Goraya Jatinder, Marks Harold, Khurana Divya, Legido Agustin, Melvin Joseph
Sections of Neurology, Department of Pediatrics, St. Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, Pennsylvania 19134, USA.
J Child Neurol. 2009 Jul;24(7):899-903. doi: 10.1177/0883073808330184. Epub 2009 Feb 9.
Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays. A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia. Brain magnetic resonance imaging revealed scattered areas of T2 and fluid-attenuated inversion recovery hyperintensities in the white matter consistent with acute disseminated encephalomyelitis. Despite treatment with intravenous methylprednisolone, intravenous immunoglobulins, and plasmapheresis, progressive neurological worsening occurred. Later during the course of her illness, subacute sclerosing panencephalitis was suspected from the appearance of burst-suppression pattern on electroencephalogram, and the diagnosis confirmed by elevated titers of measles antibodies in cerebrospinal fluid. Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.
亚急性硬化性全脑炎（SSPE）通常表现为进行性精神衰退、行为改变和肌阵挛性抽搐。非典型表现并不罕见，可能导致诊断延迟。一名9岁女孩在上呼吸道感染发作后出现平衡能力差和共济失调。神经系统检查发现轻度偏瘫和共济失调。脑部磁共振成像显示白质中散在的T2加权像和液体衰减反转恢复序列高信号区，符合急性播散性脑脊髓炎。尽管接受了静脉注射甲基强的松龙、静脉注射免疫球蛋白和血浆置换治疗，但神经系统仍进行性恶化。在病程后期，根据脑电图上出现的爆发抑制模式怀疑为亚急性硬化性全脑炎，脑脊液中麻疹抗体滴度升高证实了诊断。照顾儿童的医生需要了解亚急性硬化性全脑炎的非典型表现，并且必须保持高度的怀疑指数，以防止诊断延迟并避免不必要的诊断和治疗干预。
