Paroxysmal dystonic choreoathetosis

Abstract

A 30-year-old man with mild learning disability (IQ:72) and cerebral palsy with mild generalized choreoathetosis was referred with paroxysmal motor disorder and episodic depressive mood disorder. Aged 11 months, he had developed episodes of alternating flaccid hemiparesis followed by episodes when he was unable to move or talk but remaining conscious lasting from several minutes to longer. Episodes could be precipitated by excitement such as opening Christmas presents or, later, his favourite football teams scoring a goal or losing. His longest interictal period had been 4 weeks. There was a family history of epilepsy. His EEG aged 6 showed paroxysmal spike and slow waves but no change during episodes of weakness. There was no improvement with phenobarbitone or phenytoin. Aged 1 I, oculogyric crises started during episodes. Aged 19, the EEG showed photosensitivity but was otherwise normal. Episodes were made worse with Sinemet or amphetamines. From age 20, he regularly needed 2 to 5 days a month off work because of episodes. From age 27, episodes could be followed by depression lasting days to months, including suicidal attempts such as swallowing bleach aged 29. Once started on carbamazepine 400 mgs daily, he still had episodes every 2 weeks but has not needed any further time off work, has had no further depressive episodes and is now able to live independently. He could still have rare episodes lasting up to 3 days such as when England won at Wembley.