Twenty-one-year course of adult-onset Rasmussen's encephalitis and bilateral uveitis: Case report

We report the longitudinal history of a 48-year-old, right-handed woman with Rasmussen's encephalitis (RE) who presented with seizures and cerebral atrophy confined to the left hemisphere, as well as with bilateral uveitis, during her 21-year disease course. Neurological symptoms included recurrent partial seizures with secondary generalized convulsions, reduced visual acuity of the left eye with optic atrophy, right hemianopsia, right hemiplegia and aphasia. MRI T2-weighted images revealed progressive atrophy and high signal intensity lesions localized in the left cerebral hemisphere. An interictal electroencephalogram showed slowing of background activities to 4–7 c/s and epileptiform discharges in the left hemisphere. Anti-glutamate receptor (GluR) ε2 IgG and IgM antibodies were detected in her serum. Our diagnosis was RE. Intravenous administration of high-dose methylpredonisolone immediately ameliorated her condition. Use of interferon β-1b, as well as immunosuppressants, appeared to reduce seizure frequency, prevented exacerbation of her other central nervous system symptoms and slowed development of brain hemiatrophy. Her case is notable because it was complicated with bilateral uveitis and managed favorably by immunotherapy.