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December 1971

Creutzfeldt-Jakob Disease

Author Affiliations

Durham, NC
From the Divisions of Neurosurgery and Neurology, Duke University Medical Center and the Durham Veterans Administration Hospital, Durham, NC.

Arch Neurol. 1971;25(6):572. doi:10.1001/archneur.1971.00490060106011

IN 1920, Hans Gerhard Creutzfeldt of Berlin (1885 to 1964) published a detailed case report of a patient with an unusual combination of neurological signs and pathological findings.1,2 Then within three years, the neuropathologist Alfons Jakob of Hamburg (1884 to 1931), a student of Nissl and Alzheimer,3 described five similar cases and established this condition as a distinct disease entity.4-7 Despite Creutzfeldt's priority, the disorder is often called Jakob-Creutzfeldt disease because of Jakob's greater contribution to its delineation.8

It is now recognized that this disease or group of diseases is manifested by a rapidly progressive dementia combined with pyramidal and extrapyramidal signs, myoclonus, and often with amyotrophy.9-10 The disease affects middle aged individuals of both sexes and is usually fatal in a few months to a few years. Neuronal degeneration and gliosis occur in the brain and spinal cord, and glial rosettes may be found

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