Guillain-Barre syndrome with hyperreflexia: A variant

Abstract

Subacute sclerosing panencephalitis (SSPE) is typically observed in school-going children, adolescents, and young adults. Herein, we report a 3-year-old boy of SSPE and systematically review all such reported cases (age ≤3 years). We searched PubMed database on June 16, 2020 with “English language & Human only” restrictions. Eligible publications were screened and retrieved by three authors (NK, SP, and RU). Variables like author, year of publication, country, age of onset, sex, duration of illness, immunization, history of measles, clinical presentation, neuroimaging, treatment, and outcome were extracted. Neurological outcome was assessed in terms of improvement, static course or death. The defined search criteria resulted in 791 manuscripts (1951 to June 16, 2020). Only 47 manuscripts were eligible for data extraction. Seventy cases were retrieved. The mean age of onset was 26.34 months. The M:F ratio was 3.2:1. Turkey (19 cases), India (10 cases), and Japan (10 cases) collectively comprised more than half cases of SSPE. Only six children were immunized, 17 have no measles immunization, and the rest 47 had insufficient immunization records. Preceding history of measles was present in 27 cases. Most patients presented in stage II/III with altered sensorium, myoclonic jerks, and seizures. The most common reported outcome was death (18/70) followed by vegetative state (10/70). The absence of measles vaccination is frequently associated with childhood SSPE. Early measles vaccination may prevent SSPE in toddlers. Perinatal measles infection results in short onset latency and fulminant course. Outcome was not good in reviewed age group of ≤3 years.