Subacute sclerosing panencephalitis with a fulminant course: Case series

Abstract

Subacute sclerosing panencephalitis (SSPE) is a life-threatening and delayed complication of measles. It may rarely follow a fulminant course, with rapid progression to a vegetative state. There have been case reports of this challenging and aggressive form of SSPE in previously immunised children. The cause of the rapid progression in many patients is unknown. However, it is postulated that genetically determined immune dysfunction can prevent cell-mediated immune clearance of the measles virus. This was a retrospective study of five patients who presented with clinical features and laboratory investigations suggestive of fulminant SSPE over the previous year. The diagnosis of SSPE was established using Radermecker’s complexes in electroencephalogram, encephalitis on brain magnetic resonance imaging and elevated titres of immunoglobulin (Ig) M and IgG measles antibodies in the serum and cerebrospinal fluid. Treatment included isoprinosine, intravenous Igs, sodium valproate, levetiracetam and clobazam. The outcome was dismal in all patients, with progression to a bedridden state despite timely intervention. In our series of five cases, the diagnosis of SSPE was often missed because the children were immunised and healthy before the onset of symptoms. However, after excluding other central nervous system viral infections, the clinical presentation and laboratory findings corroborated the fulminant course of SSPE. This article captures the presentation and outcomes of five patients and emphasises the importance of clinicians’ awareness of this condition.

Keywords

• measles
• myoclonus
• neuroregression