Initial clinical and radiological findings in patients with SSPE: are they predictive of neurological outcome after 6 months of follow-up?

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive, devastating neurologic disorder caused by mutant measles virus. In this study we evaluated the prognostic value of neuroimaging abnormalities in SSPE. Thirty consecutive patients with SSPE were included. Diagnosis of SSPE was based on the criteria described by Dyken. Patients were followed for 6 months. Neuroimaging studies were performed at inclusion and after 6 months. Regression or progression of the disease was defined as a change of one, or more than one, stage in Jabbour’s staging system. Degree of disability was assessed using the modified Rankin scale (mRS) score. Neuroimaging abnormalities were seen in 27 patients. Dominant imaging abnormalities were cerebral atrophy, white matter signal changes, cortical grey matter abnormalities and signal change in the basal ganglia. After 6 months of follow-up, 18 (60%) patients had a poor outcome (mRS score: 3–6); one patient died. The remaining 12 patients (40%) had a stabilized clinical condition (mRS score: 0–2). On univariate analysis, predictors of death or disability were: poor mRS score at baseline ( p = 0.003) and Jabbour’s clinical stage III ( p = 0.019). None of the neuroimaging abnormalities were associated with a poor prognosis or clinical deterioration ( p > 0.05). We conclude that we did not observe any association between cerebral neuroimaging at baseline and neurological outcome after 6 months in patients with SSPE.