A 12-year-old girl with a family history of migraine and attacks of hemiplegia was treated successfully with topiramate (TPM), in a report from the University Hospital of Messina, Italy; and Leiden University Medical Centre, The Netherlands. Hemiplegic attacks began at 7 months of age, they averaged 2 or 3 times per week, lasted 24 to 36 hours, resolved with sleep, shifted from one side to the other, and were associated with pendular nystagmus. Attacks were triggered by emotional stimuli or fever. Her psychomotor development was delayed; she talked at 3 years and walked at age 4.5 years. A video-EEG showed posterior slow waves during an attack of hemiplegia. At follow-up she had dystonia, dysarthria, ataxia, and pyramidal tract signs. Treatment with valproate, carbamazepine, and benzodiazepine failed to control attacks, and flunarazine 5 mg/day reduced duration of attacks to 2 to 3 hours for 5 months, Attacks and motor and social skills worsened, and her IQ measured by the WISC was 48. TPM was introduced, 1-3 mg/kg/day, with a prompt reduction in severity and frequency of attacks, and continued improvement of dystonia and ataxia through 18-month follow-up. The mechanism of action was attributed to the inhibition of carbonic anhydrase. [1]

COMMENT. Alternating hemiplegia of childhood (AHC) typically begins in infancy, and the etiology is unknown. It resembles migraine and familial hemiplegic migraine (FHM), but only a few familial cases of AHC have been reported (Ped Neur Briefs Jan 1993 and Nov 1994) [2, 3, 4]. Flunarazine is the most frequently used treatment, with variable response. In the present case, TPM was effective and deserves further study.