Parkinsonian features in advanced Down's syndrome.

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.