A Case of Alternating Hemiplegia of Childhood / 대한소아신경학회지

ABSTRACT

Alternating hemiplegia of childhood(AHC) is a rare but clinically distinct syndrome characterized by onset before 18 months of age, frequent attacks of alternating paralysis, nystagmus transient ocular palsies, other autonomic dysfunction, and the development of cognitive impairment and a choreoathetotic movement disorder. The case is a 13 month-old boy who has presented repeated episodes of alternating hemiplegia from the age of 6 months at a frequency of several times per month, which was lasting for two or three days. Ths diagnosis was based on clinical features. Frequently such hemiplegic attacks were accompanied by irratability. He had mental retardation, neurodevelopmental delay, and seizure attacks. EEG, brain MRI, brain MR angiography, and Tc-99m HMPAO-brain single photon emission computed tomography(SPECT) failed to reveal any significant abnormal finding during the hemiplegic attacks. We tried calcium-entry block and flunarizine to relieve the hemiplegic attacks, but the frequency and severity of the hemiplegic attacks were not decreased by flunarizine therapy.