Fulminant adult-onset subacute sclerosing panencephalitis

Summary

We present the case of a young adult who developed acute encephalopathy with severe status epilepticus and rapid deterioration to a vegetative state and death within 6 weeks. Although the clinical picture, MRI and EEG findings were atypical, the hypothesis of subacute sclerosing panencephalitis (SSPE) was suggested by markedly increased intrathecal IgG synthesis in the cerebrospinal fluid (CSF) and diagnosis was confirmed by high antimeasles-antibodies in CSF and brain biopsy findings. Acute SSPE is an exceptionally rare and little-known form of SSPE with protean symptomatology and to our knowledge this case is the first observation of SSPE presenting with status epilepticus in an adult. Our case reiterates the need to include, even in developed countries, SSPE as a diagnostic possibility in unexplained acute encephalopathies.