Abstract

A 72-year-old right-handed man with a history of coronary artery disease, aortic valve replacement, and hypothyroidism presented with 3 years of progressive difficulty using his right upper extremity, which had resulted in a largely nonfunctional limb within a year. In contrast, he reported relatively intact left upper limb function. He had developed gait instability within 6 months of onset and by 2 years he was nonambulatory. He reported slurring of speech and difficulty moving his eyes to either side, but especially to the right. He denied visual loss, diplopia, cognitive decline, visual hallucinations, sensory loss, autonomic symptoms, sleep disturbance, or perception of an alien limb. There had only been a nonsustained levodopa response. His medications included atenolol, monopril, simvastatin, warfarin, and levothyroxine. There was no family history of neurologic disease. He denied toxic exposures and had a distant history of tobacco use. On examination, he was not orthostatic. He was fully oriented, could recite the months backwards, and had fluent speech and normal comprehension and naming. He recalled 2 out of 3 words after 5 minutes. He accurately drew a clock. He required 3 attempts to correctly imitate the Luria 3-step test (normal ≤2 attempts) and he could not sustain the sequence. The go–no go task consistently showed errors of commission and he was concrete with proverb interpretation. He had abnormal eye movements (videos on the Neurology ® Web site at www.neurology.org) and minimal dysarthria. He had mild hypophonia, a reduced blink rate, and bilateral lead pipe rigidity, greater on the right. Strength was full. There was no tremor or myoclonus. Additional motor features are demonstrated on the videos. He had normoactive reflexes and flexor plantar responses. While primary modality sensation was normal, he had bilaterally impaired 2-point discrimination, right hand astereognosis, and agraphesthesia, without extinction to double simultaneous touch. …

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