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We report a 40-year-old-woman, who had been diagnosed as having multiple sclerosis (MS) at age 38 after developing several episodes of transient diplopia, ataxia, and facial weakness on the right side. She was admitted to our hospital due to stupor, nystagmus, diplopia, and fever. Neurological findings included stupor, ataxic speech, nystagmus, diplopia on the right horizontal gaze, hyperreflexia on the dominant right side, bilateral extensor plantar responses, hypoesthesia on the left side of the face and extremities, hypermetria on the extremities, the presence of Romberg\u0027s sign, and ataxic gait. Brain MRI showed abnormal lesions in the peri-third ventricle, the periaqueductal gray matter of the midbrain, the hypothalamus, and the pontine tegmenta on the right side. The cerebrospinal fluid (CSF) contained 96 mg/dl protein and increased IgG. Serum and CSF lactic acid and pyruvate levels were increased. 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  1. A 資料種別
  2. 010 東京女子医科大学雑誌
  3. 010A 論文
  4. 78巻
  5. 臨時増刊号

多発性硬化症様症状を呈した成人型Leigh脳症(神経内科学教室岩田誠教授退任記念特別号)

http://hdl.handle.net/10470/27743
http://hdl.handle.net/10470/27743
d5de65c9-374a-4425-94c5-31978c22ed80
名前 / ファイル ライセンス アクション
KJ00006015118.pdf KJ00006015118.pdf (1.9 MB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2010-08-10
タイトル
タイトル 多発性硬化症様症状を呈した成人型Leigh脳症(神経内科学教室岩田誠教授退任記念特別号)
言語
言語 jpn
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 journal article
別タイトル
その他のタイトル A Case of Adult Leigh's Disease With Multiple Sclerosis-Like Symptoms(Special Issue in Hornor of the Retirement of Professor Makoto Iwata at the Department of Neurology, Tokyo Women's Medical University)
著者名 清水, 優子

× 清水, 優子

WEKO 293820

清水, 優子

Search repository
竹内, 恵

× 竹内, 恵

WEKO 293821

竹内, 恵

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太田, 宏平

× 太田, 宏平

WEKO 293822

太田, 宏平

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永井, 知代子

× 永井, 知代子

WEKO 293823

永井, 知代子

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吉澤, 浩志

× 吉澤, 浩志

WEKO 293824

吉澤, 浩志

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迫村, 泰成

× 迫村, 泰成

WEKO 293825

迫村, 泰成

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内山, 真一郎

× 内山, 真一郎

WEKO 293826

内山, 真一郎

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岩田, 誠

× 岩田, 誠

WEKO 293827

岩田, 誠

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著者別名
姓名 SHIMIZU, Yuko
著者別名
姓名 TAKEUCHI, Megumi
著者別名
姓名 OTA, Kohei
著者別名
姓名 NAGAI, Chiyoko
著者別名
姓名 YOSHIZAWA, Hiroshi
著者別名
姓名 SAKOMURA, Yasunari
著者別名
姓名 UCHIYAMA, Shinichiro
著者別名
姓名 IWATA, Makoto
出版者
出版者 東京女子医科大学学会
受付日付
日付 2010-08-10
日付タイプ Created
ISSN
収録物識別子タイプ ISSN
収録物識別子 0040-9022
NCID
収録物識別子タイプ NCID
収録物識別子 AN00161368
書誌情報 東京女子医科大学雑誌

巻 78, 号 Extra, p. E75-E82, 発行日 2008-02-25
著者版フラグ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
抄録
内容記述タイプ Abstract
内容記述 Leigh's disease (subacute necrotizing encephalomyelopathy) is a progressive neurological disorder caused by mitochondrial dysfunction that occurs primarily in infancy and childhood and only rarely in adulthood. We report a 40-year-old-woman, who had been diagnosed as having multiple sclerosis (MS) at age 38 after developing several episodes of transient diplopia, ataxia, and facial weakness on the right side. She was admitted to our hospital due to stupor, nystagmus, diplopia, and fever. Neurological findings included stupor, ataxic speech, nystagmus, diplopia on the right horizontal gaze, hyperreflexia on the dominant right side, bilateral extensor plantar responses, hypoesthesia on the left side of the face and extremities, hypermetria on the extremities, the presence of Romberg's sign, and ataxic gait. Brain MRI showed abnormal lesions in the peri-third ventricle, the periaqueductal gray matter of the midbrain, the hypothalamus, and the pontine tegmenta on the right side. The cerebrospinal fluid (CSF) contained 96 mg/dl protein and increased IgG. Serum and CSF lactic acid and pyruvate levels were increased. Aerobic testing was positive; muscle biopsy and mitochondrial DNA analysis were negative. ^<123>I-BMIPP myocardial images detected reduced uptake in the anterior-wall and apical portion. The myocardial biopsy revealed degeneration of myocardial fibers and mitochondrial abnormalities, such as increased size and abnormal inclusions. Therefore, adult Leigh's disease was diagnosed. The patient died unexpectedly due to acute respiratory failure at age 42. An autopsy was not performed. In patients with MS-like symptoms and MRI findings in the midbrain, brain stem, and basal ganglia, the possibility of adult Leigh's disease should be considered.
注記
内容記述タイプ Other
内容記述 神経内科学教室岩田誠教授退任記念特別号
著者所属
東京女子医科大学医学部神経内科学
著者所属
東京女子医科大学医学部神経内科学
著者所属
東京理科大学理学部
著者所属
東京女子医科大学医学部神経内科学
著者所属
東京女子医科大学医学部神経内科学
著者所属
東京女子医科大学医学部循環器内科学
著者所属
東京女子医科大学医学部神経内科学
著者所属
東京女子医科大学医学部神経内科学
著者キーワード
主題Scheme Other
主題 adult Leigh's disease
著者キーワード
主題Scheme Other
主題 multiple sclerosis
著者キーワード
主題Scheme Other
主題 midbrain
著者キーワード
主題Scheme Other
主題 myocardiac biopsy
著者キーワード
主題Scheme Other
主題 mitochondria
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