Kovacs Norbert, Pal Endre, Balas Istvan, Janszky Jozsef, Nagy Ferenc, Merkli Hajnalka
Department of Neurology, University of Pecs, Pecs, Hungary.
Mov Disord. 2006 Dec;21(12):2227-30. doi: 10.1002/mds.21128.
A 53-year-old woman underwent several ischemic stroke-like episodes and later developed incomplete, bilateral ophthalmoplegia, left vision deterioration, and bilateral tremor. The clinical course, laboratory data, and muscle histology led to a diagnosis of mitochondrial encephalomyopathy. No other etiology could be identified in the background of her disabling bilateral postural-kinetic tremor. As this tremor did not respond to pharmacological therapy, left thalamotomy and subsequently right thalamic deep brain stimulator (DBS) implantation were performed, which resulted in an excellent clinical outcome. The Fahn-Tolosa-Marin Tremor Rating Scale improved from 110 to 11 points. This case suggests that the rare tremor caused by mitochondrial encephalopathy may be treated long-term with either thalamotomy or thalamic DBS implantation.
一名53岁女性经历了几次缺血性中风样发作，后来出现不完全性双侧眼肌麻痹、左眼视力减退和双侧震颤。临床病程、实验室检查数据及肌肉组织学检查结果确诊为线粒体脑肌病。在其导致残疾的双侧姿势性运动性震颤背景下，未发现其他病因。由于这种震颤对药物治疗无反应，遂进行了左侧丘脑切开术，随后植入右侧丘脑深部脑刺激器（DBS），临床效果极佳。法恩-托洛萨-马林震颤评分量表从110分改善至11分。该病例表明，线粒体脑病引起的罕见震颤可通过丘脑切开术或丘脑DBS植入进行长期治疗。
