Tourette’s-Like Syndrome and Dementia

Mr. A, a 79-year-old man with a 5-year history of frontal-temporal dementia, had developed disturbing tic-like behavior, consisting of slapping his forearm while grunting and swearing. This occurred many times per hour, generally without provocation, and was sufficiently disturbing that he and his wife were threatened with eviction by their landlord. He had been unable to tolerate a trial of haloperidol and had not responded to olanzapine or to quetiapine, the latter at 200 mg/day. Other behavioral problems included sexual disinhibition directed exclusively toward his wife and purposeless wandering. There was no evidence of mood disorder or psychosis.

Mr. A’s past medical and psychiatric history was unremarkable, and there was no prior history of tics. He had a remote history of moderate alcohol consumption. He was taking no medications. There was no family history of psychiatric or neurological illness. Upon a mental status examination, he exhibited a labile affect with clear witzelsucht. He was disoriented and amnestic, but his language was neurologically intact. He performed poorly on a test of controlled word fluency and showed paratonic rigidity bilaterally. A computerized tomography scan showed prominent frontal and anterior temporal atrophy. His sexual disinhibition was treated successfully with paroxetine, 40 mg/day, but this had no effect on his tic-like behavior. He was ultimately treated with clonidine, which was titrated without incident to 0.6 mg/day. This resulted in sustained, almost complete remission of the behavior over the past 5 months.