Advertisement

Acta Neuropathologica

, Volume 49, Issue 3, pp 205–212 | Cite as

Subakute spongiforme Encephalopathie mit multiformer Plaquebildung

≫Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems [spino-cerebellare Atrophie mit Demenz, Plaques und plaqueähnlichen Ablagerungen im Klein- und Großhirn≪ (Gerstmann, Sträussler, Scheinker)]
  • J. W. Boellaard
  • W. Schlote
Original Works

Subacute spongiform encephalopathy with multiform plaque formation

“Peculiar familial-hereditary disease of CNS [spinocerebellar atrophy with dementia, plaques, and plaque-like deposits in cerebellum and cerebrum” (Gerstmann, Sträussler, Scheinker)]

Summary

Report of two unrelated cases of a rare familiar disease of degenerative nature, from a clinical point of view belonging to the spinocerebellar atrophies, combined with dementia. According to the pedigrees, the disease can be followed up to 3–4 generations.

Microscopic study reveals glioneuronal dystrophy with spongiform changes together with kuru plaques and atypical plaque-like formations, hitherto only described in this disease. Nosological aspects of these findings are discussed.

Key words

Spinocerebellar atrophy Dementia Plaques Slow virus Spongiform encephalopathies 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Barret AM (1913) A case of Alzheimer's disease with unusual neurological disturbances. J Nerv Dis 40:361–373Google Scholar
  2. Becker PE (1966) Eigenartige familiärhereditäre Krankheit des Zentralnervensystems. In: Becker PE (Hrsg) Humangenetik, Bd V/1. Thieme, Stuttgart, S 285–288Google Scholar
  3. Boellaard JW (1974) Experimentelle Ergebnisse zur Frage der gliösen Dystrophie des Status spongiosus nach intracerebraler Kobalt-implantation. 18. Tg. Verg. Dtsch. Neuropath. und Neuroanat. Zentralbl Allg Pathol 118:547Google Scholar
  4. Braunmühl A von (1954) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Arch Psychiatr Neurol 191:419–449Google Scholar
  5. Corsellis JAN, Brierley JB (1954) An unusual type of pre-senile dementia (atypical Alzheimer's disease with amyloid vascular change) Brain 77:571–587Google Scholar
  6. Cremieux A, Recordier M, Bordouresques J, Roger J, Toga M, Daniel F, Dubois D (1963) Dégénérescence, spino-cérébelleuse familiale et maladie d'Alzheimer. Étude anatomo-clinique d'un cas. Rev Neurol (Paris) 109:45–54Google Scholar
  7. Eadie MJ (1975) Olivo-ponto-cerebellar atrophy. In: Vinken PJ, Bruyn GW (eds) Handbood of clinical neurology, vol 21/1. North-Holland, Amsterdam, Oxford pp 451–457Google Scholar
  8. Gerstmann J, Sträussler E, Scheinker J (1936) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Z Neurol 154:736–762Google Scholar
  9. Gibbs CJ, Jr, Gajdusek DC (1969) Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science 165:1023–1025Google Scholar
  10. Gomori AJ, Partnow MJ, Horoupian DS, Hirano A (1973) The ataxic form of Creutzfeldt-Jakob disease. Arch Neurol 29:318–323Google Scholar
  11. Hayek J, Ulrich J (1975) Kuru-plaques in Creutzfeldt-Jakob disease. Eur Neurol 13:251–257Google Scholar
  12. Hirano A, Ghatak NR, Johnson AB, Partnow MJ, Gomori AS (1972) Argentophilic plaques in Creutzfeldt-Jakob disease. Arch Neurol 26:530–542Google Scholar
  13. Horoupian DS, Powers JM, Schaumburg HH (1972) Kuru-like neuropathological changes in a North-American. Arch Neurol 27:555–561Google Scholar
  14. Jellinger K, Heiss W-D, Deisenhammer E (1974) The ataxic (cerebellar) form of Creutzfeldt-Jakob disease. J Neurol 207:289–305Google Scholar
  15. Luers Th, Spatz H (1957) Pick'sche Krankheit. In: Scholz W (ed), Handbuch der speziellen pathologischen Anatomie und Histologie, Bd XIII/1. Springer, Berlin Göttingen Heidelberg, S 614–715Google Scholar
  16. Masters CL, Richardson EP Jr (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 101:333–344Google Scholar
  17. Rosenthal NP, Keesey J, Crandall B, Brown J (1976) Familial neurological disease associated with spongiform encephalopathy. Arch Neurol 33:252–259Google Scholar
  18. Seitelberger F (1962) Eingenartige familiär-hereditäre Krankheit des Zentralnervensystems in einer niederösterreichischen Sippe. Wien Klin Wochenschr 74:687–691Google Scholar
  19. Spielmeyer W (1922) Histopathologie des Nervensystems. Springer, BerlinGoogle Scholar
  20. Traub RD, Gajdusek DC, Gibbs CJ, Jr (1975) Transmissible virus dementias. The relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease. In: Kinsbourne M, Smith L (eds) Aging, dementia, and central function. Spectrum Flushing, New YorkGoogle Scholar
  21. Worster-Drought C, Greenfield JC, McMenemy WH (1944) A form of familial dementia with spastic paralysis. Brain 67:38–43Google Scholar

Copyright information

© Springer-Verlag 1980

Authors and Affiliations

  • J. W. Boellaard
    • 1
  • W. Schlote
    • 1
  1. 1.Institut für Hirnforschung und Abteilung für submikroskopische Pathologie und Neuropathologie der Universität TübingenTübingenBundesrepublik Deutschland

Personalised recommendations