Abstracts

In addition to refractory epilepsy, hypothalamic hamartomas (HH) are associated with cognitive and behavioral disturbances that often appear to worsen over time. Recent neurosurgical advances have led to improved surgical and seizure outcome for patients with HH (Harvey et al., 2003). However, the cognitive outcome of these patients has not been systematically studied. The present study evaluated pre- and post-surgical neuropsychological functioning in a series of patients., Eleven HH patients were seen for pre- and post-surgical neuropsychological evaluations. Five patients (4 males; 10-16 years) had received no prior neurosurgical intervention. Standardized neuropsychological tests measuring a range of cognitive functions were administered. The 5 patients undergoing their first neurosurgery (endoscopic resection) had Type II (Delalande and Fohlen, 2003) HH with left-sided or bilateral attachment and seizure onset in infancy. Mean follow-up interval was 11.6 months. Pre-post surgical comparisons of neuropsychological and seizure variables were made., Pre-surgery, cognitive functioning of the HH patients ranged from mental retardation to average IQ, with the majority displaying some memory impairment. The 5 neurosurgery-na[iuml]ve patients averaged 2 anti-epileptic medications (AEDs) at the time of surgery and 1 at follow-up. All experienced a reduction in their seizures with 4/5 being seizure free. Two sustained small, clinically asymptomatic thalamic infarcts. Neuropsychologically, the patients averaged a 1 standard deviation or greater improvement in their information processing speed, attention, Full Scale IQ, and left-hand motor speed. None experienced a clinically significant decline in their intellectual functioning. There were inconsistent changes in memory functioning with a possible trend toward increased impairment. Similar trends were observed in the larger sample of 11 patients. Several patients were noted to have improved emotional/behavioral functioning post-surgery., Surgical resection of HH was associated with improved seizure control, information processing speed, attention, and intellectual functioning, while memory functioning tended to be impaired both pre- and post-surgery. Improved cognitive functioning may be partially related to decreased need for AEDs. Further study of the cognitive and behavioral outcome of HH patients is needed., (Supported by Barrow Neurological Foundation; Women[apos]s Board of the BNI.)