INITIAL CLINICAL AND MRI FINDINGS IN SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE)

Objectives: SSPE is a persistent CNS infection caused by defective measles virus. Clinical course can be highly variable. Cognitive and neurological deterioration and myoclonus usually evolves to a vegetative, decorticated state and death. Diagnosis is based on clinical features, high titers of serum and cerebrospinal fluid measles antibodies, EEG and MRI.

Methods: A group of 14 children (11 boys, 3 girls) with SSPE, aged 4.5 to 14 years (mean 10.2) was diagnosed and treated in our Clinic from 1990 to 2005. Disease onset ranged from 4.5 to 12.5 years (mean 8.3). Initial MRI examination was performed in IB or IIA clinical stage of SSPE. Eleven of 14 children died after 3 to 19 months (mean 11.2). Imaging results were analyzed with respect to the clinical course and therapeutic response.

Results: Behavior disorder and/or learning difficulties of various severities were recognized as initial clinical picture in 5 children. Other patients presented with focal motor deficits (2), seizures (2), cortical blindness (1), optic disc swelling (1) and psychotic behavior (1). Segmental myoclonus in two children was the initial SSPE manifestation, whereas periodical myoclonic jerks followed first clinical finding for 15 days to 3.5 months. Initial CT scans in 3 patients showed moderate cortical atrophy and/or occipital hypodense area. First MRI disclosed no lesions in 3 patients. In 5/11 patients MRI revealed bilateral and diffuse abnormal T2 increased signal in the periventricular and subcortical white matter. Focal parenchymatous changes were seen in two, till lesions to the basal ganglia were disclosed in one boy. Two boys with normal initial MRI and fulminant SSPE died 3 and 5 months after the disease onset. Changes in MRI did not closely correlate with early therapeutic effects of oral inosiplex, intrathecal alpha-2b interferon or high-dose ivIgGs.

Conclusion: Initial clinical manifestations of SSPE are highly variable. First MRI findings were mostly abnormal and did not closely correlate with initial clinical presentation, SSPE stage and early treatment.