Vieregge P, Ziemens G, Piosinski A, Freudenberg M, Kömpf D
Klinik für Neurologie, Medizinische Universität zu Lübeck, Federal Republic of Germany.
J Neural Transm Suppl. 1991;33:119-24. doi: 10.1007/978-3-7091-9135-4_19.
The results of a clinical study on extrapyramidal, frontal release, and other neurological signs in 54 demented and non-demented patients with Down's syndrome (DS) are presented. Fourteen patients were demented, five of them showed extrapyramidal signs, mainly of the rigid-hypokinetic spectrum and rather similar to Parkinsonian features in advanced Alzheimer's disease (AD). None of the non-demented patients had Parkinsonian signs. The mean ages of the demented DS patients with extrapyramidal signs was significantly higher than that of the patients without the respective signs. Frontal release signs were present in demented and nondemented patients. From a questionnaire neither a raised proportion of early- or senile-onset dementia nor of Parkinsonism among first- and second-degree relatives of DS patients could be traced. Parkinsonian signs seem to be present at a lower frequency in DS than in advanced AD. A speculative hypothesis about a gene dosage effect of Cu/Zn-superoxide dismutase in preventing toxic radical formation in the substantia nigra of DS patients is presented.
本文呈现了一项针对54名患有唐氏综合征（DS）的痴呆和非痴呆患者的锥体外系、额叶释放及其他神经学体征的临床研究结果。14名患者患有痴呆，其中5名表现出锥体外系体征，主要为强直 - 运动减少型，与晚期阿尔茨海默病（AD）的帕金森特征颇为相似。非痴呆患者均无帕金森体征。有锥体外系体征的痴呆DS患者的平均年龄显著高于无相应体征的患者。额叶释放体征在痴呆和非痴呆患者中均有出现。通过问卷调查，未发现DS患者的一级和二级亲属中早发型或老年型痴呆以及帕金森病的比例有所升高。DS患者中帕金森体征的出现频率似乎低于晚期AD患者。本文还提出了一个关于铜/锌超氧化物歧化酶基因剂量效应在预防DS患者黑质中有毒自由基形成的推测性假说。
