This 65-year-old right-handed woman was admitted to the hospital because of progressive confusion. She had been apparently healthy until about 2 months prior to admission, when she disclosed to her husband that “people were growing funny, long noses” and that “everyone on TV looked like cartoons.” By the time of our evaluation, 3 months into her illness, she was globally aphasic, exhibited an alien limb syndrome, and had increased sound-sensitive startle.

This patient had a rapidly progressive encephalopathy, heralded by the development of metamorphopsias (distortions of visual images, in this case expressed as “funny, long noses” she noted in people), followed by cortical blindness, and eventually aphasia, apraxia, a left dystonic and levitating arm, and ultimately (not shown) intermittent myoclonus. The protein 14-3-3 was elevated in cerebrospinal fluid (CSF). Her brain magnetic resonance imaging (MRI) showed a cortical ribbon in the parietooccipital region (Fig. 91.1). These features suggested the posterior or Heidenhain variant of CJD. The patient died 4 months after the onset of symptoms, 1 month after the video was recorded.