The dementias of the Creutzfeldt-Jakob group have been the subject of much controversy since their first descriptions by Creutzfeldt2 in 1920 and Jakob18 in 1921. Their etiology and pathogenesis are unknown, and both the clinical and morphological manifestations have often been under debate. In that member of the group usually referred to as Creutzfeldt-Jakob disease, the most characteristic clinical picture is that of a rapidly progressing intellectual deterioration, having its onset in the middle decades of life, and accompanied by signs of involvement of the extrapyramidal and pyramidal motor systems, and sometimes of the lower motor neurones. There may be hyperkinetic manifestations, often of a myoclonic type, along with spasticity and rigidity. The average duration of the disease is about 12 months. Pathologically, there are cytological alterations, cell loss, and status spongiosus affecting primarily the cerebral cortex, caudate nucleus, putamen, and anterior columns of the spinal cord, with
FRIEDE RL, DeJONG RN. Neuronal Enzymatic Failure in Creutzfeldt-Jakob Disease: A Familial Study. Arch Neurol. 1964;10(2):181–195. doi:10.1001/archneur.1964.00460140067009
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