A 67-year old woman presented with visual disturbancesfollowed by rapid cognitive decline in three months.She was disoriented on admission, with spatial hemineglect,oculomotor apraxia, and levitation of her left hand.She could not recognize numbers or letters during visualtests. Examination revealed prominent frontal reflexes,limb rigidity, and bilateral Babinski’s sign. A brain MRIshowed cortical DWI hyperintensities (Figure 1). EEG waspositive for generalized complex periodic waves and CSFanalysis revealed elevated 14-3-3 protein levels, leading tothe diagnosis of probable Creutzfeldt-Jakob Disease (CJD).The Heidenhain variant of CJD is an atypical presentationwith predominant visual disturbances followed by rapidlyprogressive dementia1,2.
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