Paroxysmal Kinesigenic Dyskinesia - A Case Series of 20 Children From North India.

BACKGROUND

Paroxysmal kinesigenic dyskinesia, though has world-wide distribution, is more common in persons of Asian lineage. There are no large case series of this entity from India.

OBJECTIVES

To describe clinical characteristics of paroxysmal kinesigenic dyskinesia from India.

METHODS AND MATERIAL

Retrospective chart review of patients with paroxysmal kinesigenic dyskinesia from North India.

RESULTS

We identified 20 patients, 16 boys and 4 girls, from 16 families with paroxysmal kinesigenic dyskinesia. The age at presentation ranged from 5 to 28 years. The age at onset varied from 4 years to 19 years with 75% children having onset between 10 years and 19 years. There was average delay of 2.7 years (range 1 month to 10 years) between the onset of symptoms and the diagnosis. Nine patients had misdiagnosis of epilepsy. Clinical diagnosis of paroxysmal kinesigenic dyskinesia was corroborated by videos and/or direct observation of the event in the clinic in 65% cases. Nineteen (95%) children had primary paroxysmal kinesigenic dyskinesia, and one had underlying hypoparathyroidism. Episodes of dyskinesia were brief, occurred several times a day, and were triggered by sudden movements in all. Symptoms were unilateral in ten, bilateral in eight, both unilateral and bilateral in one, and confined to the head and neck region in one. Two cases had convulsions during infancy. The majority of the children showed dramatic response to low-dose carbamazepine administered as a single daily nightly dose.

CONCLUSIONS

Paroxysmal kinesigenic dyskinesia is reasonably common in Indian children and has clinical presentation and therapeutic response similar to that described in the literature.