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3) ジョセフ病型を示した脊髄小脳変性症の2剖検例(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/10191/35753
http://hdl.handle.net/10191/3575353eec39d-907e-4724-a9de-682e001b81b3
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
100(12)_721-725.pdf (450.9 kB)
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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 | 3) ジョセフ病型を示した脊髄小脳変性症の2剖検例(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 | 3) ジョセフ病型を示した脊髄小脳変性症の2剖検例(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 | Joseph disease | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Autosomal dominant ataxia | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | Multisystemic degeneration | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | 優性遺伝性失調症 | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | 多系索性症 | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | departmental bulletin paper | |||||
| その他のタイトル | ||||||
| その他のタイトル | The autopsy cases of spino-cerebellar degeneration of Joseph type(Hereditary Spinocerebellar Degeneration : recent advances) | |||||
| 著者 |
若林, 允甫
× 若林, 允甫× 小柳, 新策 |
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| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145734 | |||||
| 姓名 | Wakabayashi, Masatoshi | |||||
| 著者別名 | ||||||
| 識別子Scheme | WEKO | |||||
| 識別子 | 145735 | |||||
| 姓名 | Oyanagi, Shinsaku | |||||
| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Here, we present two autopsy cases, first of which was clinically similar to Machado-Joseph disease, and the second one, her neuropathological findings were almost the same to the first case. In the first case, her disease began as Marie's ataxia at age 17. Then, extrapyramidal signs, external ophthalmoplegia, symptome of secondary motor neuron impairment and autonomic nerve disturbance developed gradually. She died of pneumonia at age 35. In the second case, her unsteady gait began early in her life and became getting worse. Then, superficial and deep sensory impairment, secondary motor nerve impairment, and external ophthalmoplegia developed gragually. She died at age 69. Neuropathological findings of these two cases were almost the same, which were summarized as below: 1）Subthalamo-pallidal and Pallido-nigral system, 2）Dentato-rubral system, 3）Ponto-cerebellar system, 4）Cranial nerve nuclei, 5）Spinal cord（spino-cerebellar tract, posterior funiculus, lateral pyramidal tract, posterior funiculus, lateral pyramidal tract, anterior horn, Clarch's column, and only in the first case, intermedio-lateral column）. With regard to the distribution of the degenerative lesion, the present cases have a great similarity to Friedreich's ataxia, but the lesions outside spinal cord are far severe. So, we can not classify the present cases into any of known disorders, and expect these types of spino-cerebellar degeneration to be estabrished as a unique system degenerative disease of the central nervous system. | |||||
| 書誌情報 |
新潟医学会雑誌 en : 新潟医学会雑誌 巻 100, 号 12, p. 721-725, 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 | |||||
