To highlight a case of Aicardi Syndrome in a patient with improved seizure control after undergoing hemispherectomy.

Aicardi syndrome (AS) is a rare X-linked genetic disorder characterized by infantile spasms, corpus callosal agenesis and chorioretinal lacunae. Most patients have shortened life expectancy, median survival of 18 years of age, psychomotor delay, and intractable epilepsy.

Post procedure, patient seizure frequency decreased significantly to twice yearly with 1 episode of isolated right hand shaking lasting less than one minute. In light of dramatic improvement in seizure frequency and severity, she weaned off multiple antiepileptic medications, now on cenobamate 300 mg and klonopin 0.5 mg daily. Positive nonseizure outcomes included improved verbal ability, ambulation, and behavior, as well as quality of life and functioning, with resolution of lethargy secondary to antiepileptics.

This case of AS demonstrates the spectrum of this rare disorder, with our patient unique in terms of longevity (age 27), and positive clinical response to epilepsy surgery after becoming refractory to multiagent pharmacotherapy.