Rivest J, Quinn N, Marsden C D
University Department of Clinical Neurology, National Hospital, London, UK.
Neurology. 1990 Oct;40(10):1571-8. doi: 10.1212/wnl.40.10.1571.
Adult-onset dystonia-parkinsonism is a syndrome in search of a pathology. We therefore reviewed the literature on dystonic manifestations in autopsy-proven cases of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and idiopathic Parkinson's disease (PD). Only 6 of 140 autopsy reports of MSA remarked on the presence of dystonia in life, but personal observations suggest prominent antecollis may develop at some stage in up to 1/2 of sufferers. Similarly, very few (15/118) clinicopathologic observations on PSP included convincing dystonic manifestations, in contrast to some clinical reports where blepharospasm and early limb dystonia were prominent. Virtually any form of focal and segmental dystonia may sometimes occur with clinically diagnosed PD, with occasional descriptions of hemidystonia-hemiparkinsonism. However, there is pathologic confirmation of this diagnosis in only 1 case. With many patients thought clinically to have PD proving pathologically to have another cause for their parkinsonism, the true frequency and the range of dystonic manifestations acceptable in PD remain unknown.
成人起病的肌张力障碍-帕金森综合征是一种病因不明的综合征。因此，我们回顾了关于多系统萎缩（MSA）、进行性核上性麻痹（PSP）和特发性帕金森病（PD）尸检确诊病例中肌张力障碍表现的文献。在140份MSA尸检报告中，只有6份提到生前存在肌张力障碍，但个人观察表明，多达1/2的患者在某个阶段可能会出现明显的颈前屈。同样，在PSP的临床病理观察中，只有极少数（15/118）包括令人信服的肌张力障碍表现，这与一些临床报告中眼睑痉挛和早期肢体肌张力障碍突出的情况形成对比。实际上，几乎任何形式的局灶性和节段性肌张力障碍有时都可能与临床诊断的PD同时出现，偶尔也有偏侧肌张力障碍-偏侧帕金森综合征的描述。然而，只有1例病例有该诊断的病理证实。由于许多临床上被认为患有PD的患者经病理证实其帕金森综合征另有病因，PD中肌张力障碍表现的真实发生率和可接受范围仍然未知。
