Hypothalamic Hamartoma With Infantile Spasms: Case Report With Surgical Treatment.

ABSTRACT

We report a 10-month-old boy with treatment-resistant infantile spasms associated with hypothalamic hamartoma (HH). Electroencephalography before surgical treatment showed modified hypsarrhythmia. Transventricular endoscopic resection and disconnection resulted in immediate and enduring disappearance of the epileptic spasms and improvement in the postoperative electroencephalography. After 8 years of treatment, the patient has nondisabling gelastic seizures associated with a small amount of residual HH but no other seizure types. He is not taking any antiepilepsy drugs. He is academically and socially successful. We are not aware of any prior reports of surgical treatment of HH with concurrent infantile spasms as an uncontrolled seizure type. The immediate disappearance of infantile spasms demonstrates that the HH lesion itself is an active and necessary component within the epileptic network responsible for spasms in this particular condition. This case contributes to the recognition that focal pathologies can be responsible for infantile spasms with hypsarrhythmia and respond successfully to surgical intervention.