Abstracts

Neuropsychological impairments have been reported in children and adults with hypothalamic hamartomas (HH). Mechanisms responsible for these impairments have remained unclear. Small sample sizes have limited our ability to examine the relationship between clinical and brain imaging variables and neuropsychological impairments.
The present study evaluated the correlates of neuropsychological dysfunction in 58 HH patients with refractory epilepsy., Fifty-eight HH patients (between 5 and 55 years of age; mean age = 15.97 years) were administered various neuropsychological tests, depending on their clinical state. An attempt was made to administer to all subjects at least three subtests of the Wechsler Intelligence Scale (Vocabulary, Block Design, and Coding). These variables were used to estimate verbal and nonverbal problem-solving skills, as well as processing speed. The sum of these three subtests was also calculated to obtain a marker of their relative cognitive status.
Age of seizure onset, age at time of testing, lifetime duration of epilepsy, the size and location of the HH, and the presence of precocious puberty (PP) were recorded. The size of HH in cubic centimeters was determined via MRI scanning. The Delalande and Fohlen (2003) method of classifying HH typology was employed.
Analysis of variance and multiple regression analyses were performed to determine the relationship of neuropsychological findings to the clinical and MRI data referenced above., As a group, HH patients showed significant neuropsychological impairments, but great variability was noted. The size of the HH and the absence of PP predicted level of cognitive impairment. Age of seizure onset, age at testing, and duration of lifetime epilepsy were not related to neuropsychological status. Those patients whose HH volume was 2 cubic centimeters or greater and who had seizure onset within the first month of life, showed the worst neuropsychological outcomes, with the effect being most pronounced for processing speed. The Delalande and Fohlen (2003) anatomical classification system significantly related to the degree of neuropsychological impairment observed. Mentally retarded HH patients tended to show large HH volumes which displaced the third ventricle, and frequently involved the mamillary bodies., The size of HH in conjunction with early seizure onset was most predictive of overall cognitive status in 58 HH patients who had refractory epilepsy. Moreover, the anatomical location of the HH was associated with cognitive status. Surprisingly, the absence of PP was associated with better cognitive test performance., (Supported by Barrow Neurological Foundation
Women[apos]s Board of the BNI.)