathological study of a case of parkinsonian phenotype of SCA2 with Lewy body pathology
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology¡¤2) Dept. of Neurology, National Tokyo Hospital¡¤3) Department pf Pathology, National Tokyo Hospital¡¤
 Sakiyama Y.1), Yomono H.2), Kurisaki H.2), Saito Y.1), Hebisawa A.3), Murayama S.1)
 BACKGROUND: SCA2 is reported to be the most frequent in familial Parkinson disease (PD) in China. This is the first autopsy report of such a phenotype in Japan. CASE: A 75 year-old man at death, as well as his mother and son suffered from genetically confirmed SCA2. At age 50, he complained of unstable gait. At age 52, he received diagnosis of PD and medication of trihexyphenydil. At age 65, he presented with slow eye movement, in addition to typical parkinsonism. MIBG- cardiac scintigraphy demonstrated null uptake. He died of pneumonia at age 75. NEUROPATHOLOGY: Brain weighed 1,150 g. An old hemorrhage was seen in the left tegmentum of pons. The base of pons showed severe atrophy and loss of pigmentation in substantia nigra(SN) and locus ceruleus(LC) was remarkable. 1C2- immunoreactivity was detected in neuronal perikarya from anterior horn, Clarke's column, cerebellum, and dentate nucleus. In addition, Lewy body (LB) related alpha-synucleinopathy was immunohistochemically detected in LC, and dorsal motor nucleus of vagus, SN, limbic system, and unmyelinated axons in the heart. CONCLUSION: This case was interpreted to be SCA2 complicated by incidental LB disease.
 
4¸¶È¯ÁãÉÔÌÀ¤Ç¡¢¼ðáî¤ò·ÁÀ®¤»¤ºÃæ¿õ¿À·ÐÆâ¤Ë¿»½á¤·¤¿BºÙ˦À­°­À­¥ê¥ó¥Ñ¼ð¤Î1Îã
 1)ÅÔΩ¿À·Ðɱ¡¡¡Ç¾¿À·ÐÆâ²Ê¡¤2)ÅÔΩ¿À·Ðɱ¡¡¡¸¡ºº²ÊÉÂÍý¿ÇÃÇÉôÌ硤3)ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡Î×¾²¿À·ÐÉÂÍý¡¤4)Åìµþ°åÂçȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼¡¡ÉÂÍý¿ÇÃÇÉôÌ硤
 À¾Àî¡¡ÆàÄÅ»Ò1)¡¤ÈÄÅì¡¡½¼½©1)¡¤ÎÓ¡¡²íÀ²3)¡¤Ë¾·î¡¡ÍÕ»Ò2),ߧë¡¡À¿2)4),µÜËÜ¡¡Ï¿Í1)¡¤Àи¶¡¡¾´¿Í2),¿åë¡¡½Óͺ2)¡¤
 
5ξÇö«¤ÎÆó¼¡ÊÑÀ­¤òȼ¤¦Â¿È¯À­ÀÔ¿ñ¹¼ºÉ¤Î84ºÐÃËÀ­Ë¶¸¡Îã
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, 2) Department of pathology, Tokyo Metropolitan Geriatric Hospital, 3) Department of nephrology, Tokyo Metropolitan Geriatric Hospital
 Hatsuta H 1), Saito Y 1)2), Ymamaga R 3), Hamamatsu M 2), Sawabe M 2), Murayama S 1)
 
6Two cases of the acoustic nerve tumor
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan¡¤3Department of Pathology, Fujioka General Hospital, Gunma, Japan¡¤
 Atsushi Sasaki1, Shogo Ishiuchi2, Takatomo Yoshida3, Takuma Oishi1, Yoichi Nakazato1
 
7ÆñÄ°¤Çȯ¾É¤·¤¿ËýÀ­¿ñËì±ê¤Î64ºÐÃËÀ­Îã
 1) ½çŷƲÂç³ØǾ¿À·ÐÆâ²Ê¡¢2) ½çŷƲÂç³ØÉÕ°±Ûëɱ¡Ç¾¿À·ÐÆâ²Ê
 ¹âÍü²í»Ë 1)¡¢±ö·î´²Èþ 1)¡¢µ×Êݿ°ìϺ 1)¡¢¿¹¡¡½¨À¸ 2)
 
8Autopsy case of D-bifunctional protein deficiency
 Division of Child Neurology and Pathology, Kanagawa Children's Medical Center, Dept.of Genome Research, Life Science Research Center,Univ.of Gifu
 S.Yamashita,¡¡H.Osaka, S.Matsui, M.Tsuji, M.Iai, Y.Tanaka, Y.Sato, M.Tanaka, N.Shimozawa
 Case report: He was 1 year and 7 months old boy at the autopsy. His birth history was normal, but convulsions commenced at the age of 1 day and became intractable. He showed muscle hypotonia, few voluntary movements, no eye contact. EEG revealed frequent multifocal spikes, brain MRI showed polymicrogyria. Serum very long chain fatty acid was increased and cultured fibroblasts revealed deficiency of peroxisomal bifunctional protein. He deceased of central respiratory failure.¡¤Pathological findings: Both adrenal glands were atrophic and cortex cells were swollen. Total brain weigtht was 1126g, and normal for age. Cerebral cortical neurons showed disarrangement and polymicrogyria. Protoplasmic astrocytes were increased in number in the both cortex and white matter where the myelinaion was hypoplastic. The cerebellar cortex showed moderate loss of Purkinje cells and granular cells. The white matter showed severe astrocytic gliosis and perivascular cuffing. The neuronal cell loss was severe in dentate nucleus and olive.¡¤Discussion: This disorder is considered to be consisted of dysgenesis of cerebral cortical neurons and degenerative changes by the toxic effect of very long chain fatty acid.¡¤
 
9Cerebral cortical pathology in MERRF and MELAS¡¤
 1) Dept of Neurol Musashi Hosp, NCNP¡¤2) Dept of Lab Med
 Fumto Endo 1),Yasushi Oya 1),Miho Murata 1),Kunimasa Arima 2)
 Two autopsied patients with mitochondrial cytopathy were examined and similarities were found in cortical necrotic lesions between MERRF and MELAS.¡¤A 26-year-old woman with MERRF having A8344G mutation had cystic softening foci in the crest of inferior frontal, superior and middle temporal, and medial occipitotemporal gyri. The first cortical layer was involved in the necrotic lesions to some extent. There was patchy incomplete necrosis in the crest of orbital and middle frontal gyri. Incomplete necrosis was found in the gray matter of the thoracic cord.¡¤A 35-year-old man with MELAS having A3243G mutation had scattered cystic softening foci in the gyral crest in the posterior lateral tenporal, parietal, and occipital lobes. The first cortical layer was spared and constituted the cyst wall. Foci of incomplete laminar cortical necrosis were scattered, sometimes adjacent to cystic foci. The depth of the gyri appeared least involved. There were incomplete necrotic foci in the gray matter of thoracic spinal cord.¡¤
 

85 KNP

*Date¡§20070106
*Manager¡§Hiroko Nonaka

1An autopsy case of non-herpetic acute limbic encephalitis
 1ÅÔΩ¿À·Ðɱ¡¸¡ºº²ÊÉÂÍý¿ÇÃÇÉôÌ硤2ÅÔΩËÌÎÅ°é°åÎÅ¥»¥ó¥¿¡¼Æâ²Ê¡¤3ÅÔΩ¿À·Ðɱ¡Ç¾¿À·ÐÆâ²Ê¡¤4¹ñΩÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¾®»ù²Ê¡¤5Åìµþ°åÂçȬ²¦»Ò°åÎÅ¥»¥ó¥¿¡¼ÉÂÍý¿ÇÃÇÉô¡¤6ĹºêÂç³Ø°å³ØÉôÂè°ìÆâ²Ê¡¤7¹ñΩ´¶À÷¾É¸¦µæ½ê´¶À÷ÉÂÍýÉô¡¤8ÆüËÜÂç³Ø¿À·ÐÆâ²Ê¡¤
 Mochizuki Y, Mizutani T, Isozaki E, Ohtake T, Takahashi T, Shibuya M, Motomura M, Shiraisi Y, Sata T, Kamei S
 
2Èó¥Ø¥ë¥Ú¥¹À­µÞÀ­ÊÕ±ï·ÏǾ±ê¤ÎÉã̼Îã
 »°½ÅÂç³Ø°å³ØÉô¿À·ÐÆâ²Ê1¡¢µªÆîɱ¡¿À·ÐÆâ²Ê2¡¢¥È¥è¥¿µ­Ç°É±¡¿À·ÐÆâ²Ê3¡¢¡¤Ë̳¤Æ»Âç³Øʬ»ÒºÙ˦ÉÂÍý³Ø4¡¢µªÆîɱ¡³°²Ê¡¤
 Yasumasa Kokubo
 
3A Parkinson disease patient presenting progressive supranuclear palsy like clinical manifestations.
 1)Department of Neurology, Chigasaki Tokushukai General Hospital, 2)Department of Neurology, Makita General Hospital, 3)Department of Pathology, Chigasaki Tokushukai General Hospital, 4)Uchiyama Clinic
 Naoki Kasahata1)2), Yoshio Miyazawa3), Fujio Uchiyama4), Ikuo Torii1), Tetsumasa Kamei1)
 A 71-year-old female patient, suffered Parikinson diseae for two-years, was admitted to the hospital because of difficulty to standing, dysphagia, melena and anemia on September 27, 2003. Physical examination showed inspiratory stridor. Neurological examination revealed subcortical dementia, supranuclear ophthalmoplegia, pseudobulbar palsy, retroflexion of neck and postural refex disturbance. Although the dose of l-DOPA was increased, she became cardiopulmonary arrest state on the morning of October 20. CPR was performed, but her general condition became worse because of respiratory infection. She died on November 23.¡¤Clinical diagnosis: Parkinsonism (PSP s/o), hypoxic encephalopathy, pneumonia and gastric ulcer.¡¤Pathological findings: aspiration pneumonia with organization, gastric ulcer (scar) + gastrointestinal tract bleeding, atrophy of organs and malnutrition.¡¤Neuropathological findings: hypoxic encephalopathy (laminar sclerosis of cerebral cortex, pallidal necrosis and hypoxic change of cerebellum), Parkinson disease (or DLB: neuronal loss and Lewy bodies of substantia nigra, locus ceruleus and dorsal motor nucleus of Vagus).¡¤Question: clinico-pathological dissociation (responsible lesions for subcortical dementia, supranuclear ophthalmoplegia and retroflexion of neck).
 
4¸åº÷¤ÎÇ˲õ¡¦Ã¦¿ñ¤ò¼çÂΤȤ·¤¿¡¢·ÛÄǾÉÀ­ÀÔ¿ñ¾É¤Î92ºÐ½÷À­Ë¶¸¡Îã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê ϷǯÉÂ¥²¥Î¥à¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ ˶¸¡ÉÂÍý²Ê¡¤3)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÉí°¿ÇÎŽꡦ¥Ý¥¸¥È¥í¥ó¸¦µæ»ÜÀß¡¤4)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ À°·Á³°²Ê¡¤5)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ Êü¼ÍÀþ²Ê
 ½éÅÄ͵¹¬1)¡¢óîƣʹ»Ò1)2)¡¢Àа渭Æó3)¡¢·ê¿å°Í¿Í4)¡¢ÆÁ´Ý°¤Ìí5)¡¢ÂôÊÕ¸µ»Ê2)¡¢Â¼»³ÈËͺ1)
 
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Two cases of the sellar region tumor
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan
 Atsushi Sasaki1, Shogo Ishiuchi2, Hideaki Yokoo1, Junko Hirato1, Yoichi Nakazato1
 
6¥Ú¥ó¥È¥µ¥ó¥Ý¥ê¥µ¥ë¥Õ¥§¡¼¥ÈǾ¼¼Æâ»ý³ÅêÍ¿Ãæ¤Ë»àË´¤·¤¿¡¢¥¯¥í¥¤¥Ä¥Õ¥§¥ë¥È¥ä¥³¥ÖÉ¡ÊCJD¡Ë¤Î°ì˶¸¡Îã
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 ÀÐÄÅĪδ1¡¢ºê»³²÷É×2,3¡¢óîƣʹ»Ò2¡¢¾¾ËÜ¥ë¥ß¥Í3¡¢ÄÚ°æµÁÉ×4¡¢»³ÅÄãÉ×4¡¢ËÌËÜůǷ5¡¢Æ²±º¾¡Èþ6¡¢¼Øß·¾½7¡¢·ªºêÇî»Ê1¡¢Â¼»³ÈËͺ2¡¤
 
7¥Ú¥ó¥È¥µ¥ó¼£ÎŸå¤ÎCreutzfeldt-Jakob disease¡ÊCJD¡Ë¤Î°ìÎã
 ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤*ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤**Ê¡²¬Âç³ØÂè¸ÞÆâ²Ê
 »ûÅÄã¹°¡¢¾®ÈøÃÒ°ì¡¢¿ù±ºÌÀ¡¢»³ºê¸øÌé¡¢¹Â¸ý¸ù°ì¡¤*¼»³ÈËͺ¡¢*óîƣʹ»Ò¡¤**ÄÚ°æµÁÉס¢**»³ÅÄãÉ×
 
8¼ºÄ´À­Êâ¹Ô¤è¤ê½éȯ¤·¡¢Ëö¾¿¿À·Ð¾ã³²¡¢µ¯Î©À­Äã·ì°µ¤òǧ¤á¤¿·Ð²áÌó30ǯ¤Îspinopontine atrophy¤Î86ºÐÃËÀ­Îã
 £±µµÅÄÁí¹çɱ¡¿À·ÐÆâ²Ê¡¤£²¹ñÉÜÂæɱ¡¿À·ÐÆâ²Ê¡¤£³ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯
 ³ùÅÄÀµµª£±¡¤£²,Ê¿ÅĽ¨¼¤£±¡¢ÊÒ¿»ËÌÀ£±¡¢º´Æ£¿Ê£±¡¢Ê¡ÉðÉÒÉ×£±¡¤Â¼»³ÈËͺ£³
 
9°äÅÁÎò¤¬ÌÀ¤é¤«¤Ç¤Ê¤¯´Ë½ù¤Ê·Ð²á¤òé¤Ã¤¿Â¿·ÏÅý°à½Ì¾É¤È¿ÇÃǤµ¤ì¡¢Ë¶¸¡¤Ë¤ÆǾ´´¡¦¾®Ç¾¤Î°à½Ì¤È¼ç¤Ë¶¶³Ë¤Ë£²³Ë¿À·ÐºÙ˦¤¬Ç§¤á¤é¤ì¤¿ÉÂÍý¿ÇÃǤκ¤Æñ¤Ê°ìÎã
 ÅìµþÅÔÊÝ·ò°åΟø¼Ò±Á¸¶É±¡ ¸¡ºº²Ê(1)¡¢¿À·ÐÆâ²Ê(2)¡¢ÅÔΩ¶ð¹þɱ¡ ÉÂÍý²Ê(3)¡¢ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡¿À·ÐÉÂÍý³Ø¸¦µæÉôÌç(4)¡¤
 Á¥ÅÄ¿®¸²(1)(3)¡¤Ä¹Èøµ£É§(2)¡¢Æ⸶½Óµ­(4)¡¢¹â¶¶³Ø(1)¡¢²£ÃÏÀµÇ·(2)¡¤
 

84 KNP

*Date¡§20060722
*Manager¡§Tomohiko Mizutani

1A 23-year-old man with AML treated by chemotherapy and whole brain irradiation whose autopsy exhibits swollen axon-laden necrotic foci mainly located in the white matter of the cerebrum and brain stem.
 Departments of Pathology 1), Neurology 2) and Hematology 3), Toranomon Hospital¡¤ Neurology, Jichi Medical University School 4)¡¤
 Yasunori Ota 1), Yasushi Shiio 2), Hiroko Noguchi 1), Kenichi Ohashi 1), Naoyuki Uchida 3), Atsushi Wake 3), Shuichi Taniguchi 3), Madoka Yoshimura 2), Hirofumi Nakase 2), Imaharu Nakano 4)
 
2An autopsy case of amyotrophic lateral sclerosis presenting Bunina bodies, severe degeneration of the spinal anterolateral funiculus, marked gliosis of the subthalamic nucleus, iron deposition in the substantia nigra and degeneration of the spinal ganglia
 1) Dept. of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, 2) Dept. of Pathology, Tokyo Metropolitan Neurological Hospital, 3) Dept. of Neurology, Tokyo Metropolitan Neurological Hospital¡¤
 Tomoyo Hashimoto1), Kiyomitsu Oyanai1),2), Shiro Matsubara3),Yoko Mochizuki2), Toshio Mizutani2)
 
3An autopsy case of Alzheimer's disease clinically daignosed as Corticobasal degeneration
 1)Dept. of Neurology, Tokyo Metropolitan Neurological Hospital¡¤2)Dept. of Pathology Tokyo Metropolitan Neurological Hospital¡¤
 Miho Osako, Yoko Mochiduki, Yumiko Kugio, Toshio Mizutani, Hideaki Hayashi
 
4A 32-year-old female with left parietal tumor showing marked calcification
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan
 Atsushi Sasaki1, Shogo Ishiuchi2, Hayato Ikota1, Yoichi Nakazato1
 
5A brain biopsy case of a 24-year-old man presenting with acute encephalopathy due to multiple lesions in the cerebral white matter
 Departments of Neurology and Pathology, Nihon University School of Medicine
 Masaki Ishihara, Taku Honma, Kazuma Ishida, Satomi Toriumi, Tomoka Naganuma, Masahiko Sugitani, Norimichi Nemoto, Tomohiko Mizutani
 
6¥×¥ê¥ª¥óÃÁÇò°äÅÁ»Òcodon180¤ÎÅÀÊÑ°Û¡ÊV180I¡Ë¤Ècodon129¤ÎÀµ¾ï¿·¿¤òǧ¤á¤¿Creutzfeldt-JacobÉ¡ÊCJD¡Ë¤Î°ìÎã
 ÆüËÜ°å²ÊÂç³ØÉհɱ¡ Ï·¿Í²Ê¡¤Æ±¡¡Âè2Æâ²Ê*¡¤Æ± ÉÂÍýÉô**¡¤·²ÇÏÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÉÂÂÖÉÂÍý³ØʬÌî***
 ÎëÌÚ°ìÀ®¡¤ËÙÅÄÀµ·¼¡¢¾¾Â¼Åµ¾¼¡¤ÎëÌÚãÌ顤ÃæÌîÇî»Ê¡¤ÂçÄí·ú»°¡¤±Ê»³´²*¡¤ÊÒ»³ÂÙϯ*¡¤Åļ¹À°ì**¡¤¿ùºêÍ´°ì**¡¤²£Èø±ÑÌÀ***
 
7Adult type neuronal ceroido-lipofuscinosis. A case report.
 1) Department of Legal Medicine, School of Medicine, Keio University¡¤2) Department of Pathology, School of Medicine, Keio University¡¤3) Department of Neurology, Yomiuriland Keiyu Hospital¡¤4) Department of Neurology, Ashikaga Red Cross Hospital¡¤
 Takao M, Ikeda E, Fujita MQ, Koto A, Fukuuchi Y
 
8An autopsy case of frontotemporal dementia with ubiquitinated neuronal intracytoplasmic inclusions (FTD-U), complicated by severe cervical compression meylopathy
 1) Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology¡¤2) Department of Neurology, University of Tokyo¡¤3) Department of Pathology, Tokyo Metropolitan Geriatric Hospital¡¤4) Department of Neurology, Tokyo Metropolitan Geriatric Hospital¡¤5) Department of Radiology, Tokyo Metropolitan Geriatric Hospital¡¤6) Department of Clinical Pathology, Tokyo Metropolitan Geriatric Hospital¡¤
 Yoshio Sakiyama 1,2), Yuko Saito 1,3), Masako Ikemura 1), Renpei Sengoku 1), Hiroyuki Hatsuta 1), Kazutomi Kanemaru 4), Aya Tokumaru 5), Tomio Arai 6), Shigeo Murayama 1)
 A 61-year old man was first admitted to our hospital because of bronchitis. He had past history of ossification of posterior longitudinal ligament (OPLL) and severe cervical compression myelopathy since his forties. He underwent a decompression surgery with posterior approach at the age of 50. However, he experienced stepwise worsening and could not walk by himself at the admission. He presented with agitation, noncooperation and do as he will. MMSE 15/30 and HDS-R 25/30 showed discrepancy. MRI of the brain revealed left side-dominant moderate to severe atrophy of the medial temporal lobe, including hippocampus. MRI of the cervical spinal cord demonstrated with atrophy and myelomalacia of the cervical spinal cord. He received diagnosis of Pick¡Çs disease. He refused to admit to care facility and returned to his home, where he live alone. He maintained total bedridden state with volunteers¡Ç aid. At the age 63, he died of aspiration pneumonia. Autopsy was performed with postmortem interval of 41 hours. The brain weighed 1220g before fixation. Macroscopically, left dominant atrophy of the temporal pole was evident. Serial coronal sections showed cortical atrophy, accentuated in the medial temporal pole, amygdala, and entorhinal and transentorhinal cortex. The first temporal gyrus and hippocampal proper were relatively spared. Histologically, severe neuronal loss and gliosis, with loss of myelin in the subcortical white matter involved medial temporal pole, anterior entorhinal cortex and pes hippocampi, and accompanied abundant ubiquitinated neurites , threads¡¤and globules and scattered ubiquitinated neuronal intracytoplasmic inclusions (UIs), which were negative for Gallyas-Braak silver stain, or not immunoreactive to anti-phosphorylated tau or alpha-synuclein antibodies. The similar degeneration was present in the septum and nucleus accumbens, as well as the medial head of the caudate nucleus, and the lateral zone of the anterior putamen. UIs were abundant in the granular cell layer of the dentate gyrus. Motor cortex was spared and no definite neuronal loss was seen in hypoglossal and ambiguous nuclei. Severe compression cervical myelopathy, most prominent at C6/7 segment of the spinal cord, was observed. Neither Bunina body nor skein like inclusion was observed in the anterior horn of the lumbosacral spinal cord. This case was consistent with frontotemporal dementia with ubiquitinated inclusions. Paucity in degeneration of upper and lower motor neurons is distinct from Yuasa-Mitsuyama type amyotrophic lateral sclerosis (YMALS). However, presence of UIs and severe degeneration of anterior basal forebrain shares pathology with YMALS.
 
9
 
 
 

83 KNP

*Date¡§20060311
*Manager¡§Takahiro Fukuda

1An autopsy case of neurosyphilis with meningoencephalitis and Fischer¡Çs plaques
 1)Juntendo University Depertment of Neurology 2)Tokyo Metropolitan Matuzawa 3)Tokyo Institute of Psychiatry
 Kimiko Obi, Kuniaki Tuchiya, Ryousuke Nakamura, Midori Anno, Satoshi Ohota
 The Treponema (T) pallidum infection was once thought to be overcome after penicillin came into general use. In the last few decades, however, the disease has rather revived partly because of ignorance of the disease and the unchanged promiscuous habits of man. Neurosyphilis is a manifestation of late T pallidum infection. It is characterized by meningeal and perivascular inflammation with leukocyte infiltration in active stages. In later, more chronic stages, brain parenchyma is predominantly involved, which clinically exhibits dementia paralytica. Pathology of parenchymal neurosyphilis is characterized by neuronal cell loss and multiple focal demyelination described by Fischer. We here report the autopsy findings of a 59 yo man who died of neurosyphilis one and a half years after the clinical symptoms such as personality changes and disorientation appeared. Neuropathologically, this patient revealed meningeal and perivascular leukocyte infiltration together with Fischer¡Çs plaques particularly in the frontal cortex. Neuronal cell loss was not evident, however. Parenchymal involvement may occur in some atypical cases of the stage 3, meningo-vascular neurosyphilis.
 
2An autopsy case of neuro-Behcet's disease (NB) with fresh infarct-like lesion in the pontine base and arteritis in the prepontine cistern
 1)Dept of Neurol, Teikyo Univ Sch of Medicine, 2)Dept of Neurol, Tokyo Metro Hiroo Hosp, 3)Anatomy, and 5)Pathol, Teikyo Univ Sch of Medicine, 4)Dept of Neurol, Jichi Med Sch
 K Arai1)2), K Matsumura1), T Shimizu1), J Shiga3), I Nakano4), S. Mori5)
 A 64-year-old man presented with recurrent oral ulcers at age 56, and transient right abducens nerve palsy and delirium at age 62. An MRI then showed high intensity areas at the pontine tegmentum, bilateral thalamus and right temporal lobe. These findings combined with elevated CSF IL-6 activity (52.6pg/ml) strongly indicated NB as the diagnosis. Although methylprednisolone pulse therapy and oral low dose methotrexate therapy (10mg/week) were effective to the symptoms, MRI findings and CSF IL-6 activity, a dementia progressed. He died of sepsis after onset of dysarthria and ataxia and a CT revealing low density area at the left pontine base. The brain (1,430g) exhibited fresh infarct-like lesions in the pontine base, which were microscopically fresh necrotic lesions consonant with the perfusion area of the paramedian arteries. There were scattered perivascular lymphocytic cuffings. Some arteries in the prepontine cistern had marked infiltration of lymphocytes, histiocytes and plasma cells in the adventitia. The media was spared. Prominent foamy macrophages were observed in the intima. The pontine base changes seem to be characteristic features in this case.
 
3A 28-y.o. female biopsy-case with the tumor of right parietal lobe
 1Neuropathology, The Jikei University School of Medicine, 2Pathology and 3Neurosurgery, Atsugi Municipal Hospital
 Takahiro Fukuda1, Tadaaki Tokuda2, Shougo Kaku3
 A 28-year-old female complained of left leg weakness lasting a few years and was referred to our unit for generalized convulsion. Magnetic resonance (MR) revealed a right frontoparietal mass without the attachment on the dura and the communication to the lateral ventricle. A gross total resection was achieved. The patient¡Çs postoperative course was uneventful. The tumor was composed of uniform, closely packed cells with round, centrally siturated nuclei, delicate nucleoli, and small to moderate amounts of clear to focally eosinophilic cytoplasm. Both true rosette and perivascular pseudorosette were lacking. Immunohistochemical stains for GFAP demonstrated many fibrillated processes and a few cytoplasm of tumor cells, but stains for EMA was negative. Cytokeratin was identified immunocytochemically in many tumor cells. The ultrastructural microscopy revealed the intracellular microrosettes, cilia-like structure and many junctional complexes. The diagnosis is the clear cell ependymoma with the tanycytic component.
 
4Two cases of ganglioglioma
 Depts. of Human Pathology1 and Neurosurgery2, Gunma Univ. Graduate School of Medicine, Gunma and Dept. of Neurosurgery3, Tatebayashi Municipal Hospital, Gunma, Japan
 Atsushi Sasaki1, Akio Takahashi2, Osamu Miyagi3, Yoichi Nakazato1
 
5Old age onset intractable choreoballism associated with selective degenerationof subthalamic nuclei
 1 Division of Neurology, Kameda Medical Center¡¤2 Department of Neuropathology, Tokyo MetropolitanInstitute of Gerontrogy
 Hidehiro Shibayama1¡¤Shigeo Murayama2¡¤Toshio Fukutake1
 [Clinical Course] An 80 years old woman without pertinent history admitted on June 30 '00 for evaluation of involuntary movement (IM) of 5 weeks duration ensuing gait difficulty. Neurological examination revealed accelerated speech and choreoballistic IM of left leg, right leg, trunk, and left arm in order of frequency and severity. HDS-R was 21/30. Blood examination including thyroid function, serum cupper and ceruloplasmin turned out all normal. Cranial MRI disclosed nonspecific mild cortical atrophy. No malignant lesions were found on imaging studies. Medication like haloperidol up to 9mg/day, perphenadine 10mg/day showed only modest effect for IM. Cognitive decline and dysphagia progressed and she died of aspiration pneumonia on Feb 11 '05. [Neuropathology] Severe degeneration of bilateral subthalamic nuclei consisting of neronal loss and gliosis was noticed microscopically. In contrast, caudate nucleus, putamen, outer segment of globus pallidus, nucleus ruber, and dentate nucleus (mild glumose degeneration +) were well preserved. [Discussion] This case provides a new disease spectrum depending on the result of gene examination for dentaterubropallidoluysial atrophy now going on.
 
6An atuopsy case of 33-year old man with multifocal microscopic tuopathy,¡¡clinically presenting motor delay and severe involuntanry movement¡¤
 1)Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, 2)Department of Pathology, Tokyo Metropolitan Geriatric Hospital(TMGH), 3)Department of Neurology, TMGH¡¤¡¤
 Sengoku R1), Saito Y1)2), Mitani K3), Nishina Y3)¡¤Ikemura M1), Hatsuta H1), Sakiyama Y1), Sawabe M2), and Murayama S1)¡¤
 
7·Ð²áÃæÀµ¾ï°µ¿åƬ¾É¤¬µ¿¤ï¤ì¤¿¤¬¡¢¾É¾õ¤Î¿Ê¹Ô¤Ëȼ¤¤¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤È¿ÇÃǤµ¤ì¤¿79ºÐÃËÀ­Îã
 1) »ü·Ã²ñ°å²ÊÂç¿À·ÐÆâ²Ê¡¡2)Ʊ¿À·ÐÉÂÍý¡¡¡¤3)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êϷǯÉÂ¥²¥Î¥à¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤
 °ëÉô·òÉ×1)¡¡Æ£¥öºê½ã»Ò2)¡¡ºäËܹä1)¡¡Ê¡ÅÄδ¹À2)¡¡Â¼»³ÈËͺ3)¡¡°æ¾åÀ»·¼1)¡¡
 
8Î×¾²Åª¤ËÁ°Æ¬ÍÕ·¿Ç§Ãε¡Ç½Äã²¼¤òȼ¤¦¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É(ALS)¤ò¼¨¤·¡¢ÉÂÍý³ØŪ¤ËÓ϶äðùγ¤ò¹çÊ»¤·¤¿ALS¤ÎÉÂÍýÁü¤òÄ褷¤¿°ì˶¸¡Îã
 1)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼Ë¶¸¡ÉÂÍý²Ê¡¢2)ÅìµþÅÔÏ·¿Í¸¦µæ½ê¡¡Ï·¿ÍÉ¤Υ²¥Î¥à²òÀÏ(¿À·ÐÉÂÍý)¡¢3)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê
 ÃÓ¼²í»Ò1)2)¡¢óîƣʹ»Ò1)2)¡¢Â¼¾å´îÀ¸3)¡¢½éÅÄ͵¹¬2)¡¢ÀçÀÐϣʿ2)¡¢ºê»³²÷É×2)¡¢¿·°æÉÚÀ¸1)¡¢ÂôÊÕ¸µ»Ê1)¡¢Â¼»³ÈËͺ2)
 
9An autopsy case of 79 years old male with Parkinsonism, dementia and motor neuron disease¡¤
 Departments of Pathology Japanese Red Cross Medical Center (1), Medical Research Institute, Tokyo Medical and Dental University (2)¡¤
 Toshihiko Kuroiwa(1,2), Hideji Hashida(1), Mutunori Fujiwara(1), Katsuhiko Takeda(1), Tamiko Takemura(1)¡¤
 A 78 years old male showed Parkinsonian gait, memory disturbances, bilateral muscle weakness¡¥CT scan revealed brain atrophy and EMG lower motor disturbances. The patient died of dyspnea 1 year after onset of the symptoms. Family history was negative. Brain was mildly atrophic. Neuronal loss of the hypoglossal nucleus and spinal anterior horn with gliosis, demyelination of the corticospinal tract and senile plaque with neurofibrillary tangle formation was found. Neuronal loss of substantia nigra was mild. Tufted astrocyte (-), Astrocytic plaque (-), Pick body (-), Glial cytoplasmic inclusion (-)¡¤
 

82 KNP

*Date¡§20060107
*Manager¡§Toshihiko Kuroiwa

1An autopsy case of amyotrophic lateral sclerosis with unique cystatin C immunoreactivity
 £±¡ËÅìµþ°å²Ê»õ²ÊÂç³Ø¿À·ÐÆâ²Ê¡¢£²¡Ë¡¤Åìµþ°å²Ê»õ²ÊÂç³Ø»õ³ØÉô¸ý¹ÐÉÂÍý¡¢£³¡ËÅìµþ°å²Ê»õ²ÊÂç³ØÆñ¼£¼À´µ¸¦µæ½ê¿À·ÐÉÂÍý
 Kinya Ishikawa
 
2Angiotrophic lymphoma, presenting with multiple hemorrhages and infarcts mainly involving the cerebral white matter
 Department of Neurology, Kameda General Hospital, Department of Neurology, Tokyo Metropolitan Institute of Gerontology
 Masanori Kamata, Hideji Hirata, Shin Nishie, Susumu Sato, Hidehiro Shibayama, Toshio Fukutake and Shigeo Murayama
 
3¶µ°é¾ÉÎã
An autopsy case of glioblastoma with extensive, diffuse extension in the brain
 Departments of Human Pathology, Gunma University Graduate School of Medicine,¡¤Gunma, Japa
 Atsushi Sasaki, Yuko Yamane, Yoichi Nakazato
 
4¶µ°é¹Ö±é
Surgical pathology of epilepsy: microdysgenesis
 Tokyo Metropolitan Institute of Gerontology
 Arai, Nobutaka
 
5A long-term follow-up autopsy case of neuro-Behcet disease presenting with ring¡¤enhancement in the pontine base by MR imaging
 1)Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤2)Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology,¡¤Tokyo, Japan¡¤3)Department of Neurology, , Tokyo Metropolitan Geriatric Hospital, Tokyo,¡¤Japan¡¤4)Human Tissue Research Group, Tokyo Metropolitan Institute of Gerontology,¡¤Tokyo, Japan¡¤5)Department of Radiology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan¡¤6)Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan
 Yoshio Sakiyama1),2), Yuko Saito1), 2), Takahiro Saito3), Masako Ikemura2), Hiroyuki¡¤Hatsuta2), Renpei Sengoku2), Masatoshi Yoshino3), Junko Aida4), Aya Tokumaru5), Kenji Ishii6), Motoji Sawabe1), Shigeo Murayama2)
 A 65-year old man first presented with multiple aphthous ulcers in the mouth¡¤and painful erythema nodosum on the legs. At the age of 70, he was admitted to¡¤our hospital because of headache, character change and gait disturbance. He¡¤had the bruise-like rash on the face, folliculitis on the arms, old ulcers in¡¤the scrotum, in addition to the painful erythema nodosum on the legs.¡¤Neurological examination disclosed euphoric and childish character and right¡¤hemiparesis. MRI showed ring enhancement in the left pontine base and the T2¡¤high intensity lesion in the left occipital lobe. PET studies presented with¡¤severe decrease in glucose hypometabolism but preserved or increased uptake of¡¤methionine in the area. Pleocytosis and elevated IL6 level were detected in¡¤the cerebrospinal fluid. He showed good response to high-dose prednisolone¡¤therapy. He had not experienced overt relapse until he died of aspiration¡¤pneumonia at the age of 80. The autopsy was done with postmortem interval of 8¡¤hours. The brain weighed 1086g before fixation. Macroscopically, multiple¡¤brownish atrophic lesions involved the left base of pons, midbrain, mammillary¡¤bodies, hypothalamus, internal capsule on globus pallidus, and left¡¤parieto-occipital lobe. Histologically, the lesions presented with¡¤demyelination and remyelination, accompanying axonal loss, deposition of¡¤hemosiderin, and lymphocytic cuffing around small vessels. Inflammatory cells¡¤focally infiltrated into leptomeninges, posterior horn, spinal nerve roots, and¡¤dorsal root ganglia. The morphological features were consistent with Behcet¡¤disease. The smoldering nature of the disease may be responsible for chronic¡¤fatal decline in this patient.
 
6ÆÃÊ̶µ°é¹Ö±é
Neuro-Behcet disease
 Department of Allegy and Collagen disease, Teikyo University
 Hirohata, Shunsei
 
7¿·Èé¼Á·¿Lewy¾®Âη¿ÃÔÊòÉÂÊѤÈÀг¥ÄÀÃå¤òȼ¤¦¤Ó¤Þ¤óÀ­¿À·Ð¸¶Àþ°ÝÊѲ½ÉÂ(DNTC)Îà»÷¤ÎÉÂÍý½ê¸«¤òÄ褷¤¿1˶¸¡Îã
 1)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê¡¡Ï·¿ÍÉÂ¥²¥Î¥à¡¦¹âÎð¼Ô¥Ö¥ì¥¤¥ó¥Ð¥ó¥¯¡¤2)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ÉÂÍýÉô¡¤3)ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼´¶À÷¾É²Ê
 ÃÓ¼²í»Ò1)¡¢óîƣʹ»Ò1)2)¡¢ÁýÅĵÁ½Å3)¡¢½éÅÄ͵¹¬1)¡¢ÀçÀÐϣʿ1)¡¢ºê»³²÷É×1)¡¢Â¼»³ÈËͺ1)
 
8A 62-year-old man with atrophy of the caudate and neurodegeneration of the substantia nigra who presented stuttering, dementia and parkinsonism.
 Department of Neurology Juntendo University School of Medicine, Zushi Aoki Hospital
 Yuji Tomizawa, Masashi Takanashi, Kazuyuki Noda, Sadako Sumino, Yoshikuni Mizuno, Hideo Mori
 

81 KNP

*Date¡§20050730
*Manager¡§Hiroko Nonaka

1PEHO¾É¸õ·²¤È¿ÇÃǤµ¤ì¤¿Ãæ¿õ¿À·Ð·ÏÊÑÀ­¼À´µ¤Î£±Ë¶¸¡Îã
 £±¡ËÅìµþ°å²ÊÂç³ØË¡°å³Ø¶µ¼¼¡¢£²¡ËÅìµþÅÔ´Æ»¡°å̳±¡¡¢£³¡ËÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êϷǯÉÂ¥²¥Î¥à¡Ê¿À·ÐÉÂÍý¡Ë£´¡ËÅìµþ½÷»Ò°åÂç¾®»ù²Ê
 ¿å¾å¡¡ÁÏ£±¡Ë¡¢£²¡Ë¡¢óîƣʹ»Ò£³¡Ë¡¢¾®Ô¢¹°ÎÌ£´¡Ë¡¢ßÀ¾¾¾½É§£²¡Ë¡¢Ã滳ÃÒ¹¨£´¡Ë¡¢¸¶¡¡½¤°ì£±¡Ë¡¢Â¼»³ÈËͺ£³¡Ë¡¢±óƣǤɧ£±¡Ë¡¢£²¡Ë
 
2An autopsy case of 23 y.o. male with megalencephaly and leukoencephalopathy
 1 Neuropathology, 2 Pediatrics, 3 Pathology, clinical service, The Jikei Univ. Schl. of Med.
 Fukuda T1, Itoh T2, Kato H3, Eto Y2
 
3¶µ°é¹Ö±é
A case of atypical meningioma with extracranial extension
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan
 Atsushi Sasaki1, Yasuo Aihara2, Shogo Ishiuchi2, Yoichi Nakazato1
 
4An autopsy case of mantle-cell lymphoma with clinical diagnosis of metastatisis to falx, directly infiltrating into the anterior cingulated gyrus
 Departments of 1. Hematology and Oncology, 2. Neurology, and 3. Pathology, Kameda General Hospital, and 4. Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology
 Mihoko Koseki1, Hideji Hirata2, Takao Matsuei1, Makoto Narita3, Toshio Fukutake2 and Shigeo Murayama4
 
5¶µ°é¹Ö±é
An autopsy case of pathologically presenting with the phenotype of CADASIL
 ¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼¡¡Éð¢É±¡¡¡Î×¾²¸¡ººÉô
 Arima, Kunimasa
 
6An autopsy case of cerebral type Lewy body disease
 1) Department of Psychogeriatrics, Tokyo Institute of Psychiatry, 2) Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital
 Osamu Yokota 1), Kuniaki Tsuchiya 1,2), Haruhiko Akiyama 1)
 We present an autopsy case of the cerebral type of Lewy body disease. A 49-year-old man first showed a decline in efficiency at work, and subsequently, obvious anxiety and irritability. At age 51, he presented with obvious intellectual impairment, slight dysarthria, and rigidity in his upper extremities and neck without tremor. The clinical diagnosis at this time was unclassified dementia with very mild parkinsonism. However, parkinsonism was not detected at subsequent neurological assessments, and movement in his daily life was not disturbed throughout the course. At age 53, a depressive mood and agitation became obvious, and he was admitted to a psychiatric hospital. At age 55, his Hasegawa dementia scale-revised score was 7 points. He died at the age 56. The brain weighed 1,300 g. Macroscopically, the substantia nigra did not show depigmentation, but the locus ceruleus showed mild pallor. The most remarkable histopathological finding was numerous alpha-synuclein-positive Lewy bodies and Lewy neuritis predominantly in the amygdala and insula, cingulate, and temporal cortices, and to lesser degree but not negligibly, in the substantia nigra, locus ceruleus, and dorsal vagal nucleus. The neuronal population was reduced most prominently in the amygdala and insula and cingulate cortices. In contrast, neurons were relatively well preserved in the substantia nigra, locus ceruleus, and dorsal vagal nucleus. In addition, severe Alzheimer¡Çs disease pathology was superimposed on the Lewy pathology: the Braak stages of neurofibrillary changes and senile plaque were stages V and C, respectively. Combined, these findings suggest that this case corresponded to cerebral type Lewy body disease in addition to neocortical type dementia with Lewy bodies (DLB). This case suggests the possibility that in at least some DLB cases, Lewy pathology occurs in the cerebrum, especially in the limbic system, before its development in the brainstem, an expansion pattern that is inconsistent with the Lewy pathology stages proposed by Braak et al.
 
7A biopsy of 70-year-old man of recurrent encephalopathy showed astrocytes abnormalities.
 1) Department of Neurology of Japanese Red Cross Medical Center¡¤2) Department of Pathology of Japanese Red Cross Medical Center¡¤3) Research Institute, Tokyo Medical and Dental Univ
 Meiko Hashimoto 1), Toshihiko Kuroiwa 2), 3), Tamiko Takemura 2), Satoko Unno 1), Hideji Hashida 1), Katsuhiko Takeda 1)
 At the age of 60, a Japanese right-handed male patient was admitted to a hospital in Australia because of consciousness disturbance, high fever, and headache. Then he showed aphasia and behavior abnormalities. Brain MRI T2 wighted images (T2WI) showed high intensities areas in the left temporal lobe. These areas were enhanced with gadrinium. He improved gradually without any deficits. At the age of 70, he showed conscious disturbances once again. On admission, he showed left hemiparesis, grand mal epilepsy, and psychiatric symptom such as hallucination. MRI T2WI showed high intensity areas in the entire right hemisphere. A brain biopsy was performed at the right frontal and parietal lobe. Edema in the white matter, diffuse astrocytosis with atypical astrocytes showing markedly swollen pale nuclei mainly in the white matter and mild loss of cortical neuron were observed. The nuclei often have eosinophilic granular structures. There was small lymphocyte infiltration in the subarachnoid and perivascular. The observed astrocytic change may have a causative role in the pathogenesis of this case of a recurrent encephalopathy.
 
8Neuropathological correlation with diffusion tensor image in a case of amyotrophic lateral sclerosis with prominent alteration of the upper motor neurons
 1. Department of Neurology, Shizuoka Insitute of Epilepsy and Neurological Disorders, 2. Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, 3. Department of Radiology, the University of Tokyo Hospital
 Kimiya Yamazaki1, Tomokazu Obi1, Kouiti Mizoguchi1, Ryuu Kuroda1, Tatsuhiro Terada1, Yuuko Saito2, Shigeki Aoki3 and Shigeo Murayama2
 
9An autopsy case of l-dopa-responsive hemiparkinsonism of forty-year clinical course, pathologically presenting with commensurate laterality in the degeneration of substantia nigra
 Department of Neuropathology(The Brain Bank for Aging Research), Tokyo Metropolitan Institute of Gerontology¡¤
 Sengoku R, Saito Y, Sakiyama Y, Ikemura M, Hatsuta H, and Murayama S
 A man of his thirties first presented with resting tremor of the left hand. The symptoms very slowly exacerbated and he experienced accelerating gait at age 69. L-dopa was effective to the resting tremor. He was complicated by cerebral hemorrhage and died at age 73. The postmortem examination showed degenerative pathology restricted to substantia nigra (SN). The degeneration exhibited with ventrolateral gradience and marked right-sided predominancy. Remaining neurons frequently showed decrease in melanin pigment. Neither Lewy body nor neurofibrillary tangle was present. The cytopathological features resemble those of PARK2. The genetic study is now on going.
 

80 KNP

*Date¡§20050319
*Manager¡§Tomohiko Mizutani

1An case of 4-month-old female baby with bilateral putaminal hemorrhage
 1 Neuropathology, The Jikei Univ. Schl. of Med.¡¤2 Dept of Legal Med., Dokkyo Univ. Schl. of Med.
 Fukuda T1, Kido M2, Kurosu A2, Hitosugi M2, Tokutome S2
 
2Eight-months-old infant of pontine hypoplasia
 Division of Child Neurology1, Division of Neonatology2, Division of Pathology3 Kanagawa Children's Medical Center. Laboratory of Molecular Neurobiology, Dept. of Developmental Biology and Neurosciences, Graduate School of Sciences, Univ. of Tohoku
 Sumimasa Yamashita1,Hitoshi Osaka1, Makiko Ooyama2, Mio Tanaka3, Yukiti Tanaka3, Yuji Watanabe4
 This male infant was born at term. He was transferred to our neonatal care unit because of a transient respiratory disturbance after mild birth asphyxia. He showed severe increase of muscle tonus and opisthotonic posture, bilateral facial nerve palsy and lack of eye movements. MRI revealed hypolasia of pons and cerebellum. He died of encephalopathy at the age of 8 months. Autopsy findings were the followings. The middle part of the pons became extremely narrow, consisted of only pyramidal tracts and no cranial nerve nuclei were observed. The lower part was surrounded by the thick glial tissue layers. The olivary nuclei were hypolastic and the dentate nuclei were dysplastic. The middle and inferior cerebellar peduncles were not formed. The structures of the midbrain were normal. There were no abnormal cellular architectures in the cerebrum and spinal cord except for mild gliosis. We considered the abnormalities of the anterior hindbrain organizer gene, Unplugged (Gbx2), as a pathogenesis of this case. The genome DNA, however, revealed a normal structure.
 
3¶µ°é¾ÉÎã
Two cases of pineal region tumors
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
 1AtsushiSasaki, 2Keishi Horiguchi, 1Yoichi Nakazato
 
4Diagnostic Slide Session
Diagnostic Slide Session: 64ºÐ¤Î½÷À­Îã
 ÆüÂç¿À·ÐÆâ²Ê1¡Ë¡¤°°Ãæ±ûɱ¡¿À·ÐÆâ²Ê2)¡¤°°Ãæ±ûɱ¡Î×¾²ÉÂÍý²Ê3¡Ë
 ¿åëÃÒɧ
 
5An autopsy case of progressive cerebellar ataxia associated with anti-glutamic acid decarboxylase antibody
 Department of Neurology, Tamagawa hospital
 Kazuyuki Ishida (Dpt. of Neurol., Tamagawa Hosp.), Hiroshi Mitoma (Mitoma Neurol. Clin.), Yoshiaki Wada (Dpt. of Rehab., Tamagawa Hosp.), Teruaki Oka (Dpt of Pathol., Kanto Central Hosp.), Jyunji Shibahara (Dpt. of Pathol., Univ. of Tokyo), Yuko Saito (Dpt. of Neuropathol., Tokyo Metro. Inst. of Gerontol.), Shigeo Murayama (Dpt. of Neuropathol., Tokyo Metro. Inst. of Gerontol.), Hidehiro Mizusawa (Dpt. of Neurol., Tokyo Med. and Dent. Univ.)
 Anti-glutamic acid decarboxylase (GAD) antibody is considered to be causative for progressive cerebellar ataxia (PCA), stiff-person syndrome (SPS) and slowly progressive insulin-dependent diabetes mellitus (SPIDDM). A 66-year-old woman presenting with PCA developed hyperglycemia seven months after the onset of PCA. A diagnosis of SPIDDM with anti-GAD antibody was made. At age 70, painful spasm and rigidity of trunk mimicking SPS occurred and subsided spontaneously in two months. At age 71, she died of pneumonia. Neuropathologically, there was selective total depletion of Purkinje cells, a subset of GAD-containing neurons. Loss of other GAD-containing neurons, including basket cells and Renshaw cells, was not clear. Using whole-cell recording, IgG in the cerebrospinal fluid from this patient selectively suppressed inhibitory postsynaptic currents in Purkinje cells mediated by GABA (Ann Neurol 1999;46:263). Thus, Purkinje cells were excessively activated by anti-GAD antibody, leading to neuronal death. The autopsy finding of loss of Purkinje cells seems to agree with the result of whole-cell recording. As far as we know, this is the first autopsy case of this disorder.
 
6An autopsy case of multiple system atrophy characterized by precentral atrop¡¤hy and remarkable histological findings
 1Dept. of Pathol., and 2Dept. of Neurol., Tokyo Met. Neurol. Hosp
 Mochizuki Y1, Yamaoka Y2, Kawata A2, Isozaki E2, Mizutani T1, Hayashi H2
 
7²È²À­¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¤ò¼¨¤·¤¿¥¿¥¦N279KÊÑ°ÛÎã
 ½çŷƲÂç³Ø°å³ØÉô¡¡Ç¾¿À·ÐÆâ²Ê¡¤½éÀÐɱ¡¡¡¿À·ÐÆâ²Ê
 ¹âÍü²í»Ë¡¢¾®¾¾ºêȬ¼÷»Ò¡¢ÈÄë¾»»Ò,¡¤ËÜ°æ¤æ¤ß»Ò,¿åÌîÈþË®¡¢¿¹¡¡½¨À¸
 
8ÃÔÊò¡¢Êâ¹Ô¾ã³²¡¢¼«È¯À­Äã²¼¤Ê¤É¤òÄ褷¤¿Binswanger·¿Çò¼ÁǾ¾É
 ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø1)¡¢ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê2)¡¢ÅìµþÂç³Ø°å³ØÉô¿À·ÐÆâ²Ê3)
 ßÀÅIJí1)3)¡¢óîƣʹ»Ò1)¡¢»³Ç·ÆâÇî2)¡¢Â¼»³ÈËͺ1)
 
9An autopsied case of generalized variant of Pick¡Çs disease (Munoz-Garcia) presenting with severe pyramidal tract degeneration and widespread lesion in the central nervous system
 Department of Neurology, Usihoda General Hospital1), Kawasaki Coop Hospital2), Showa University School of Medicine3), Jichi Medical School4)
 Ishihara K1), Shiota J1), Araki S2), Kawamura M,3) Nakano I4)
 

79 KNP

*Date¡§20041113
*Manager¡§Takahiro Fukuda

1¶µ°é±éÂê
A case of pilocytic astrocytoma
 ·²ÇÏÂç³ØÂç³Ø±¡°å³Ø·Ï¸¦µæ²ÊÉÂÂÖÉÂÍý³Ø1¡¢Ç¾ÀÔ¿ñÉÂÂÖ³°²Ê³Ø2
 1º´¡¹ÌÚ¡¡Æס¢2ÀÐÆ⾡¸ã¡¢1ÃæΤÍΰì
 
2Four-repeat tauopathy with numerous Pick body-like inclusions and glial inclusions
 1) Department of Neurology, Juntendo University School of Medicine¡¤2) Department of Molecular Neurobiology, Tokyo Institute of Psychiatry¡¤3) Fifth Department of Internal Medicine, Fukuoka University¡¤
 Yumiko Motoi1), Masako Itaya1), Masato Hasegawa2), Tomonori Kobayashi3), Masashi Takanashi1), Yoshikuni 1), Hideo Mori 1)
 A 63-year-old woman developed parkinsonism, character change and antisocial behavior. She died after 13-years of illness. Neuropathologic examination revealed marked frontotemporal atrophy and numerous Pick body-like inclusions and glial inclusions in the cerebral cortices and subcortical nuclei. Western blotting of sarkosyl-insoluble tau showed the accumulation of four-repeat tau.
 
3³¤ÇϹŲ½¤ò¼¨¤·¤¿¥¢¥ë¥Ä¥Ï¥¤¥Þ¡¼É¤Σ±Îã
 1)µµÅÄÁí¹çɱ¡¡¢2)ÅìµþÏ·¿ÍÁí¹ç¸¦µæ½ê
 ³ùÅÄÀµµª1)¡¢¼Æ»³½¨Çî1)¡¢Ê¡ÉðÉÒÉ×1)¡¢Â¼»³ÈËͺ2)
 
4Diagnostic slide session
20ºÐ»þ¤Ë¿¶Àï¤Çȯ¾É¤·¡¤¾®Ç¾¼ºÄ´¡¤¥Ñ¡¼¥­¥ó¥½¥Ë¥º¥à¡¤Ç§Ãε¡Ç½¾ã³²¤Ê¤É¿ºÌ¤Ê¾É¸õ¤ò¤­¤¿¤·¤¿¾ÉÎã
 ·ÄØæµÁ½ÎÂç³Ø°å³ØÉô¿À·ÐÆâ²Ê
 ¹âÈø¾»¼ù¡¡¤Û¤«¡¥
 
5¹âIgM¾É¸õ·²¤Ë¹çÊ»¤·¤¿¿Ê¹ÔÀ­Â¿ÁãÀ­Çò¼ÁǾ¾É¤Î£±Ë¶¸¡Îã ¡Á·Ð»þŪMRI½ê¸«¤ÎÊѲ½¤È˶¸¡½ê¸«¤ÎÂбþ¡Á
 ÀŲ¬¤Æ¤ó¤«¤ó¡¦¿À·Ð°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê£±¡ËÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø£²¡Ë
 ¹õÅÄζ£±¡Ë»ûÅÄã¹°£±¡Ë»³ºê¸øÌ飱¡Ë¾®ÈøÃҰ죱¡Ë¹Â¸ý¸ù°ì£±¡Ë¼»³ÈËͺ£²¡Ë
 
6Spinal cord infarction with chief complaint of limb pain
 *1 Dpt. of Rehabilitation, Tokyo Metropolitan Geriatric Hospital, *2 Dpt. of Pathology, *3 Dpt. of Neurology, *4 Dpt. of Neuropathology, Tokyo Metropolitan Institute of Gerontology
 Kato, T., Saito, Y., Kasahara, I., Yamanouchi, H., Murayama, S.
 
7An autopsy case of "heat stroke" presenting with atypical clinical¡¤features: with special reference to differentiation from "malignant¡¤syndrome".
 1)ÅìµþÅÔΩ¾¾Âôɱ¡¡¡2)ÅìµþÅÔÀº¿À°å³ØÁí¹ç¸¦µæ½ê
 ¿·Î¤Ï¹°1)¡¡ÅÚëˮ½©1)¡¡ÂçÅç·ò°ì1)¡¡Ãæ¼μ²ð1)¡¡¾åÌ¼ù1)¡¡½©»³¼£É§2)
 
8¶µ°é±éÂê
¤Æ¤ó¤«¤ó¾ÇÅÀÉô¤Ë¤ª¤±¤ëÈù¾®·ÁÀ®ÉÔÁ´¡Êmicrodysgenesis¡Ë¤Ë¤Ä¤¤¤Æ
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡Î×¾²¿À·ÐÉÂÍý¸¦µæÉôÌç¡¡
 ¿·°æ¿®Î´
 

78 KNP

*Date¡§20040724
*Manager¡§Toshihiko Kuroiwa

1An autopsy case of cerebral amyloid angiopathy with epilepsy and various mental and neurological symptoms
 Departments of Pathology(1) and Neurosurgery(2), Japanese Red Cross Medical Center, Department of Neuropathology, Tokyo Medical and Dental University(3), Department of Neurology, Kanazawa Univiersity(4) Department of Neuropathology, Tokyo Medical and Dental University
 Toshihiko Kuroiwa(1,3), Mayumi Kubota(2), Shunsuke Ichi(2), Ichiro Suzuki(2), Mutunori Fujiwara(1),Masahito Yamada(4), Tamiko Takemura(1)
 A 63 years old Japanese male suffered from various mental and neurological symptoms including memory disturbances, disorientation and decreased consciousness level for a half year before exitus. Biopsy performed at the neurological deterioration revealed amyloid vessels with mild peivascular lymphocytic infiltration. Autopsy revealed cerebral amyloid angiopathy with multiple microinfarcts of the brain.
 
2An autopsied case of MV2 type Creuzfeldt-Jakob disease presenting with severe status spongiosus in the cerebral cortex
 Dept of Neeurol., Ushioda General Hospital1¡Ë¡¡Dert. of Neurol., Showa Univ. Sch. of Med.2¡Ë¡¡Graduate Sch. Tohoku Univ.3¡Ë¡¡Jichi Med. Sch.4¡Ë ¡¤
 Keinji ISHIHARA1¡Ë¡¡Jun'ichi SHIOTA1¡Ë¡¡Masayuki SUGIE2¡Ë¡¡Mitsuru KAWAMURA2¡Ë¡¡Tetsuyuki KITAMOTO3¡Ë¡¡Imaharu NAKANO4¡Ë ¡¤
 
3An Autopsy Case of Progressive Multifocal Leukoencephalopathy without Obvious Underlying Diseases
 1)Tokyo Metropolitan Matsuzawa Hospital 2)Tokyo Institute of Psychiatry
 Oshima K 1), Tsuchiya K 1), Niizato K 1), Akiyama H 2)
 
4¶µ°é±éÂê
Two cases of medulloblastoma
 Departments of 1Human Pathology and 2Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
 1Atsushi Sasaki, 2Keishi Horiguchi, 1Hideaki Yokoo, 1Yoichi Nakazato
 
5Ëö¾¿À­¿ñ¾äºÆÀ¸¤ò¹­ÈϤËǧ¤á¤¿·Ð²á19ǯ,ºÆȯ´²²ò·¿Â¿È¯À­¹Å²½¾É,51ºÐ½÷À­Îã
 1)ÅìµþÂç³Ø°å³ØÉôÉհɱ¡¿À·ÐÆâ²Ê¡¤2)²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê¡¤3)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø
 Å츶¿¿Æà1), Ã滳µ®Çî2), Ã濹Ãε£2), º£Ê¡°ìϺ2), óîƣʹ»Ò3), ¼»³ÈËͺ3)
 
6ÃÔÊò¤òȼ¤¦¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É¤¬µ¿¤ï¤ì¡¤Á´·Ð²á11ǯ¤Ç»àË´¤·¤¿74ºÐÃËÀ­Pickɤΰì˶¸¡Îã
 1ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ ¿À·ÐÆâ²Ê¡¤2ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½ê Ï·²½Î×¾²¿À·Ð²Ê³Ø
 ¡û¿Î²Ê °ìδ1¡¢óîÆ£ Í´»Ò2¡¢¶â´Ý ÏÂÉÙ1¡¢»³Ç·Æâ Çî1¡¢Â¼»³ ÈËͺ2¡¤
 
7A rare autopsied case diagnosed as Primary malignant lymphoma that has been diagnosed as toxoplasmosis clinically¡¤
 Tokyo medical and dental university
 Takashi Matsumoto
 
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ÊÒ¦µðǾ¾É¡Êhemimegalencephaly¡Ë¤Î³°²Ê¼ê½ÑÎ㡤
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½êÎ×¾²¿À·ÐÉÂÍý¸¦µæÉôÌç
 ¿·°æ¿®Î´
 
9A case of anti-Hu antibody-positive paraneoplastic syndrome (ganglioradiculopathy) in a 84-year-old patient with small cell lung carcinoma
 Division of Neurology, Department of Medicine, and Department of Pathology, Nihon University School of Medicine, and Department of Neurology, Brain Institute, Niigata University
 ËÜ´Ö¡¡Âö¡¢»°ÌÚ·ò»Ê¡¤
 

77 KNP

*Date¡§20040327
*Manager¡§Hideo Mori

1¹¥±ö´ðÀ­ÉõÆþÂΤòȼ¤Ã¤¿¡¤¶Ú°à½ÌÀ­Â¦º÷¹Å²½¾É32ºÐÃËÀ­Îã
 1)²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê,2)ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø¸¦µæ¥°¥ë¡¼¥×¡¤
 ²¬ºê¤ß¤µ¤­¡¡Ã滳µ®Çî¡¡Ã濹ͧµ£¡¡º£Ê¡°ìϺ 1)¼»³ÈËͺ2)¡¤
 
2ÃÔÊò¤òÄ褷ÂçǾÈé¼Á°à½Ì¤¬¸«¤é¤ì¤¿¿Ê¹ÔÀ­³Ë¾åÀ­Ëãáã¤Î£±Ë¶¸¡Îã
 1. Åìµþ°å²Ê»õ²ÊÂç³Ø°å³ØÉôÉÂÍý¡¤2. Åìµþ°å²Ê»õ²ÊÂç³Ø°å³ØÉô¿À·ÐÆâ²Ê¡¤3. ¹ñΩɱ¡ÅìµþºÒ³²°åÎÅ¥»¥ó¥¿¡¼¡¡¿À·ÐÆâ²Ê¡¤4. Åìµþ°å²Ê»õ²ÊÂç³ØÆñ¼£¼À´µ¸¦µæ½ê¿À·ÐÉÂÍý¡¤
 ¾®ÎÓÂçÊå(1)¡¢ÆüµÍÀµ¼ù(2,3)¡¢¹õ´ä½Óɧ(4)¡¢¾¾ËÜÂî(1,2)¡¢¿åß·±ÑÍÎ(2)¡¢¾®ÃÓÀ¹Íº(1)
 
3¥×¥ë¥­¥ó¥¨ºÙ˦¤Îsomtodendritic sprouting¤òÃæ³ËÉÂÍý¤È¤¹¤ë²È²À­¾®Ç¾ÊÑÀ­¾É¤Î°ì˶¸¡Îã
 1, ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤2, ÅìµþÂç³Ø°å³ØÉôÉհɱ¡¿À·ÐÆâ²Ê¡¤3, ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø
 µ×ÊÝÅÄ¡¡¶Ç¡¢»°Ã«Ï»ҡ¢»³Ç·Æâ¡¡Çî(1)¡¢¸åÆ£¡¡½ç(2)¡¢óîÆ£¡¡Í´»Ò¡¢Â¼»³¡¡ÈËͺ(3)
 
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 1)Åìµþ°å²Ê»õ²ÊÂç³Ø¿À·ÐÆâ²Ê 2)¹ñΩÎÅÍܽ꾾¹¾É±¡¿À·ÐÆâ²Ê
 ÀÐÀî¶ÖÌé1) ÂçÏÂÅÄ·é1) ÀÐÅĸ¼2)¡¡Í»½°ÂÀ1)¡¡¿åß·±ÑÍÎ1)
 
5¿´ÇÙÄä»ß¤Ç¤ÏÀâÌÀ¤·¤¬¤¿¤¤ÀÔ¿ñÉÂÊѤòÍ­¤·¡¤¸ò´¶¿À·ÐÀá¤ËLewy¾®ÂΤòǧ¤á¤¿MSA¤Î70ºÐÃËÀ­Îã
 1.¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼Éð¢É±¡ ¿À·ÐÆâ²Ê¡¤2.¹ñΩÀº¿À¡¦¿À·Ð¥»¥ó¥¿¡¼Éð¢É±¡ Î×¾²¸¡ººÉô¡¤
 À¶¿å¹¨(1)¡¤ÂçÌðÇ«(1)¡¤¾®Àî²íʸ(1)¡¤Àî°æ½¼(1)¡¤Âç½Ðµ®»Î(2)¡¤Í­ÇÏË®Àµ(2)
 
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Autopsy case of Niemann Pick type C
 1)Division of Child Neurology and 2)Pathology, Kanagawa Children's Medical Center, 3)Department of Neuropathology, Tokyo Metropolitan Institute of Gerontrogy
 Sumimasa Yamashita1), Keisuke Kato2), Yuko Saito3), Shigeo Murayama3)
 We presented the neuropathologic findings of Niemann Pick type C. Main features consited of the ballooning of neronal cells which contained many membranous cytoplanmic bodies by EM examination, spheroids and NFTs. NFT was positive in tau and negative in A¦Â. These findings are considered to have the relation to the cholesterol tansport disturbance.
 
7·ì´É±ê¡¢¹¥»Àµå¿»½á¤òȼ¤¦¿ñËì¤ÎÆù²ê¼ðÀ­ÉÂÊѤÎÀ®¿ÍǾÀ¸¸¡Îã
 1)¡¡½çŷƲÂç³Ø¡¡Ç¾¿À·ÐÆâ²Ê¡¡2) ½çŷƲÂç³Ø¡¡Ç¾¿À·Ð³°²Ê¡¡3) ½çŷƲÂç³Ø¡¡ÉÂÍý³ØÂè°ì
 ¹âÍü²íʸ1)¡¢¿¹¡¡½¨À¸1)¡¢À¾²¬·òÌï1)¡¢ËÎÉôµ®É×1)¡¢¹©Æ£·òÂÀϺ2)¡¢¾¾ËܽӼ£3)¡¢¿åÌîÈþË®1)
 
8Sneddon¾É¸õ·²¤Î58ºÐÃËÀ­Ë¶¸¡Îã
 (1)¼«¼£°å²ÊÂç³ØÉÂÍý¿ÇÃÇÉô¡¤(2)¼«¼£°å²ÊÂç³Ø¿À·ÐÆâ²Ê
 ²ÏÅÄ ¹ÀÉÒ(1)¡¤³êÀî Æ»¿Í(2)¡¤Ý¯°æ ¿®»Ê(1)¡¤ÅÄÃæ µü(1)¡¤ºØÆ£ ·ú(1)¡¤ÃæÌî º£¼£(2)
 
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Typical case of angiotrophic lymphoma
 First Department of Pathology, Gunma University, School of Medicine
 Jun Sasaki
 
10Characteristic vascular change after chemotherapy in an autopsy case of angiotrophic lymphoma
 Department of Neurology, Kameda Medical Center; Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology
 Yuko Hiroyoshi, Toshio Fukutake, Shigeo Murayama
 Objective: To report the autopsy case of angiotrophic lymphoma after chemotherapy.¡¤Case report: A 74 year-old male developed general fatigue and appetite loss in July,2000, and subacute progressive dementia in October,2000. MRI of the head showed multiple, small infarction in the subcortical white matter. High serum LDH (619 IU/l) and sIL-2R (3300 U/ml) led us to consider angiotrophic lymphoma. After chemotherapy( CHOP x3,DHAP x1,IVAM x2), CT of the head showed no improvement but his mentation improved .Four months latter ,high serum LDL and sIL-2 R, and low platelet count were noted. He was treated with DHAP x1, IVAM x2 and MACOP-B, which were not effective. He improved temporarily with rituximab, but then deteriorated. He suffered from pneumonia and died on August 15,2001.¡¤Neuropathological findings: Autopsy was done after 2hours and 50minutes.The brain weight was 1.260g. Macroscopic findings included multiple small infarction in bilateral centrum semiovale and mimimal arteriosclerosis. Histologically, small vessels were packed with diffuse large B cell lymphoma(CD 45,CD79A positive). Unique fibrotic changes similar to the blood vessels in the ventricular wall were observed in the arteriolar wall in the subarachnoid space.¡¤Conclusion: The unique changes of arteriolar wall have not been described previously in this condition, and may reflect modification with the chemotherapy.¡¤
 
11¶µ°é¹Ö±é
¦ƬÍդƤ󤫤ó¤Î¼ê½ÑÎ㡼³¤ÇÏÉÂÊѤˤĤ¤¤Æ
 ÅìµþÅÔ¿À·Ð²Ê³ØÁí¹ç¸¦µæ½ê¡¡Î×¾²¿À·ÐÉÂÍý
 ¿·°æ¿®Î´
 
12³¤ÇϹŲ½¤òǧ¤á¤¿Â¦Æ¬ÍդƤ󤫤ó¡¢88ºÐÃËÀ­Îã
 1) ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê¡¤2) ÅìµþÅÔÏ·¿Í°åÎÅ¥»¥ó¥¿¡¼ÉÂÍýÉô¡¤3) ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø
 ¿Î²Ê°ìδ¡¤ÄÇ̾ÌÁ»Ò¡¤»³Ç·ÆâÇî1)¡¤ÂôÊÕ¸µ»Ê2)¡¤óîƣʹ»Ò¡¤Â¼»³ÈËͺ3)
 

76 KNP

*Date¡§20031227
*Manager¡§Tomohiko Mizutani

1½Å¾ÉÊ£¹çÌȱÖÉÔÁ´¤Ëʻȯ¤·¤¿¥µ¥¤¥È¥á¥¬¥í¥¦¥¤¥ë¥¹Ç¾±ê
 ¿ÀÆàÀΩ¤³¤É¤â°åÎÅ¥»¥ó¥¿¡¼¿À·ÐÆâ²Ê
 »³²¼½ãÀµ
 
2ËýÀ­¤Î·Ð²á¤òé¤Ã¤¿¹âÎðȯ¾É¤Î¥Ø¥ë¥Ú¥¹Ç¾±ê¤Î£±Îã
 µµÅÄÁí¹çɱ¡¿À·ÐÆâ²Ê¡¤* ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø
 ¡ûÁ¥±Û¡¡·Ä¡¢ÂçÃÝÎé»Ò¡¢¼Æ»³½¨Çî¡¢À¾Ìî¡¡ÍΡ¤*¼»³ÈËͺ
 
3GFAP°äÅÁ»ÒÊÑ°Û¤òȼ¤¦²È²À­À®¿Íȯ¾ÉAlexanderÉ¡Êhereditary adult-onset Alexander's disease¡Ë¤Î53ºÐÃËÀ­Ë¶¸¡Îã
 ¼«¼£°åÂç¿À·ÐÆâ²Ê¡¤* Ʊ¡¡ÉÂÍý¡¤** ÅìËÌÂç³Ø°å³ØÉô°äÅÁɳØ
 ³êÀîÆ»¿Í
 
4Ǿ·ì´ÉÉÂÊѤÎÉÂÍý
 ÆüÂçÉÂÍý³Ø¶µ¼¼¶µ¼ø
 »°Ëó¾»»Ò
 
5Anaplastic oligodendroglioma¤Î1Îã
 ·²ÇÏÂç³ØÂç³Ø±¡ÉÂÂÖÉÂÍý³Ø¡¤* ·²ÇÏÂç³ØÂç³Ø±¡Ç¾ÀÔ¿ñÉÂÂÖ³°²Ê³Ø
 º´¡¹ÌÚÆס¤²£Èø±ÑÌÀ¡¤*ËÙ¸ý·Ë»Ö¡¤*ÀÆÆ£±ä¿Í¡¤ÃæΤÍΰì
 
6An autopsy case of frontotemporal demdntia with Pick body-like-inclusions¡¤positively stained with Gallyas-Braak method. ¡¤
 Dept. of neuroloogy, Jutendo University School of Medicine
 Masashi Takanashi
 
7ÃÔÊò¤òȼ¤Ã¤¿±¿Æ°¥Ë¥å¡¼¥í¥ó¼À´µ¤Î1Îã
 ÆüËÜÂç³ØÉÂÍý³Ø¶µ¼¼¡¤* ÆüËÜÂç³ØÆâ²Ê³Ø¹ÖºÂ¿À·ÐÆâ²ÊÉôÌç
 ËÜ´Ö¡¡Âö¡¤*ÅļÀµ¿Í¡¤*ÄÅÅĹÀ¾»¡¤*±Ê²¬¸­°ì¡¤*ÅÄ¿¬¿­Íº¡¤*¿åëÃÒɧ¡¤º¬Ëܧƻ
 

75 KNP

*Date¡§20030726
*Manager¡§Toshihiko Kuroiwa

1An autopsy case of spinocerebellar ataxia with progressive dementia
 Departments of Neuropathology and Neurology, Tokyo Medical and Dental University¡¤Department of Pathology and Neurology, Japanese Red Cross Medical Center
 Toshihiko Kuroiwa, Katsuhiko Takeda, Kinya Ishikawa, Hidehiro Mizusawa, Hitoshi Okazawa, Tamiko Takemura
 This is an autopsy case of 47 years old Japanese male suffered from spinocerebellar ataxia for 19 years. His sister suffered from similar disorder.The initial symptoms found at 28-years-old was dementia, being followed by aphasia, cerebellar ataxia, pyramidal/extrapyramidal sings and sensory disturbances. The patient died of sepsis after repeated respiratory and urinary tract infection. Autopsy revealed atrophic brain (930g) with olivo-ponto-cerebellar atrophy, lateral and third ventricular dilatation, cerebral cortical and subcortical gliosis. Pathological diagnosis is autosomal dominant spinocerebellar ataxia. The subtype will be discussed in the KNP75.
 
2Spinocerebellar degeneration with a slight elongation in sca1 gene
 Departments of 1.Pathology, 2 Neuropathology, and 7. Neurology, Tokyo Medical and Dental University.¡¤3.Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience,¡¤Departments of 4.Neurology, 5.Internal Medicine and 6. Pathology, Nakano General Hospital
 Takashi Endo1, Katsuiku Hirokawa1, Toshihiko Kuroiwa2, Toshiki Uchihara3, Takayoshi Kobayashi4, Yoshie Takeda6, Takeshi Kasuga6, Kinya Ishikawa 7
 
3Dementia with Lewy body disease or argyrophilic grain disease? : a report of an autopsy case with senile onset.
 1)Tokyo Metropolitan Matsuzawa Hospital, 2)Tokyo Institute of Psychiatry¡¤
 Kenichi Oshima 1,2)¡¤Kuniaki Tsuchiya 1,2)¡¤Tetsuaki Arai 2,1)¡¤Haruhiko Akiyama 2,1)¡¤Kenji Ikeda 2,1)
 
4An autopsy case of a 48-year-old man with non-Hodgkin lymphoma accompanied by limbic encephalitis, cerebellar degeneration, and olivary hypertrophy. Paraneoplastic encephalitis?
 Department of Neurology, Ushioda General Hospital1, Department of Neurology, Showa University School of Medicine2, Department of Neurology, Jichi Medical School3
 Kenji Ishihara1, Yoshio Suzuki1, Jun-ichi Shiota1, Mitsuru Kawamura2, Imaharu Nakano3
 
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A case of primary central nervous system lymphoma
 1 Gunma Univeresity Graduate School of Medicine, Dept. of Human Pathology¡¤2 Gunma University Graduate School of Medicie, Dept. of Neurosurgery
 1 Atsushi Sasaki, Yoichi Nakazato¡¤2 Shogo Ishiuchi, Nobuhito Saitoh¡¤
 
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A 2-years old male with intractable epilepsy - a typical case with cortical tuber
 1 Dept.Clin.Neuropathol., Tokyo Metropolitan Institute for Neuroscience¡¤2 Dept.Neuroradiol., Tokyo Metropolitan Neurological Hospital¡¤3 Dept.Neurosurg., Tokyo Metropolitan Neurological Hospital
 1 Nobutaka Arai¡¤2 Akira Yagishita¡¤3 Kensuke Kawai, Hiroyuki Shimizu
 
7ÅÀƬ¤Æ¤ó¤«¤ó¤«¤éLennox-Gastaut¾É¸õ·²¤Ë°Ü¹Ô¤·¡¢áÛÚ»½ÅÀÑȯºî¤Ç»àË´¤·¤¿°ìÎã
 ²£ÉÍÏ«ºÒɱ¡¿À·ÐÆâ²Ê¡¤*ÅìµþÅÔÏ·¿ÍÁí¹ç¸¦µæ½êÏ·²½Î×¾²¿À·Ð²Ê³Ø¸¦µæ¥°¥ë¡¼¥×
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8A case of Creutzfeldt-Jakob disease presenting with "emperiolesis (oligodendrocytes within astrocytes)" in the cerebral white matter
 1. Department of Pathology, Oosaka Red Cross Hospital, 2. Departmnet of Pathology, Natrional Cadiovascular Disease Center
 Masayuki Shintaku 1 and Chiako Yutani 2
 
9Adult-onset intranuclear inclusion body disease,accompanying severe degeneration of the cerebral white matter,clinically presenting with normal pressure hydrocephalus
 1.Geriatric Neuroscience(Neuropathology),Tokyo Metropolitan Institute of Gerontology¡¤Departments of 2.Neurology and 3.Pathology,Tokyo Metropolitan Geriatric Hospital
 Yuichi FUMIMURA1,2,Yuko SAITOU1, ¡¤Ichiro KASAHARA3,Kazuko MITANI2, Kazutomi KANEMARU2,¡¤HiroshiYAMANOUCHI2,Shigeo MURAYAMA1
 
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