Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma

Objective: To characterize the cognitive deficits in children with gelastic seizures and hypothalamic hamartoma and investigate the relationship of se izure severity to cognitive abilities. Methods: Eight children with gelasti c seizures and hypothalamic hamartoma completed a neuropsychological batter y of standardized and age-normed tests, including the Woodcock-Johnson Psyc he-Educational Battery-Revised: Tests of Cognitive Ability, Peabody Picture Vocabulary Test-III, and initial-letter word fluency measure. Results: All children displayed cognitive deficits, ranging from mild to severe. Gelast ic/complex partial seizure severity was correlated with broad cognitive abi lity standard scores (r = -0.79; r(2) = 0.63; (F[1,6] = 10.28; p = 0.018]. Frequency of gelastic/complex partial seizures was also correlated with bro ad cognitive ability standard scores (r = -0.72; r(2) = 0.52; F[1,6] = 6.44 ; p = 0.044). Significant intracognitive standard score differences were fo und, with relative weaknesses in long-term retrieval (mean = 64.1; SD = 13. 3) and processing speed (mean = 67.7; SD = 21.6) and a relative strength in visual processing (mean = 97.6; SD = 12.8). Performance in visual processi ng differed from performance in long-term retrieval (p = 0.009) and process ing speed (p = 0.029). Conclusion: These findings are consistent with cogni tive functions and affective/emotional states associated with conduction pa thways of the hypothalamus involving cortical association areas and amygdal a and hippocampal formation. These abnormalities can account for the promin ent deficit found in integrating information in the processing of memories.