Department of Paediatrics, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India.
Department of Paediatrics, Midnapore Medical College, Midnapore, West Bengal, India.
Neuropediatrics. 2021 Feb;52(1):52-55. doi: 10.1055/s-0040-1715623. Epub 2020 Oct 16.
Subacute sclerosing panencephalitis (SSPE) is a rare and progressive inflammatory disease of central nervous system due to aberrant measles virus with an outcome that is nearly always fatal. In acute fulminant SSPE, the disease rapidly evolves leading to death within 3 months of the diagnosis. We report here four cases of fulminant SSPE with atypical presentations, two of them presented at very early age with history of congenital measles infection in first case and gait abnormality as initial symptom in second case; acute disseminated encephalomyelitis (ADEM) with refractory seizures in third case, unilateral myoclonus with hemiparesis in fourth case at the onset of disease, respectively. The typical periodic electroencephalographic (EEG) complexes, elevated cerebrospinal fluid (CSF), and serum antimeasles antibodies in our patients led to the diagnosis of SSPE. A high index of clinical suspicion in fulminant type with awareness of atypical features, EEG, and CSF studies are of paramount importance in establishing its diagnosis.
亚急性硬化性全脑炎（SSPE）是一种罕见的进行性中枢神经系统炎症性疾病，由异常麻疹病毒引起，几乎总是致命的。在急性暴发性 SSPE 中，疾病迅速进展，导致诊断后 3 个月内死亡。我们在此报告了 4 例具有非典型表现的暴发性 SSPE，其中 2 例在非常早的年龄出现，第 1 例有先天性麻疹感染史，第 2 例有步态异常作为首发症状；第 3 例为急性播散性脑脊髓炎（ADEM）伴难治性癫痫发作，第 4 例在疾病发作时出现单侧肌阵挛伴偏瘫。患者的典型周期性脑电图（EEG）综合波、升高的脑脊液（CSF）和血清抗麻疹抗体导致了 SSPE 的诊断。在暴发性疾病中，具有高度的临床怀疑，对不典型特征、脑电图和 CSF 研究的认识对于确立其诊断至关重要。
