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Creutzfeldt‐Jakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis
Author(s) -
Brown Paul,
RodgersJohnson Pamela,
Cathala Françoise,
Gibbs Clarence J.,
Gajdusek D. Carleton
Publication year - 1984
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410160305
Subject(s) - myoclonus , pathological , disease , spongiosis , medicine , gliosis , age of onset , incubation period , pediatrics , pathology , psychology , anesthesia , incubation , psychotherapist
Five to 10% of patients with Creutzfeldt‐Jakob disease have a clinical course that extends for 2 years or more. In the present series 33 (9%) of 357 histopathologically verified cases, including 15 (7%) of 225 transmitted cases, fell into this long‐duration category, the longest transmitted case having had a 13‐year duration. As a group these cases were characterized by a higher familial representation (30%), a younger age at onset (average, 48 years), and lower frequencies of myoclonus (79%) and periodic electroencephalographic activity (45%) than occur in series of unselected cases. The clinical course most often showed a long, very slowly progressive terminal stage with both mental and physical deterioration. Although transmission of disease by primate inoculation periods and durations of illness in the long‐duration cases (64%) than in cases of shorter duration (88%), incubation periods and duration of illness in the inoculated animals bore no relation to the duration of illness in the patients. Clinical differentiation from other chronic dementing processes, particularly Alzheimer's disease, can prove impossible; however, the correct pathologica diagnosis was readily evident by light microscopic examination, and spongiosis was at least as prominent as gliosis in all but 4(15%) of the 27 patients studied postmortem, the same frequency found in case series not selected for length of illness.