Adult-onset subacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks

Background:    Subacute sclerosing panencephalitis (SSPE) is a progressive, devastating neurologic disorder caused by mutant measles virus. SSPE is a rare neurologic disorder of childhood and adolescence and after adolescence is extremely rare.
    Case Report:    A 26-year-old boy was admitted to the clinic because of behavioral changes consist of intellectual decline, slowed speech, mental confusion, and in advanced stages he showed progressive right-sided weakness and myoclonic jerks or stereotypic movements in the left arm. He contracted measles at 1 year of age. His prenatal, natal and development milestones were normal except for difficulties walking due to poliomyelitis sequelae, and his mental development was normal. The diagnosis of SSPE was confirmed by anti-measles antibodies in the cerebrospinal fluid. Cerebral MRI showed diffuse white matter abnormalities in fronto-occipital regions. MRG done outside our clinic 1 month into the illness showed bilateral, newly-formed basal ganglia and thalamic lesions. The major abnormality on EEG was the appearance of a periodic EEG pattern with bilateral sharp, sharp and wave, and spike complexes. One month later he was in a vegetative state and only episodic visual contact was possible. Glasgow coma scale was 6 of 15 (E4,M1,V1). The disease progressed and he was discharged home in a dependent state. This case is interesting because of the late onset and rapid progression of the disease.
    Conclusions:    Clinical features of patients with SSPE who have uncommon presentations or features of the disease may lead to diagnostic difficulties. Our experience with this case suggests that clinicians should be aware of similar clinical characteristics.

Keywords: subacute sclerosing panencephalitis, measles, adult-onset , rapid progress, subacute sclerosing panencephalitis, Measles, adult-onset, rapid progress