Atypical adult-onset subacute sclerosing panencephalitis

Abstract

Although subacute sclerosing panencephalitis is almost exclusively a childhood disease, it can occur in adults as well. We present an atypical case of adult-onset subacute sclerosing panencephalitis. The disease was characterized by prolonged insidious course followed by accelerated and aggressive phase, atypical EEG findings, and absence of myoclonic jerks. The diagnostic and treatment-related pitfalls are discussed.

Keywords: Adult onset; MRZ (measles, rubella, varicella-zoster) reaction; Multiple sclerosis; Subacute sclerosing panencephalitis.

Fig. 1

Axial fluid attenuation inversion recovery (FLAIR) sequence of the brain: (a) hyperintensity in subcortical gray matter of temporal lobes; and (b) in periventricular white matter (arrows) with diffuse brain atrophy.

Fig. 2

Histopathologic examination revealed perivascular infiltration of T-lymphocytes (hemalaun-eosin, X20) (a); microglial nodules, reactive astrogliosis (hemalaun-eosin, X40) (b); and neuronophagia, marked with asterisks (hemalaun-eosin, X40) (c), compatible with viral encephalitis.

Fig. 3

Electroencephalography showing periodic biphasic and triphasic delta wave discharges bilaterally and synchronously, larger in amplitude over the left temporo-occipital region, occurring at intervals of 3-4 s.