Subacute Sclerosing Panencephalitis in a 63-Year-Old Woman Presenting as Generalized Choreoathetosis

Abstract

The persistence of measles virus infection in childhood and early adolescence can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE), characterized by behavioral disturbances and intellectual disability followed by myoclonic jerks and occasional negative myoclonus. Movement disorders are rarely presenting manifestations in SSPE. We herein report a 63-year-old woman with generalized choreoathetosis as the presenting manifestation of stage-I SSPE. Our case was atypical for the patient's age and clinical presentation with generalized choreoathetosis and bilateral putaminal and caudate nucleus signal hyperintensity. Though highly uncommon, neurologists should keep SSPE as a differential diagnosis among patients with movement disorders. Measles-endemic countries should be more vigilant to the atypical and rare presentations of SSPE, such as generalized choreoathetosis.

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

“… 19,23–31 In most patients, chorea/athetosis was noted to develop during the course of the disease, although acute development was reported in one patient 32 . Although most of these patients were children or young adults, one case was reported in a 63‐year‐old female, who presented with asymmetrical generalized chorea, cognitive decline, myoclonic jerks and pyramidal signs 28 . A 15‐year‐old male was reported to have ballistic movements, combined with seizures, myoclonus and cognitive impairment 33 …”

“…13,17,18 We could find 10 case reports of patients with SSPE who had prominent chorea (Table 2). 19,[23][24][25][26][27][28][29][30][31] In most patients, chorea/athetosis was noted to develop during the course of the disease, although acute development was reported in one patient. 32 Although most of these patients were children or young adults, one case was reported in a 63-year-old female, who presented with asymmetrical generalized chorea, cognitive decline, myoclonic jerks and pyramidal signs.…”

“…32 Although most of these patients were children or young adults, one case was reported in a 63-year-old female, who presented with asymmetrical generalized chorea, cognitive decline, myoclonic jerks and pyramidal signs. 28 A 15-year-old male was reported to have ballistic movements, combined with seizures, myoclonus and cognitive impairment. 33 In terms of distribution, chorea may be focal or unilateral but may eventually generalize.…”