NR AGMK
AU Kirk,A.; Ang,L.C.
TI Unilateral Creutzfeldt-Jakob disease presenting as rapidly progressive aphasia
QU Canadian Journal of Neurological Sciences 1994 Nov; 21(4): 350-2
PT journal article
AB A 64-year-old man presented with a three day history of progressive Broca's aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasic. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.
IN Ein 64 Jahre alter Mann starb 7 Wochen nach den ersten Symptomen an der Creutzfeldt-Jakob-Krankheit. Sein Gehirn zeigte fast nur in der linken Gehirnhälfte die typischen schwammförmigen Veränderungen.
MH Aphasia, Broca/*pathology; Brain/pathology; Case Report; Cerebellum/pathology; Cerebral Cortex/pathology; Creutzfeldt-Jakob Syndrome/*pathology; Diagnosis, Differential; Human; Magnetic Resonance Imaging; Male; Middle Age; Movement Disorders/pathology; Support, Non-U.S. Gov't; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed
AD Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatoon, Canada.
SP englisch
PO Kanada