A case of fulminant subacute sclerosing panencephalitis presenting with acute myoclonic-astatic epilepsy

Abstract

The neurologic sequelae post-measles are less common than other complications measles-related and can lead to severe disability or death: primary measles encephalitis (PME), acute post-infectious measles encephalomyelitis (APME), Measles Inclusion Body Encephalitis (MIBE), and Subacute Sclerosing Panencephalitis (SSPE). SSPE syndrome can affect people years from the acute measles virus infection, as result of the persistence of defective viral particles in brain cells. Clinical onset typically manifests with progressive intellectual deterioration, behavioral changes, and myoclonic jerks. The course of SSPE in the majority of affected children is that of a progressive worsening with fatal outcome within 2 years. This report described an Italian case of fulminant SSPE syndrome that led to death within few months from the initial onset.