Summary
Light and electron microscopic observations are reported on a brain biopsy of a man of 59 with a rare familial disease of the CNS and a 5-year clinical course. Electron micrographs of the frontal biopsy reveal plaque-like deposits composed of amyloid cores, often multicentric in the cortex and subcortical white matter. They are localized between enlarged astrocytic processes. In the neuropil they are sometimes associated with abnormal neuritic processes, in the white matter with processes of fibrous astroglia and basement membranes. There are no signs of primary neuritic or synaptic involvement in the plaque formation which is more obviously associated with altered astrocytic processes. Moreover, degenerative alterations in the cortical vessels and slight astroglial spongiform changes as well as oligodendroglial proliferation can be found. Plaques are considered to be mainly of the kuru type; the relationship with transmissible spongiform encephalopathies is discussed.
Similar content being viewed by others
References
Adams H, Beck E, Shenkin AM (1974) Creutzfeldt-Jakob disease. Further similarities with kuru. J Neurol Neurosurg Psychiatry 37:195–200
Boellaard JW, Schlote W (1980) Subakute spongiforme Encephalopathie mit multiformer Plaquebildung Acta Neuropathol (Berl) 49:205–212
Braunmühl A von (1954) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Arch Psychiatr Neurol 191:419–449
Bruce ME, Fraser H (1975) Amyloid plaques in the brains of mice infected with scrapie. Morphological variation and staining properties. Neuropathol Appl Neurobiol 1:189–202
Chou SM, Martin JD (1971) Kuru plaques in a case of Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 17:150–155
Field EJ, Mathews JD, Raine CS (1969) Electron-microscopic observations on the cerebellar cortex in kuru J Neurol Sci 8:209–224
Gerstmann J, Sträussler E, Scheinker J (1936) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems Z Neurol 154:736–762
Glenner GG Ein D, Terry WD (1972) The immunoglobulin origin of amyloid. Am J Med 52:141–147
Klatzo J, Gajdusek DC, Zigas V (1959) Pathology of kuru. Lab Invest 8:799–847
Masters CL, Gajdusek DC, Gibbs CT Jr, Bernoulli C, Asher DM (1979) Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible modes of transmission of virus-induced familial diseases. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system. Academic Press, New York London, vol I, pp 143–194
Pearse DC (1964) Histological techniques for electron microscopy. 2nd edn. Academic Press, New York London
Rosenthal NP, Keesey, J, Crandall B, Brown J (1976) Familial neurological disease associated with spongiform encephalopathy. Arch Neurol 33:252–259
Seitelberger F (1962) Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems in einer niederösterreichischen Sippe. Wien Klin Wochenschr 74:687–691
Terry RD, Gonatas NK, Weiss M (1964) Ultrastructural studies in Alzheimer's presenile dementia. Am J Pathol 44:269–297
Terry, RD, Wisniewsky H (1970) The ultrastructure of the neurofibrillary tangle and the senile plaque. In: CIBA Foundation Symposium on Alzheimer's disease and related conditions. Churchill Livingston, London, pp 145–165
Wisniewsky H, Johnson AB, Raine CS, Kay WJ, Terry RD (1970) Senile plaques and cerebral amyloidosis in aged dogs. A histochemical and ultrastructural study. Lab Invest 23:287–296
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Schlote, W., Boellaard, J.W., Schumm, F. et al. Gerstmann-Sträussler-Scheinker's disease. Acta Neuropathol 52, 203–211 (1980). https://doi.org/10.1007/BF00705808
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00705808