Subacute sclerosing panencephalitis: Disease profile in early childhood

Abstract

Subacute sclerosing panencephalitis (SSPE), a progressive inflammatory neurodegenerative disease of the central nervous system caused by the measles virus, is experiencing a significant resurgence following recent measles epidemics. This study aimed to investigate the pathology of SSPE in early childhood and determine its clinical, electrophysiological, and radiological characteristics.

Materials and methods: This retrospective, descriptive study was conducted at the Pediatric Department of Nafissa Hamoud University Hospital in Algiers. The study included children aged 2-6 years who were hospitalized for subacute neurological disorders. Data collection spanned a 3-year period from January 1, 2020, to December 31, 2022.

Results and discussion: A total of 47 patients were examined, comprising 37 boys and 10 girls. The average age was 45.9 months (range: 24-72 months). Notably, 41 (87 %) of the patients had not received measles vaccination, and 37 (80 %) had contracted the measles virus, with 31 cases (83.8 %) occurring in children under 1 year of age. SSPE manifested approximately 3 years after infection (range: 1-5 years). Clinical events primarily included myoclonic seizures (26 cases), tonic-clonic seizures (6 cases), atonic seizures (6 cases), and focal seizures (3 cases); movement disorders were observed in 25 patients, and cognitive decline in 28 patients. Encephalitis manifestations, such as alterations in consciousness or behavior and psychiatric symptoms, were frequently observed. Electroencephalograms (EEG) revealed characteristic periodic complexes in only 22 patients. Magnetic resonance imaging (MRI) scans were normal in 24 patients but showed characteristic anomalies in 21 patients, primarily consisting of white matter hyperintensity and cortico-subcortical atrophy.

Conclusion: SSPE is not rare in early childhood. The latency period between virus infection and disease onset can be very short. Clinical, electroencephalographic, and radiological signs of the disease in young children may be atypical. Genetic studies are necessary to establish a genetic predisposition to the disease. Measles vaccination remains the most effective preventive measure against SSPE.

Keywords: Encephalitis; Measles; Myoclonus; Periodic; Vaccination.