소아에서 발생한 교대성 편마비 1례

A Case of Alternating Hemiplegia of Childhood

저자들은 29개월 된 남아에서 생후 6개월경부터 교대성으로 편마비 증상을 보였으며, 편마비 증상을 보일 때 시행한 뇌파 검사, 뇌 자기 공명 영상 촬영, 뇌 자기 공명 영상 혈관 조영술 및 뇌 단일 전자 방출 전산화 단층 촬영상 특이 소견을 보이지 않았던 소아 교대성 편마비 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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. The 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 compute 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.