Subacute Sclerosing Panencephalitis in Children: A Case Series in Dr. Cipto Mangunkusumo Tertiary General Hospital, Jakarta, Indonesia

Objective: Subacute sclerosing panencephalitis (SSPE) is a rare and progressive inflammatory disease of central nervous system due to aberrant measles virus with a fatal outcome. In acute fulminant SSPE, the disease rapidly evolves leading to death within three months. We aimed to present three cases of children with SSPE at Dr. Cipto Mangunkusumo Tertiary General Hospital, Jakarta, Indonesia.

Cases: We report three cases of SSPE based on Dykens criteria with different stages (Jabbour’s clinical stages). The first case female, 6 years old had atonic seizure as initial symptom (clinical stage IIIB); male, 6 years old had motoric regression in second case (clinical stage IIIA): male, 6 years old had gait abnormality and jerking movement in third case (clinical stage IIIA). The typical periodic lateralized epileptiform discharges (PLEDs) were found in these patients and elevated cerebrospinal fluid (CSF) and serum anti-measles antibodies in our patients led to the diagnosis of SSPE. All patients received Isoprinosine and supportive therapies. We also planned to try ketogenic diet or intravenous immunoglobulin. All patients are on long term monitoring and follow up.

Conclusion: A rare case of SSPE could be suspected in children with atonic seizure, motoric regression, and jerking movement who are not responding to standard treatment. Patients with suspected SSPE need to be monitored closely in long term due to high chance of fatal and progressive neurologic deterioration. Isoprinosine, intravenous immunoglobulin, or ketogenic diet may be give benefit to SSPE but, nowadays there is no consensus about treatment of SSPE.
Keywords: Subacute sclerosing panencephalitis, jerking movement, measles, intravenous immunoglobulin, ketogenic diet