Wang H S, Kuo M F, Huang S C, Chou M L
Department of Pediatrics, Chang Gung Medical College, Taiwan, Republic of China.
Pediatr Neurol. 1994 Nov;11(4):341-5. doi: 10.1016/0887-8994(94)90014-0.
Twelve children with type 1 herpes simplex encephalitis (3 with relapse, 9 without) have been monitored during the past 7 years. Ten of the children received intravenous infusion of acyclovir (30 mg/kg/day) for 10 days, 1 child who experienced relapse received 15 mg/kg/day, and another relapsed child received no antiviral agents until relapse. Relapse occurred 20-36 days after initial onset. All relapsed patients underwent another 10 days of acyclovir treatment (30 mg/kg/day). Choreoathetosis appeared as the initial sign of relapse followed by rapidly progressive unresponsiveness in all 3 relapsed patients: in 1 nonrelapsed patient choreoathetosis occurred during the recovery period. In these 4 patients involuntary movement was remitted within 3 months to 2 years. One patient with choreoathetosis died of measles pneumonia 4 months after onset of herpes simplex encephalitis and the surviving 3 were severely retarded. Although neuroimaging sparing of basal ganglia does not indicate structural and functional abnormalities, the disturbance of the neural connection among the basal ganglia and the cerebral cortex, which manifested severe damage over frontal, temporal, and parietal mantles on CT, may be the source of movement disorders in these patients. We conclude that choreoathetosis may be the first sign of relapse of herpes simplex encephalitis in children and may be an indicator of poor prognosis. The neuropathogenesis of choreoathetosis requires further investigation.
在过去7年中，对12例1型单纯疱疹性脑炎患儿（3例复发，9例未复发）进行了监测。10例患儿接受了静脉输注阿昔洛韦（30mg/kg/天）治疗10天，1例复发患儿接受15mg/kg/天治疗，另1例复发患儿在复发前未接受抗病毒药物治疗。复发发生在初次发病后20 - 36天。所有复发患者均接受了为期10天的阿昔洛韦再次治疗（30mg/kg/天）。舞蹈手足徐动症是所有3例复发患者复发的初始症状，随后迅速进展为无反应状态：1例未复发患者在恢复期出现舞蹈手足徐动症。在这4例患者中，不自主运动在3个月至2年内缓解。1例患有舞蹈手足徐动症的患者在单纯疱疹性脑炎发病4个月后死于麻疹肺炎，其余3例存活患者严重智力发育迟缓。尽管基底节在神经影像学上未受累并不表明存在结构和功能异常，但基底节与大脑皮质之间神经连接的紊乱（在CT上表现为额叶、颞叶和顶叶皮质严重受损）可能是这些患者运动障碍的根源。我们得出结论，舞蹈手足徐动症可能是儿童单纯疱疹性脑炎复发的首发症状，且可能是预后不良的指标。舞蹈手足徐动症的神经发病机制需要进一步研究。
