후두부 발작성 간질파와 동반된 양성 소아기 간질의 임상적 연구

Abstract

Background & Objectives : Although benign childhood epilepsy with controtemporal spikes (BCECT) has been well characterized and extensively studied, the clinical findings and prognosis of benign childhood epilepsy with occipital paroxysms (BCEOP) have been less well understood. The purpose of this study was to evaluate clinical manifestations, responses to antiepileptic drugs, and longterm. Prognosis of BCEOP. Methods and Subjects : We studied 10 BCEOP patients seen between 1985 and 1995 and followed up from 2 to 14 years(7.2±2.9). Results : In 10 patients, there were 7 females and 3 males. The age of onset ranges from 4 to 9 year-old(6.4±1.5). In the cases whose age of onset was below 6 year-old, the main clinical manifestations were tonic deviation of eye bal1, ictal vomiting and frequent development of status epilepticus in the sleep. In the cases whose age of onset was above 7 year-old, the main clinical manifestations were visual symptoms (blurring or blindness) associated with ictal vomiting/headache and complex visual hallucination evolved to automatism. The typical EEG findings were occipital paroxysms, characterized by repetitive high amplitude spike or sharp and slow wave complexes in the occipital area, mainly when the eyes were closed. Except one patient, the seizures responded well to anticonvulsant therapy and did not occur after the age of 11. Conclusion : Although the number of cases was small and the follow-up period was not long enough, our data indicated that BCEOP had a good prognosis and showed diverse clinical manifestations, which might be dependent on the age of onset.

저자들은 1985년 1월부터 1995년 10월사이에 동산병원에 내원하여 Panayiotopopoulos(1989)의 BCEOP 진단기준에 합당한 10례를 대상으로 경련양상, 약물에 대한 반응 및 예후에 대하여 알아 보았다. 1. 남녀비는 3:7이었고, 초발연령 분포는 4-9세였다. 2. 경련양상은 초발연령이 6세 이하인 5례는 구토를 동반한 긴장성 안구편위를 보였고 이들 중 4례는 상기 증상이 수면중에 간질중첩증으로 나타났다. 발병연령이 7세 이상인 4례에서는 경련 시작시에 시맹 혹은 시혼탁의 시증상후에 긴장성 안구 편위 및 구토를 보이는 양상과 복합 시환 각 증상후에 자동증이 동반되는 양상을 각각 2례에서 보였다. 3. 뇌파가 정상적으로 기록되는 연령은 10세부터 16세 사이였다. 4. 약물에 대한 반응은 1례를 제외한 9례에서 좋았으며, 9례중 4례는 약물중단 후에도 경련의 재발이 없었다.