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. 1985 Mar;48(3):234–238. doi: 10.1136/jnnp.48.3.234

Familial clustering of the ataxic form of Creutzfeldt-Jakob disease with Hirano bodies.

L Cartier, S Gálvez, D C Gajdusek
PMCID: PMC1028256  PMID: 2984334

Abstract

A family cluster of the ataxic form of Creutzfeldt-Jakob disease with one probable and two autopsy proven cases that occurred in a single generation between 1974 and 1982 is reported. The clinical characteristics of the cases are closely similar to those of kuru patients, with a fair correlation between the prominent truncal ataxia and the intense devastation of the cerebellar cortex most marked in the vermis. Pathologically, the marked hippocampal involvement rarely seen in typical transmissible Creutzfeldt-Jakob disease and the finding of Hirano bodies in the Ammon's horn without specific Alzheimer's senile changes are noteworthy features.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BROWNELL B., OPPENHEIMER D. R. AN ATAXIC FORM OF SUBACUTE PRESENILE POLIOENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE). J Neurol Neurosurg Psychiatry. 1965 Aug;28:350–361. doi: 10.1136/jnnp.28.4.350. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Boudin G., Pépin B., Milhaud M. Maladie de Creutzfeldt-Jakob à symptomatologie cérébelleuse dominante. (Etude anatomo-clinique d'un cas) Rev Neurol (Paris) 1965 Jul;113(1):73–75. [PubMed] [Google Scholar]
  3. Cobb W. A., Hornabrook R. W., Sanders S. The EEG of kuru. Electroencephalogr Clin Neurophysiol. 1973 Apr;34(4):419–427. doi: 10.1016/0013-4694(73)90093-x. [DOI] [PubMed] [Google Scholar]
  4. David-Ferreira J. F., David-Ferreira K. L., Gibbs C. J., Jr, Morris J. A. Scrapie in mice: ultrastructural observations in the cerebral cortex. Proc Soc Exp Biol Med. 1968 Jan;127(1):313–320. doi: 10.3181/00379727-127-32680. [DOI] [PubMed] [Google Scholar]
  5. Field E. J., Mathews J. D., Raine C. S. Electron microscopic observations on the cerebellar cortex in kuru. J Neurol Sci. 1969 Mar-Apr;8(2):209–224. doi: 10.1016/0022-510x(69)90111-7. [DOI] [PubMed] [Google Scholar]
  6. Flament-Durand J., Couck A. M. Spongiform alterations in brain biopsies of presenile dementia. Acta Neuropathol. 1979 Apr 12;46(1-2):159–162. doi: 10.1007/BF00684819. [DOI] [PubMed] [Google Scholar]
  7. Gibbs C. J., Jr, Gajdusek D. C. Virus-induced subacute slow infections of the brain associated with a cerebellar-type ataxia. Adv Neurol. 1978;21:359–372. [PubMed] [Google Scholar]
  8. Gálvez S., Cartier L., Monari M., Araya G. Familial Creutzfeldt-Jakob disease in Chile. J Neurol Sci. 1983 Apr;59(1):139–147. doi: 10.1016/0022-510x(83)90087-4. [DOI] [PubMed] [Google Scholar]
  9. Hirano A., Malamud N., Elizan T. S., Kurland L. T. Amyotrophic lateral sclerosis and Parkinsonism-dementia complex on Guam. Further pathologic studies. Arch Neurol. 1966 Jul;15(1):35–51. doi: 10.1001/archneur.1966.00470130039004. [DOI] [PubMed] [Google Scholar]
  10. Kott E., Bornstein B., Sandbank U. Ataxic form of Creutzfeldt-Jakob disease. Its relation to subacute spongiform encephalopathy. J Neurol Sci. 1967 Jul-Aug;5(1):107–113. doi: 10.1016/0022-510x(67)90011-1. [DOI] [PubMed] [Google Scholar]
  11. Masters C. L., Gajdusek D. C., Gibbs C. J., Jr Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. Brain. 1981 Sep;104(3):559–588. doi: 10.1093/brain/104.3.559. [DOI] [PubMed] [Google Scholar]
  12. Michel D., Tommasi M., Masson R., Rochet M., Berthezène F., Schott B. Encéphalopathie spongieuse tardive de type Creutzfeldt-Jakob avec importantes lésions cérébelleuses (étude anatomo-clinique d'un cas) J Med Lyon. 1968 Apr 5;49(140):575–passim. [PubMed] [Google Scholar]
  13. Ogata J., Budzilovich G. N., Cravioto H. A study of rod-like structures (Hirano bodies) in 240 normal and pathological brains. Acta Neuropathol. 1972;21(1):61–67. doi: 10.1007/BF00688000. [DOI] [PubMed] [Google Scholar]
  14. Ramsey H. J. Altered synaptic terminals in cortex near tumor. Am J Pathol. 1967 Dec;51(6):1093–1109. [PMC free article] [PubMed] [Google Scholar]
  15. Schochet S. S., Jr, Hardman J. M., Ladewig P. P., Earle K. M. Intraneuronal conglomerates in sporadic motor neuron disease. A light and electron microscopic study. Arch Neurol. 1969 May;20(5):548–553. doi: 10.1001/archneur.1969.00480110112012. [DOI] [PubMed] [Google Scholar]
  16. Zarranz J. J., Rivera Pomar J. M., Salisachs P. Kuru plaques in the brain of two cases with Creutzfeldt-Jakob disease. A common origin for the two diseases? J Neurol Sci. 1979 Oct;43(2):291–300. doi: 10.1016/0022-510x(79)90122-9. [DOI] [PubMed] [Google Scholar]

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