Wüllner U, Schmitz-Hübsch T, Abele M, Antony G, Bauer P, Eggert K
Department of Neurology, UKB, Bonn, Germany.
J Neural Transm (Vienna). 2007 Sep;114(9):1161-5. doi: 10.1007/s00702-007-0746-0. Epub 2007 May 18.
We identified 221 patients with probable multiple system atrophy (MSA) among 4770 patients enrolled in the multicentre registry of the German Competence Network on Parkinson's disease (PD) according to the established consensus criteria to characterize their clinical presentation. Analyses of more than 100 recorded clinical items revealed several specifics: I) 50% of patients with probable MSA had asymmetry of symptoms at disease onset and tremor at rest was present in 25%; II) a positive response to levodopa was recorded in 51% of patients identified initially with severe autonomic failure and cerebellar ataxia; III) a positive family history was recorded in 11% (n = 23), two of these patients were identified with spinocerebellar ataxia type 3 (SCA3). Thus asymmetry of symptoms, tremor at rest and a positive response to levodopa are not as specific for idiopathic PD as believed previously. Patients with SCA3 may present with the clinical features of MSA.
在德国帕金森病(PD)能力网络多中心登记处登记的4770名患者中,根据既定的共识标准,我们识别出221例可能患有多系统萎缩(MSA)的患者,以描述其临床表现。对100多项记录的临床项目进行分析后发现了几个特点:I)50%可能患有MSA的患者在疾病发作时症状不对称,25%的患者存在静止性震颤;II)最初被诊断为严重自主神经功能衰竭和小脑共济失调的患者中,51%对左旋多巴有阳性反应;III)11%(n = 23)的患者有阳性家族史,其中两名患者被诊断为3型脊髓小脑共济失调(SCA3)。因此,症状不对称、静止性震颤和对左旋多巴的阳性反应并不像之前认为的那样是特发性PD的特异性表现。SCA3患者可能表现出MSA的临床特征。