Ataxic Creutzfeldt-Jakob disease: diagnostic techniques and neuropathologic observations in early disease

We studied two cases of ataxic Creutzfeldt-Jakob disease. EEG, CT, evoked responses, and CSF were normal in one purely ataxic patient. Diagnosis was established by cerebellar biopsy. Autopsy demonstrated devastating spongiform changes in the cerebellum, basal ganglia, and thalamus with rare focal ch...

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Bibliographic Details
Published inNeurology Vol. 35; no. 2; p. 254
Main Authors Jones, Jr, H R, Hedley-Whyte, E T, Freidberg, S R, Baker, R A
Format Journal Article
LanguageEnglish
Published United States 01.02.1985
Subjects
Ataxia - diagnosis
Ataxia - diagnostic imaging
Ataxia - pathology
Cerebellum - diagnostic imaging
Cerebellum - pathology
Creutzfeldt-Jakob Syndrome - diagnosis
Creutzfeldt-Jakob Syndrome - diagnostic imaging
Creutzfeldt-Jakob Syndrome - pathology
Electroencephalography
Evoked Potentials
Female
Humans
Male
Middle Aged
Tomography, X-Ray Computed
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Summary:We studied two cases of ataxic Creutzfeldt-Jakob disease. EEG, CT, evoked responses, and CSF were normal in one purely ataxic patient. Diagnosis was established by cerebellar biopsy. Autopsy demonstrated devastating spongiform changes in the cerebellum, basal ganglia, and thalamus with rare focal changes in cerebral cortex. In the second patient, late generalized changes developed with dementia. Diagnostic studies included abnormal visual evoked responses, CSF with abnormal oligoclonal bands and IgG, and subacute spongiform encephalopathy in frontal lobe biopsy. Early diagnosis is best established by biopsy of brain areas most likely to be involved on the basis of clinical neurologic findings.
ISSN:0028-3878
DOI:10.1212/wnl.35.2.254