Acta Neuropathologica

, Volume 56, Issue 2, pp 87–92 | Cite as

Gerstmann-Sträussler's disease, atypical multiple sclerosis and carcinomas in a family of sheepbreeders

  • J. Peiffer
Original Works


A new family with the Gerstmann-Sträussler type of subacute spongiform encephalopathy is described. Atactic symptoms, dysarthrias, and personality changes characterized the clinical course. The clinical pattern of a father and his two sons was very similar. They had been in contact with sheep by occupation. A rapidly progressing demyelinating disease (transitional diffuse-multiple sclerosis) occurred in the same family, as well as numerous cases of carcinoma. Morphologically, the Sträussler type can be differentiated from other subacute spongy encephalopathies by the occurrence of Kuru-Plaques and numerous multicentric floccular plaques both in the cerebral and cerebellar cortex, basal ganglia, and white matter.

Key words

Sträussler's disease Jakob Creutzfeldt's disease Subacute spongious encephalopathy Multicentric floccular plaques Slow virus disease Multiple sclerosis 


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Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • J. Peiffer
    • 1
  1. 1.Institute for Brain ResearchUniversity of TübingenTübingen 1Federal Republic of Germany

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