Lang A E
Morton and Gloria Shulman Movement Disorders Centre, Toronto Hospital, Ontario, Canada.
Mov Disord. 1995 Jul;10(4):489-95. doi: 10.1002/mds.870100414.
A 45-year-old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right-sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion-induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism-hemiatrophy.
一名45岁女性，有围生期颅脑外伤史，可能导致轻度无症状性右侧脑萎缩，12岁时仅在长时间用力后出现右腿无力、肌张力减退，交替出现肌张力障碍。27岁时，她出现右侧帕金森综合征。运动性轻瘫、肌张力障碍和帕金森综合征对左旋多巴反应完全；然而，在治疗的前4年中，她出现了左旋多巴作用持续时间的逐渐缩短。包括计算机断层扫描、磁共振成像、[18F]氟多巴和[18F]氟脱氧葡萄糖正电子发射断层扫描在内的检查提示左侧黑质存在静态病变。这种不寻常的运动诱发无力、肌张力减退与张力亢进和肌张力障碍交替出现的情况此前未见报道。讨论了多巴胺缺乏在肌张力障碍中的作用以及左旋多巴在帕金森病症状波动发展中的作用。包括该患者在内的文献综述强调了偏侧帕金森综合征-偏侧萎缩综合征的异质性。
