Colosimo C, Albanese A, Hughes A J, de Bruin V M, Lees A J
Department of Neurology, Middlesex Hospital, London, England.
Arch Neurol. 1995 Mar;52(3):294-8. doi: 10.1001/archneur.1995.00540270090024.
The clinical recognition of multiple system atrophy (MSA) in patients presenting with parkinsonian signs is difficult. We attempted to verify the predictive value of some pointers that are used in clinical practice.
Sixteen consecutive patients with pathologically confirmed MSA who presented with a parkinsonian syndrome over an 8-year period were studied retrospectively, and their clinical features were analyzed in detail.
Parkinson's Disease Society, Brain Tissue Bank, Institute of Neurology, London, England.
Sixteen patients with pathologically proven MSA who presented with parkinsonian syndrome in the first 3 years since disease onset.
Clinical features that were analyzed included the rapidity of disease progression, the relative symmetry of symptom onset, the presence or absence of tremor at initial presentation, the therapeutic response to levodopa and the associated presence of autonomic dysfunction. Fourteen of the 16 patients also had a computed tomographic scan of the brain performed. The frequency of selected items in MSA was compared with that found in 20 pathologically confirmed cases of Parkinson's disease and 16 pathologically confirmed cases of progressive supranuclear palsy (Steele-Richardson-Olszewski disease).
It was found that a probability scale based on five selected items discriminated MSA with a pure parkinsonian presentation from Parkinson's disease, but not from progressive supranuclear palsy. Patients affected by the latter disorder, however, commonly presented with additional clinical features (supranuclear vertical down-gaze palsy, axial dystonia, and cognitive impairment), which helped to differentiate it from MSA.
对于表现出帕金森氏症体征的患者，临床上很难识别多系统萎缩（MSA）。我们试图验证一些临床实践中使用的指标的预测价值。
回顾性研究了16例在8年期间出现帕金森综合征且经病理证实为MSA的连续患者，并详细分析了他们的临床特征。
英国伦敦神经病学研究所帕金森病协会脑组织库。
16例在疾病发作后的前3年出现帕金森综合征且经病理证实为MSA的患者。
分析的临床特征包括疾病进展的速度、症状发作的相对对称性、初始表现时是否存在震颤、对左旋多巴的治疗反应以及自主神经功能障碍的相关存在情况。16例患者中的14例还进行了脑部计算机断层扫描。将MSA中选定项目的频率与20例经病理证实的帕金森病病例和16例经病理证实的进行性核上性麻痹（斯蒂尔-理查森-奥尔谢夫斯基病）病例中发现的频率进行比较。
发现基于五个选定项目的概率量表能够区分纯帕金森表现的MSA与帕金森病，但不能区分与进行性核上性麻痹。然而，受后一种疾病影响的患者通常会出现其他临床特征（核上性垂直向下凝视麻痹、轴性肌张力障碍和认知障碍），这有助于将其与MSA区分开来。
