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6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)
http://hdl.handle.net/10191/35756
http://hdl.handle.net/10191/3575695f0bbca-4534-465a-830d-a9bfad7982c6
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
100(12)_738-745.pdf (1.3 MB)
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| Item type | 紀要論文 / Departmental Bulletin Paper(1) | |||||
|---|---|---|---|---|---|---|
| 公開日 | 2015-12-16 | |||||
| タイトル | ||||||
| タイトル | 6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会) | |||||
| タイトル | ||||||
| タイトル | 6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会) | |||||
| 言語 | en | |||||
| 言語 | ||||||
| 言語 | jpn | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | myoclonus epilepsy Anraku type | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | brown pigment | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | degeneration of cerebellar cortex | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | 褐色顆粒 | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | 小脳皮質変性 | |||||
| 資源タイプ | ||||||
| 資源 | http://purl.org/coar/resource_type/c_6501 | |||||
| タイプ | departmental bulletin paper | |||||
| その他のタイトル | ||||||
| その他のタイトル | Myoclonus epilepsy, Anraku type(Hereditary Spinocerebellar Degeneration : recent advances) | |||||
| 著者 |
武田, 茂樹
× 武田, 茂樹× 高橋, 均× 大浜, 栄作× 生田, 房弘× 内藤, 明彦× 有田, 忠司 |
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| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145754 | |||||
| 姓名 | Takeda, Shigeki | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145755 | |||||
| 姓名 | Takahashi, Hitoshi | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145756 | |||||
| 姓名 | Ohama, Eisaku | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145757 | |||||
| 姓名 | Ikuta, Fusahiro | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145758 | |||||
| 姓名 | Naito, Haruhiko | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145759 | |||||
| 姓名 | Arita, Tadashi | |||||
| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Myoclonus epilepsy is neuropathologically classified into 3 major groups; Lafora body type, lipid storage type, and degenerative type. Anraku et al.（1966）made a brief description of 3 cases with brown pigments in some CNS regions as a special type of myoclonus epilepsy？. However, no such cases have been reported thereafter. We reported another case with such histological findings and described new findings. A 20-year-old woman without hereditary factors, suffered from progressive ataxic gait. Myoclonus, trunkal ataxia and dementia appeared and progressed gradually. Polyspike and wave complex were recorded in EEG. She died at the age of 28. Postmortem examination revealed severe loss of Purkinje cells and granule cells with gliosis. Globus pallidus, subthalamus, dentate and red nuclei, and pontine tegmentum showed neuronal loss and gliosis. Pontine nuclei were well preserved. Cerebral cortex also revealed mild but diffuse neuronal loss. In addition to these lesions, many autofluorescent pigments, positively stained with Fontana-Masson silver method, were present in the Ist to IIIrd layer of the cerebral cortex, Purkinje cell layer, globus pallidus and substantia nigra. The number of the pigments was not correlated with neuronal loss or gliosis. In the white matter, peripheral nerves and general visceral organs, the pigment was not observed. Ultrastructurally, the pigments were localized in astrocytes, and similar to the lipofuscin or stored substance observed in the ceroidlipofuscinosis. Our case and 3 cases previously reported strongly suggest that there is a particular subgroup in the myoclonus epilepsy, which is characterized by presence of brown pigment and degeneration of cerebellar cortex. | |||||
| 書誌情報 |
新潟医学会雑誌 en : 新潟医学会雑誌 巻 100, 号 12, p. 738-745, 発行日 1986-12 |
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| 出版者 | ||||||
| 出版者 | 新潟医学会 | |||||
| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 00290440 | |||||
| 書誌レコードID | ||||||
| 収録物識別子タイプ | NCID | |||||
| 収録物識別子 | AN00182415 | |||||
| 著者版フラグ | ||||||
| 値 | publisher | |||||
