Sébire G, Devictor D, Huault G, Aicardi J, Landrieu P, Tardieu M
Département de Pédiatrie, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
J Pediatr. 1992 Dec;121(6):845-51. doi: 10.1016/s0022-3476(05)80326-9.
We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.
我们报告了6名先前健康的儿童，他们在前驱疾病数天后出现了呈双相病程的急性脑病。这似乎构成了一种可识别的综合征，预后良好，可与Lyon等人先前定义的其他不明原因的脑病相鉴别。急性期以昏迷或意识模糊以及异常运动为主，包括杂乱的手势、口面部运动障碍发作或肢体肌张力障碍发作，并伴有持续性僵硬，最终发展为角弓反张姿势。仅在两名患者中观察到反复癫痫发作。所有患者的脑电图均记录到永久性慢波，即使在异常运动发作期间也是如此。在疾病全过程中，脑脊液和血清学检查结果均正常，病毒分离和抗病毒抗体检测均为阴性。脑部影像学检查要么未显示异常，要么提示有中度脑水肿。恢复期持续数周，其特征是运动功能迅速恢复，但行为和认知障碍持续存在。非语言推理能力在语言表达恢复正常之前很久就已恢复。4名患者最终完全康复，而2名患者有轻度后遗症。
