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NEUROLOGY 1985;35:254
© 1985 American Academy of Neurology

Ataxic Creutzfeldt-Jakob disease

Diagnostic techniques and neuropathologic observations in early disease

H. Royden Jones, Jr, MD, E. Tessa Hedley-Whyte, MD, Stephen R. Freidberg, MD and Richard A. Baker, MD

Departments of Neurology (Dr. Jones), Neurosurgery (Dr. Freid-berg), and Diagnostic Radiology (Dr. Baker), Lahey Clinic Medical Center, Burlington, the Charles S. Kubik Laboratory of Neuropathology, Massachusetts General Hospital, Boston, (Dr. Hedley-Whyte), and Harvard Medical School, Boston, MA.

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.

Address correspondence and reprint requests to Dr. Jones, Department of Neurology, Lahey Clinic Medical Center, 41 Mall Road, Box 541, Burlington. MA 01805.

Presented in part at the thirtyfifth annual meeting of the American Academy of Neurology, San Diego, CA, April 1983.

Accepted for publication June 5, 1984.




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