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Creutzfeldt-Jakob disease

Some unusual morphological features reminiscent of kuru

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Summary

The neuropathological findings in 3 cases of Creutzfeldt-Jakob disease are described.

The triad of morphological changes, i.e. neuronal loss, status spongiosus of the grey matter and proliferation and hypertrophy of fibrous astrocytes with or without microglial reaction, conforms to the typical pattern of pathology described in the literature. Degeneration of the cerebellum places these cases in the cortico-striato-cerebellar variant of the disease.

The distribution of the lesions within cerebrum and cerebellum, with a more severe involvement of phylogenetically older portions, is unusual. In the cerebrum cortices belonging to the limbic system and the striatum were the most severely affected, whilst in the cerebellum the vermis and flocculo-nodular lobe bore the brunt of the pathological process. A similar distribution of the lesions is generally found in kuru.

Further similarities with kuru are pointed out i.e. the occurrence of swollen, chromatolytic neurones predominantly within the infragranular layers of the cerebral cortex, of coarse intracytoplasmic vacuolation in many of the large striatal nerve cells and of “kuru” plaques within the cerebellum. Similarities and differences between the two diseases are discussed.

Zusammenfassung

Die neuropathologischen Befunde von 3 Fällen von Creutzfeldt-Jakobscher Krankheit werden beschrieben.

Die Trias der morphologischen Veränderungen — Nervenzelluntergang, Status spongiosus in der grauen Substanz, Proliferation und Hypertrophie faserbildender Astrocyten mit und ohne Mikrogliareaktion — entspricht dem typischen Muster der pathologischen Veränderungen in anderen Fällen der Literatur. Ihre Kleinhirnbeteiligung reiht die Fälle in die cortico-striato-cerebelläre Variante der Krankheit ein.

Ungewöhnlich ist die Verteilung der Veränderungen im Großhirn und Kleinhirn mit der Bevorzugung phylogenetisch älterer Anteile. Am Großhirn waren die Rindenabschnitte des limbischen Systems und das Striatum am stärksten befallen; am Kleinhirn hat der pathologische Prozeß hauptsächlich den Vermis und Lobus flocculo-nodularis angegriffen. Eine ähnliche Anordnung der Veränderungen findet sich bei der Kuru-Krankheit.

Weitere Ähnlichkeiten mit Kuru werden hervorgehoben, wie das Vorkommen von geschwollenen chromatolytischen Nervenzellen, besonders in den infragranulären Großhirnrindenschichten, von groben intracytoplasmatischen Vacuolen in vielen großen Striatumneuronen und von „Kuru“-Plaques im Kleinhirn. Ähnlichkeiten und Unterschiede der beiden Krankheitsbilder werden diskutiert.

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Krücke, W., Beck, E. & Vitzthum, H.G. Creutzfeldt-Jakob disease. Z. Neurol. 206, 1–24 (1973). https://doi.org/10.1007/BF00316141

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