Improved developmental quality in a patient with trisomy 21 following treatment for refractory Lennox-Gastaut syndrome by total corpus callosotomy: a case report

The indications for surgical intervention for epilepsy associated with chromosomal abnormalities remain controversial. We report a 13-year-old girl with trisomy 21 and refractory epilepsy. The patient developed West syndrome at the age of 5 months. The seizures were refractory to multiple antiepileptic drugs, adrenocorticotropic hormone therapy, and a ketogenic diet. The patient developed Lennox-Gastaut syndrome at the age of 7 years. Frequent daily seizures including several sound-induced and tonic seizures impeded daily living of the patient and her caregivers. For alleviation of these seizures, the patient underwent total corpus callosotomy at the age of 13 years. The seizures disappeared almost completely, a state that continued throughout one year of follow-up. The patient's cognitive development also improved and her caregivers were satisfied with these outcomes. Appropriate surgical intervention may suppress seizures in patients with Lennox-Gastaut syndrome and trisomy 21.