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題 名 | Osmotic Demyelination Syndrome with Two-Phase Movement Disorders: Case Report=滲透性去髓鞘症候群併兩階段之不自主運動:病例報告 |
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作 者 | 莊曜聰; 張振聲; 許世斌; 林祖功; 呂鎮中; | 書刊名 | 長庚醫學 |
卷 期 | 21:4 1998.12[民87.12] |
頁 次 | 頁526-530 |
分類號 | 415.9 |
關鍵詞 | 滲透性去髓鞘症候群; 橋腦髓鞘變性; 不自主運動; 磁共振造影; 低血鈉; Osmotic demyelination syndrome; Pontine myelinolysis; Movement disorders; Magnetic resonance imaging; MRI; Hyponatremia; |
語 文 | 英文(English) |
中文摘要 | 滲透性去髓鞘症候群(osmotic demyelination syndrome)的特徵是腦部區域性 去髓鞘,特別易發生於橋腦。此去髓鞘病變常見於伴隨電解質不平衡之酗酒或營養不良的病 人。不自主運動並不常見於滲透性去髓鞘症候群的病人。在此報告一名非酗酒之年輕女性在 矯正低血鈉症後發生滲透性去髓鞘症候群。此病患之主要神經學症狀為兩階段之不自主運動 。在第一階段之不自主運動中,病患呈現急性肢體張力異常症(dystonia);在延遲之第二 階段,不自主運動轉變成全身性的僵硬(rigidity)和震顫(tremor)。在出現第一階段之 不自主運動時,磁共振造影發現在橋腦中央、兩側視丘及基底核有去髓鞘病變。延遲之第二 階段發生時,先前在磁共振造影中發現之橋腦中央及橋腦外去髓鞘病變則逐漸消退。此兩 階段之不自主運動臨床症狀的演變與其神經放射學上去髓鞘病變穩定消退的表現無法形成病 程之相關性。病患之不自主運動對乙型交感神經阻抗劑(propranolol)和乙醯膽鹼阻抗劑 (trihexyphenidyl)的治療有良好的反應。 |
英文摘要 | Osmotic demyelination syndrome(ODS) is characterized by regions of demyelination throught the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. Movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. Magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and base ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl. |
本系統中英文摘要資訊取自各篇刊載內容。