Abstracts

Rationale: Hypothalamic hamartomas (HH) are rare congenital mass lesions, located in the ventral hypothalamus, associated with treatment-resistant epilepsy. Symptoms usually begin during early childhood, with gelastic seizures and/or central precocious puberty. Most patients develop additional seizures types during school age, along with difficulties with behavior and cognition. Behavioral problems usually consist of mood disturbance and poor frustration tolerance, leading to rage attacks, and may not respond to accepted therapies. Surgical resection of HH can lead to complete seizure control in 50-60% of patients. Many families have noted a co-incident improvement in behavior (Ng YT, et al, Epilepsia, 2006). We describe surgical outcome in a cohort of 4 patients, three with relatively infrequent seizures, and one without a history for seizures, who underwent resection of HH for psychiatric indications. Methods: The 4 patients (3 male) had a mean age of 11.9 years (range 9.8 - 15.0 years) at the time of surgery. Of the three patients with epilepsy, all had gelastic seizures, and two had additional types (complex partial and secondarily generalized tonic-clonic). Seizures were incompletely controlled, but relatively infrequent. One HH patient had no history for epilepsy. All patients had socially disabling psychiatric symptoms, including rage attacks with destruction of property, physical aggression toward family and peers, depression, and anxiety, which failed to respond to pharmacotherapy. Psychiatric diagnoses included Oppositional Defiant Disorder, Attention-Deficit/Hyperactivity Disorder, Mood Disorder, and Anxiety Disorder. All patients had a history of violence, and two patients required psychiatric hospitalization. One patient had borderline mental retardation while the other three had normal intelligence. Follow-up ranged from 6 months to 5 years following surgery (n = 1 transcallosal, n = 2 pterional, n = 1 endoscopic). Results: All patients demonstrated dramatic behavioral improvement following HH resection, with decreased irritability, better frustration tolerance, and improved social functioning. There have been no episodes of violence against others or destruction of property. Two patients were able to reduce or discontinue psychotropic medications, two patients were able to successfully return to a normal classroom, and one patient was able to return home after having been in residential placement. Two of the three patients with epilepsy were seizure-free and one had a 50-90% improvement in seizure frequency. Conclusions: This case series suggests that surgical removal of the HH may be effective in improving severe mood lability and poor frustration tolerance, even in the absence of epilepsy, or with relatively infrequent seizures. Surgical removal of HH for behavioral indications should be regarded as investigational, but worthy of further study conducted under Institutional Review Board (IRB)-approved protocols.