Abstracts

Hypothalamic hamartomas (HHs) are rare developmental malformations of the hypothalamus that typically result in gelastic and other intractable seizures. The epileptic syndrome associated with HH characteristically begins in infancy but delayed diagnosis is not uncommon until later in life. Prior studies had shown successful surgical resection of HH in children, but little is known about adult patients. We describe the outcome of surgical resection in 20 adult patients who had HH and intractable seizures., We evaluated adult patients who underwent resection of HH at age 18 or older. HHs were removed by a transcallosal-interforniceal or endoscopic approach. Additional data regarding their seizure type, seizure frequency, use of anticonvulsants, cognitive function, size of resection, seizure outcome, and surgical complication were collected by personal interview. Patients with at least a 12-month follow-up period were included in this study. Their surgical outcome was measured by seizure frequency and cognitive changes., Twenty patients underwent HH resection at age 18 years or older (11 men). Mean age at the time of surgery was 26.6 years and ranged from 18 to 55. Gelastic seizures were the initial type in all patients, but the diagnosis was delayed as late as age 21 (mean 5.3). Prior to surgery, all of them had multiple types of seizures and typically more than once a day. One half of the patients were taking 3 or more anticonvulsants and 9 patients had prior surgical treatments including vagus nerve stimulator implantation in 7. HH was removed through an endoscopic approach in 18 and transcallosal in remaining 2. Pre- and post-surgical MRI volumetric evaluation revealed more than 75% of the HH volume was resected in 14 patients including complete resection in 9 (mean 79.5%). The most common surgical complication was thalamic infarction, which was seen in 3 patients with endoscopic approach but recovered fully without functional deficit. Mean follow-up period was 22.3 months in 19 patients (range 13 to 39 months, one lost in follow-up). Fourteen out of 19 patients (73.7%) reported seizure reduction of greater than 90%, three 50-90%, and two less than 50%. Among the 6 patients who had experienced complete resolution of seizures, 4 had endoscopic resection. Ten patients (52.6%) reported improved overall cognition whereas 4 reported worsening after surgery., Surgical resection of HH in adult patients is safe and effective treatment for medically refractory seizures. The finding of this study is similar to the previously reported cases in children., (Supported by Barrow Neurological Foundation.)