Progressive subacute sclerosing panencephalitis: A study of two cases with epileptic spasms

Abstract

Myoclonus has been described as the most frequent type of seizures associated with progressive subacute sclerosing panencephalitis (SSPE). We report two patients who developed SSPE. All patients had a history of measles. The children presented with repetitive episodes of head and trunk drops with frequent falls. Polygraphic electroencephalography (EEG) recording showed electroclinical features compatible with epileptic spasms. The periodic complexes remained unchanged during sleep without concomitant epileptic spasms. The epileptic spasms had a relatively good response to vigabatrin. EEG, cerebrospinal fluid studies, and cerebral magnetic resonance imaging confirmed the diagnosis. Epileptic spasms are probably more frequently associated with SSPE than the literature mentions. Polygraphic-EEG recording is crucial to define the electroclinical features in these patients. The recognition of the types of seizures in SSPE does not only allow for adequate treatment, but also defines the clinical features of this severe and progressive encephalopathy.

Keywords

Epileptic spasms - measles - progressive encephalopathy - refractory