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The ataxic (cerebellar) form of creutzfeldt-jakob disease

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Summary

Creutzfeldt-Jakob disease (CJD) may present itself with cerebellar signs initially and in the course of the disease. In addition to the classical triad of morphological changes, i.e. status spongiosus, neuronal loss and astrocytic proliferation, in the cerebral cortex and basal ganglia, there may be cerebellar involvement with selective loss of the granule cells with associated astrocytic gliosis or sponginess and relative sparing of the Purkinje cells. Occasional systematized degeneration of the paleocerebellum, “kuru-like” plaques in the cerebellum and chromatolytic neurons in the striatum are similarities between CJD and kuru. Among 17 proven cases of CJD cerebellar onset with or without mental changes was present in 47%, and cerebellar ataxia in the course of the disease was prominent in 75%. Cerebellar lesions were noted in 81%, and were more severe and advanced than cerebral involvement in 5 cases with cerebellar onset. Kuru plaques were not seen. CJD presenting with ataxia and cerebellar symptoms constitutes about 17% of all cases of CJD reported to date, and should be considered in the differential diagnosis of cerebellar disease in middle-aged patients. The similarities and dissimilarities of this subtype of CJD to kuru and other transmissible slow virus spongiform encephalopathies in man and animals are discussed.

Zusammenfassung

Die Jakob-Creutzfeldt-Krankheit (JCK) kann initial oder im weiteren Krankheitsverlauf cerebellare Symptome zeigen. Neben der klassischen Triade morphologischer Läsionen, d. h. Status spongiosus, Neuronenausfall und Astrogliaproliferation, in Großhirnrinde und Stammganglien kann ein Kleinhirnbefall mit selektivem Ausfall der Körnerzellschicht und Astrogliose oder Status spongiosus bei relativer Verschonung der Purkinjezellen bestehen. Gelegentliche pseudosystematische Degeneration des Paläocerebellum, „Kuru-ähnliche“ Plaques im Kleinhirn sowie Chromatolyse von Striatumneuronen bilden Analogien zwischen JCK und Kuru. Unter 17 verifizierten eigenen Beobachtungen von JCK bestanden initiale Kleinhirnsymptome mit oder ohne psychische Veränderungen in 47%, während im weiteren Verlauf eine Kleinhirnataxie bei 75% hervortrat. Kleinhirnläsionen waren in 81% nachweisbar; sie waren stärker ausgeprägt und fortgeschrittener als die Großhirnveränderungen in 5 Fällen mit cerebellarem Krankheitsbeginn. Kuru-Plaques waren nicht nachweisbar. JCK mit früher Ataxie und Kleinhirnsymptomatik findet sich in 17% aller bisher berichteten Beobachtungen von JCK und sollte in der Differentialdiagnostik von Kleinhirnsyndromen im mittleren Lebensalter berücksichtigt werden. Die Ähnlichkeiten und Diskrepanzen dieses Subtyps von JCK gegenüber Kuru und anderen übertragbaren Slow-Virus bedingten spongiösen Encephalopathien bei Mensch und Tier werden diskutiert.

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Authors and Affiliations

  1. Neurological Institute and Department of Neurology, University of Vienna School of Medicine, Vienna, Austria

    K. Jellinger, W. -D. Heiss & E. Deisenhammer

  2. Wagner-Jauregg Hospital for Neuro-Psychiatry, Linz, Austria

    K. Jellinger, W. -D. Heiss & E. Deisenhammer

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Jellinger, K., Heiss, W.D. & Deisenhammer, E. The ataxic (cerebellar) form of creutzfeldt-jakob disease. J. Neurol. 207, 289–305 (1974). https://doi.org/10.1007/BF00312583

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