Unité de Neurologie et de Neurorehabilitation Pédiatrique, Département Médico-Chirurgical de Pédiatrie, CHUV, Lausanne, Switzerland.
Eur J Paediatr Neurol. 2011 Nov;15(6):544-6. doi: 10.1016/j.ejpn.2011.05.001. Epub 2011 May 31.
Subacute sclerosing panencephalitis (SSPE) is a rare and severe long-term complication of measles. Hallmarks of this entity include progressive cognitive decline, myoclonia, a generalized periodic pattern on EEG and deep white matter abnormalities on MRI. However, imaging can be normal in early stages.
We report herein the case of a previously healthy 13-years-old girl with an unusual radiological presentation.
She presented with unilateral myoclonia, cognitive decline with memory impairment and a first brain MRI with swelling of both hippocampi mimicking limbic encephalitis. Measles antibodies were positive in CSF and the EEG showed slow periodic complexes.
This unusual radiological presentation has never been described in SSPE. Relationship between virus and limbic system are discussed.
亚急性硬化性全脑炎（SSPE）是麻疹的一种罕见且严重的长期并发症。这种疾病的特征包括进行性认知能力下降、肌阵挛、脑电图上出现广泛周期性模式以及 MRI 上深部白质异常。然而，在早期阶段影像学可能正常。
我们在此报告一例此前健康的 13 岁女孩，其影像学表现不典型。
她表现为单侧肌阵挛、认知能力下降伴记忆力障碍，以及首次脑部 MRI 显示双侧海马肿胀，类似于边缘性脑炎。CSF 中麻疹抗体阳性，脑电图显示缓慢周期性复合波。
这种不典型的影像学表现从未在 SSPE 中描述过。讨论了病毒与边缘系统的关系。
