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Related Experiment Videos

Parkinsonian features in advanced Down's syndrome.

P Vieregge1, G Ziemens, A Piosinski

  • 1Klinik für Neurologie, Medizinische Universität zu Lübeck, Federal Republic of Germany.

Journal of Neural Transmission. Supplementum
|January 1, 1991
PubMed
Summary

Extrapyramidal signs, resembling Parkinsonism, were observed in demented patients with Down's syndrome (DS), but not in non-demented individuals. These neurological findings suggest differences in Parkinsonian sign prevalence between DS and Alzheimer's disease (AD).

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Area of Science:

  • Neurology
  • Genetics
  • Clinical Medicine

Background:

  • Down's syndrome (DS) is associated with an increased risk of dementia, often resembling Alzheimer's disease (AD).
  • Neurological signs, including extrapyramidal and frontal release signs, are common in neurodegenerative conditions.

Purpose of the Study:

  • To investigate the prevalence and characteristics of neurological signs, particularly extrapyramidal signs, in patients with Down's syndrome.
  • To compare the occurrence of Parkinsonian features in Down's syndrome with that in advanced Alzheimer's disease.

Main Methods:

  • Clinical assessment of neurological signs (extrapyramidal, frontal release) in 54 patients with Down's syndrome (14 demented, 40 non-demented).
  • Age comparison between demented DS patients with and without extrapyramidal signs.

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  • Family history inquiry regarding dementia and Parkinsonism.

    • Main Results:

    • Five of the 14 demented DS patients exhibited extrapyramidal signs, primarily rigid-hypokinetic, similar to Parkinsonism in advanced AD.
    • No Parkinsonian signs were noted in the non-demented DS group.
    • Demented DS patients with extrapyramidal signs were significantly older than those without.
    • Frontal release signs were present in both demented and non-demented DS patients.
    • No increased familial incidence of dementia or Parkinsonism was found.

    Conclusions:

    • Extrapyramidal signs, akin to Parkinsonism, are present in a subset of demented individuals with Down's syndrome.
    • Parkinsonian signs appear less frequent in DS compared to advanced Alzheimer's disease.
    • A hypothesis suggests a potential gene dosage effect of Cu/Zn-superoxide dismutase in protecting the substantia nigra in DS patients.