Partially Successful Treatment of Rasmussen's Encephalitis with Zidovudine: Symptomatic Improvement Followed by Involvement of the Contralateral Hemisphere

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Abstract
Summary: An 18‐year‐old woman with a 4‐year history of Rasmussen's encephalitis (RE) manifested by progressive aphasia, right hemiparesis, and nearly continuous intractable simple partial seizures that frequently secondarily generalized was treated with zidovudine (AZT). Seizures had not responded to any of the major antiepileptic drugs (AEDs) administered to high and toxic levels, or to ACTH. AZT was given for 62 days and discontinued because of granulocytopenia. Within 6 weeks of AZT initiation, seizure stopped and neurologic deterioration was arrested for ∼21 months. Subsequently, partial somatomotor seizures developed, affecting the previously uninvolved left hemibody. A 2‐week repeat course of AZT was attempted, but unremitting fever and gastrointestinal (GI) side effects precluded continuation of AZT treatment. Spontaneous, sustained remission of seizures has not been reported in untreated RE. Seizure control and arrest of neurologic deterioration in this case outlasted use of AZT by 19 months. Because of the apparent response of this patient to AZT, clinical studies designed to assess long‐term palliative/curative properties of antiviral agents, particularly in patients with involvement of the hemisphere dominant for language, appear warranted.