Background

Subacute Sclerosing Panencephalitis (SSPE) is a fatal progressive neurological disorder following measles infection.

Methods

Cases were collated from Paediatric Neurology centres in the UK over 24 months from 2017 to 2019 and represent all cases referred to the National Viral Reference Department (VRD). Diagnosis was established with detection of a raised measles index, demonstrating intrathecal measles antibody production.

Findings

Six children presented with SSPE over two years, with median age five years (range 2–7 years) and median latency period three years (range 2–6 years). The majority were exposed to measles during infancy. Atypical features were common, including visual impairment, focal and generalised tonic-clonic seizures, headache, vomiting and movement disorders. EEG demonstrated typical features in five cases, though not always at presentation. Initial MRI was normal in four cases, with two showing focal and widespread white matter changes. Antiviral and immunomodulatory treatment led to minimal or no improvement. All progressed to cognitive regression, seizures and neurological decline within six months.

Interpretation

These cases demonstrate the highest incidence of SSPE in the UK since 2000, all progressing to acute fulminant disease, following younger age of onset, short latency period and atypical presentations. Recent global surges in measles cases raise the importance of clinician awareness of SSPE as a potential diagnosis in children with neurological regression. Herd immunity remains the key protective mechanism for infants and groups that cannot be vaccinated. Health care providers, educators and governments must ensure resources continue to target effective education and access to immunisation programmes, the only means to combat this devastating and fatal condition.

中文翻译：

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英国最近爆发的暴发性和早发性亚急性硬化性全脑炎 (SSPE)：麻疹时期的出现

背景

亚急性硬化性全脑炎 (SSPE) 是麻疹感染后一种致命的进行性神经系统疾病。

方法

病例是从 2017 年到 2019 年超过 24 个月从英国小儿神经病学中心收集的，代表了所有转介到国家病毒参考部门 (VRD) 的病例。诊断是通过检测到麻疹指数升高而建立的，证明鞘内产生了麻疹抗体。

发现

6 名儿童在两年内出现 SSPE，中位年龄为 5 岁（范围 2-7 岁），中位潜伏期为三年（范围 2-6 岁）。大多数人在婴儿期接触过麻疹。非典型特征很常见，包括视力障碍、局灶性和全身性强直阵挛发作、头痛、呕吐和运动障碍。EEG 在五个案例中表现出典型特征，但并不总是在演示中。四个病例的初始 MRI 正常，其中两个显示局灶性和广泛的白质变化。抗病毒和免疫调节治疗导致的改善很小或没有改善。所有这些都在六个月内进展为认知退化、癫痫发作和神经功能衰退。

解释

这些病例表明自 2000 年以来英国 SSPE 的发病率最高，在发病年龄较轻、潜伏期短和表现不典型后，均进展为急性暴发性疾病。最近全球麻疹病例激增提高了临床医生对 SSPE 作为神经退化儿童潜在诊断的认识的重要性。群体免疫仍然是无法接种疫苗的婴儿和群体的关键保护机制。卫生保健提供者、教育工作者和政府必须确保资源继续用于有效的教育和获得免疫计划的机会，这是对抗这种破坏性和致命疾病的唯一手段。