Paroxysmal kinesigenic choreoathetosis

Overview

A rare inherited neurological disorder involving short, recurring attacks of involuntary movement. The attacks usually only last for 5 to 10 seconds.

Symptoms

* Involuntary movement * Episodic dystonic movements

Diagnosis

If possible, include the patient’s family in history taking; they may be more aware of behavior changes than the patient is. Begin by asking them when dystonia occurs. Is it aggravated by emotional upset? Does it disappear during sleep? Is there a family history of dystonia? Obtain a drug history, noting especially the use of a phenothiazine or an antipsychotic. Dystonia is a common adverse effect of these drugs, and the dosage may need to be adjusted to minimize this effect. Next, examine the patient’s coordination and voluntary muscle movement. Observe his gait as he walks across the room; then have him squeeze your fingers to assess muscle strength. Check coordination by having him touch your fingertip and then his nose repeatedly. Follow this by testing gross motor movement of the leg: Have him place his heel on one knee, slide it down his shin to the top of his great toe, and then return it to his knee. Finally, assess fine-motor movement by asking him to touch each finger to his thumb in succession.