89: HUNTINGTON DISEASE

PATIENT PRESENTATION

A 40-year-old man presents to the psychiatry emergency room for inappropriate behavior and confusion. He works as a janitor and has had reasonably good work attendance. His coworkers say that he has appeared “fidgety” for several years. They specifically mention jerky movements that seem to affect his entire body more recently. His mother is alive and well, although his father died at age 28 in an auto accident. On examination, he is alert but easily distracted. His speech is fluent but is noted to be tangential. He has trouble with spelling the word “world” backwards and counting in serial sevens, but recalls three objects at 3 minutes. When he walks, there is a lot of distal hand movement, and his balance is precarious, although he can stand with both feet together for several seconds. His deep tendon reflexes are increased bilaterally, and there is bilateral ankle clonus. A urine drug screen is negative.²

SIGNS AND SYMPTOMS

• Slight but evident alteration in character initially, followed by issues of self-control, eventually leading to failure of all cognitive functions

• Difficulty with attention and concentration, poor mental flexibility

• Movement abnormalities begin in the hands and face, initial slowing ...