Advertisement

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

Summary

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 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Boellaard JW, Schlote W (1980) Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. Acta Neuropathol (Berl) 49:205–212Google Scholar
  2. Boellard JW, Schlote W (1981) Glial plaques: Amyloid deposits characteristic of slow transmissible encephalopathies. Virchows Arch B [Cell Pathol] 37:337–341Google Scholar
  3. van Braunmühl A (1954) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Arch Psychiatr Z Neurol 191:419–449Google Scholar
  4. Brownell B, Oppenheimer DR (1965) An ataxic-form of subacute presenile polio-encephalopathy (Creutzfeldt-Jakob disease). J Neurol Neurosurg Psychiatry 28:350–361Google Scholar
  5. Gerstmann J, Sträussler E, Scheinker I (1936) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Z Gesamte Neurol Psychiatr 154:736–762Google Scholar
  6. Horoupian DS, Powers JM, Schaumburg HH (1972) Kuru-like neuropathological changes in a North American. Arch Neurol 27:555–561Google Scholar
  7. Klatzko I, Gajdusek DC, Zigas V (1959) Pathology of Kuru. Lab Invest 8:799–847Google Scholar
  8. Krücke W, Beck E, Gräfin Vitzthum H (1973) Creutzfeld-Jakob disease. Z Neurol 206:1–24Google Scholar
  9. Masters CL, Richardson EP, Jr (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). Brain 101:333–344Google Scholar
  10. Masters CL, Gajdusek DC, Gibbs CJ, Jr (1980) The Gerstmann-Sträussler syndrome and the various forms of amyloid plaques which occur in the transmissible spongiform encephalopathies. J Neuropathol Exp Neurol 39:374Google Scholar
  11. Mitrová E (1979) Focal accumulation of Creutzfeld-Jakob disease in Slovakia. In: Boese A (ed) Search for the cause of multiple sclerosis and other chronic disease of the central nervous system. Verlag Chemie, Weinheim Deerfiled Beach Basel, pp 356–366Google Scholar
  12. Neumann MA, Gajdusek DC, Zigas V (1964) Neuropathologic findings in exotic neurologic disorders among natives of the highlands of New Guinea. J Neuropathol Exp Neurol 23:486–507Google Scholar
  13. Seitelberger F (1962) Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems in einer niederösterreichischen Sippe. Wien Klin Wochenschr 41/42:687–691Google Scholar
  14. Seitelberger F (1981) Sträussler's disease. Acta Neuropathol (Berl) [Suppl] 7:341–343Google Scholar
  15. Schlote W, Boellaard JW, Schumm F, Stöhr M (1980) Gerstmann-Sträussler-Scheinker's disease. Electron microscopic observations on a brain biopsy. Acta Neuropathol (Berl) 52:203–211Google Scholar
  16. Terry RD, Wisniewski HM (1970) The ultrastructure of the neurofibrillary tangles and the senile plaque. In: Wolstenholme GEW, O'Conner M (eds) Alzheimer's disease and related conditions. Churchill, London, p 145Google Scholar

Copyright information

© Springer-Verlag 1982

Authors and Affiliations

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

Personalised recommendations