Dimova Petia S, Bojinova Veneta S
St Naum University Hospital of Neurology and Psychiatry, Sofia, Bulgaria.
J Child Neurol. 2004 Jul;19(7):548-52.
The 2-year history of a 10-year-old boy with subacute sclerosing panencephalitis is presented. After 6 months of epilepsy manifested by atypical absences and myoclonic-atonic seizures with an electroencephalographic (EEG) correlation of generalized spike-and polyspike-wave discharges, the child developed chorioretinitis and demonstrated a dramatic intellectual decline during corticosteroid treatment. Neuroimaging investigations did not demonstrate any pathologic changes, including at the time of fully developed disease, with neurologic deficits, periodic spasms, polymorphic epileptic seizures, and dementia. The typical generalized periodic complexes and general slowing replaced the epileptic abnormalities in the EEG. The initial anticonvulsant treatment was temporarily effective, and the purposeful isoprinosine therapy had no significant beneficial effect. The subacute sclerosing panencephalitis rapidly developed to stages III to IV, and only during the vegetative state did computed tomography show marked brain atrophy. This case is among the few described in the literature with atypical absences and myoclonic-atonic seizures as the first manifestation of subacute sclerosing panencephalitis.
本文介绍了一名10岁患亚急性硬化性全脑炎男孩的2年病史。在出现以非典型失神发作和肌阵挛 - 无张力性发作伴脑电图（EEG）显示广泛性棘波和多棘波放电为特征的癫痫6个月后，该患儿出现脉络膜视网膜炎，并在皮质类固醇治疗期间出现明显的智力衰退。神经影像学检查未发现任何病理变化，包括在疾病完全发展、出现神经功能缺损、周期性痉挛、多形性癫痫发作和痴呆时。EEG中典型的广泛性周期性复合波和普遍减慢取代了癫痫异常。最初的抗惊厥治疗暂时有效，而针对性的异丙肌苷治疗没有显著的有益效果。亚急性硬化性全脑炎迅速发展到III至IV期，仅在植物状态时计算机断层扫描显示明显的脑萎缩。该病例是文献中少数以非典型失神发作和肌阵挛 - 无张力性发作作为亚急性硬化性全脑炎首发表现的病例之一。
