Department of Pediatrics, Assaf Harofeh Medical Center, Zerifin, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Pediatr Neurol. 2011 Jun;44(6):467-70. doi: 10.1016/j.pediatrneurol.2011.01.011.
Subacute sclerosing panencephalitis is a rare neurologic disorder of childhood and adolescence. We describe a 16-year-old boy who manifested the disease despite proper vaccinations. He was hospitalized because of bedwetting, involuntary limb movements, abnormal speech, and balance disturbances. Immunoglobulin G antibodies against measles were strongly positive, with a high relative cerebrospinal fluid/serum ratio. Polymerase chain reaction for measles produced negative results. Electroencephalography registered slow activity with high voltage discharges every few seconds, and with triphasic complex morphology. Magnetic resonance imaging revealed diffuse white matter changes, mostly around the posterior regions and lateral ventricles. Treatment with valproic acid, levetiracetam, carbamazepine, and intravenous immunoglobulin G was ineffective. Inosiplex and interferon-β-1a were also administrated. The patient became comatose, with generalized myoclonic jerks, and died 1 year later. An autopsy was not performed. This patient illustrates that subacute sclerosing panencephalitis should be suspected among young vaccinated subjects.
亚急性硬化性全脑炎是一种罕见的儿童和青少年神经疾病。我们描述了一名 16 岁的男孩,尽管进行了适当的疫苗接种,仍表现出这种疾病。他因遗尿、不自主肢体运动、言语异常和平衡障碍而住院。麻疹免疫球蛋白 G 抗体呈强阳性,脑脊液/血清相对比值较高。聚合酶链反应检测麻疹呈阴性。脑电图显示每隔几秒钟就会出现慢波活动和三相复合形态。磁共振成像显示弥漫性白质改变,主要位于后区和侧脑室周围。丙戊酸、左乙拉西坦、卡马西平及静脉注射免疫球蛋白治疗无效。还使用了肌苷和干扰素-β-1a。患者随后出现昏迷,全身出现肌阵挛性抽搐,1 年后死亡。未进行尸检。该患者表明,在接种疫苗的年轻人群中应怀疑亚急性硬化性全脑炎。