Psychiatric aspects of patients with hypothalamic hamartoma and epilepsy.

Uncontrolled rage, while long associated with hypothalamic hamartoma, has not been as extensively studied as the epilepsy. Rage can be more detrimental to quality of life than seizures. It is now realized that behavior and aggression improve after a complete resection of the hypothalamic hamartoma correlating with a good seizure control post-surgically. We report on the longitudinal psychiatric history of a patient with hypothalamic hamartoma and rage whose severe and refractory epilepsy was ultimately treated by thalamic and intrahamartoma chronic stimulation. Our patient did not exhibit sham rage typical of hypothalamic lesions, but rather multifactorial aggressive bouts typical of challenging behaviors seen with mental retardation. The anxious and social features of the aggression suggest that psychiatric interventions, which have been neglected as the emphasis has been on seizure control, are worthwhile in the overall management of this difficult case.