Giladi N, Burke R E, Kostic V, Przedborski S, Gordon M, Hunt A, Fahn S
Neurological Institute, College of Physicians and Surgeons of Columbia University, New York, NY.
Neurology. 1990 Nov;40(11):1731-4. doi: 10.1212/wnl.40.11.1731.
We evaluated 11 patients with hemiparkinson-hemiatrophy syndrome, 6 with body and contralateral cerebral hemispheric hemiatrophy, 4 with only body hemiatrophy, and 1 with just brain hemiatrophy. The mean age of symptom onset was 38.1 years (range, 18 to 54) with 5.2 +/- 3.1 (mean +/- SD) years of illness until the last follow-up visit. The presenting symptom was unilateral tremor in 6 patients, hand dystonia in 2, bradykinesia in 2, and abnormal gait in 1 patient. Three patients had a good response to levodopa, 4 had moderate response, and 2 patients had a poor response. During a mean follow-up period of 1.7 years (range, 4 months to 5 years), the Hoehn and Yahr score changed in only 3 patients: 2 gained 1.5 points and 1 gained 3 points over 2.5 years. We discuss the association between hemiparkinsonism-body hemiatrophy and contralateral hemispheric hemiatrophy, and raise the possibility of early childhood brain insult with delayed-onset parkinsonism.
我们评估了11例偏侧帕金森-偏侧萎缩综合征患者，其中6例伴有身体及对侧大脑半球偏侧萎缩，4例仅有身体偏侧萎缩，1例仅有脑部偏侧萎缩。症状出现的平均年龄为38.1岁（范围18至54岁），至最后一次随访时患病时间为5.2±3.1（平均±标准差）年。首发症状为6例患者出现单侧震颤，2例出现手部肌张力障碍，2例出现运动迟缓，1例出现异常步态。3例患者对左旋多巴反应良好，4例反应中等，2例反应较差。在平均1.7年（范围4个月至5年）的随访期内，仅3例患者的Hoehn和Yahr评分发生变化：2例在2.5年内评分增加1.5分，1例增加3分。我们讨论了偏侧帕金森-身体偏侧萎缩与对侧半球偏侧萎缩之间的关联，并提出儿童早期脑损伤伴迟发性帕金森病的可能性。
