Hamano Kimiko, Watanabe Akimitsu, Kohyama Jun
Department of Pediatrics, Tsuchiura Kyodo Hospital, Tsuchiura, Ibaragi.
No To Hattatsu. 2003 Jan;35(1):59-64.
We present here a 5-year-old girl with acute encephalities with refractory, repetitive partial seizures (AERRPS), a new clinical entity defined by the following five criteria: 1. acute encephalitis with a prolonged acute phase of more than 2 weeks, 2. persistent partial seizures with identical phenotype both in the acute and recovery phase, 3. seizures frequently evolving into convulsive status especially during the acute phase, 4. extremely intractable, and 5. no causative lesion or agent is identified. Interestingly, her seizures had completely diminished from the fifty-sixth day of her illness with concomitant appearance of choreo-ballistic involuntary movements. After the 120th day of the illness, seizures evolved again, though the involuntary movements persisted. This transient disappearance of intractable seizures might provide a clue to the pathophysiology of seizures in AERRPS.
我们在此报告一名5岁患急性脑病伴难治性反复部分性癫痫发作(AERRPS)的女孩,AERRPS是一种新的临床病症,由以下五条标准定义:1. 急性脑炎,急性期延长超过2周;2. 在急性期和恢复期均有持续的、表型相同的部分性癫痫发作;3. 癫痫发作频繁演变为惊厥持续状态,尤其是在急性期;4. 极其难治;5. 未发现致病病变或病因。有趣的是,在其患病第56天时癫痫发作完全消失,同时出现舞蹈样-弹道式不自主运动。患病120天后,癫痫发作再次出现,尽管不自主运动持续存在。这种难治性癫痫发作的短暂消失可能为AERRPS癫痫发作的病理生理学提供线索。