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Creutzfeldt–Jakob disease (CJD) is a rapidly progressive and ultimately lethal dementing illness caused by an infectious protein named prion. Besides cognitive decline and psychiatric or behavioral abnormalities, various other neurologic deficits appear. Myoclonic jerking, in particular startle myoclonus, is seen most commonly. The lack of a characteristic clinical course (especially during the initial stages) makes the diagnosis difficult, but the potential transmissibility necessitates early recognition of the disease (for additional information, please access our Web site at www.neurology.org and click on the title link for this article).1 MRI abnormalities of the brain can aid in establishing the diagnosis (for additional information, please access our Web site at www.neurology.org and click on the title link for this article).2,3 We describe a patient with sporadic CJD in whom MRI of the brain revealed signal intensity changes of the cerebral cortex that were best demonstrated on fluid attenuated inversion recovery (FLAIR) images.
A 54-year-old woman was admitted to the hospital in August 1998 with a history of depressed mood and a gradual deterioration of cognitive functions since December 1997. She had increasing forgetfulness and concentration problems and experienced difficulty performing routine activities. Cognition had slowed and declined noticeably. Expressing herself verbally as well as understanding …
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