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Objective: To determine whether patients with hypothalamic hamartoma (HH) improve in their cognitive functioning after neurosurgical resection of their HH and explore what variables correlate with cognitive outcome., Methods: Thirty-two patients underwent preoperative and postoperative neuropsychological testing. The age range of patients was between 3.3 and 39.3 years (mean 12.2 years, SD 7.0). The average time interval between surgery and postoperative neuropsychological testing was 23.4 months (range 5.1-47.2 months). Tests administered varied on the basis of the patient's age and clinical condition., Results: As a group, measures of overall intelligence showed improvement postsurgery, with associated improvement in processing speed. Memory scores did not demonstrate consistent improvement or decline. Duration of epilepsy, age at surgery, and level of neurocognitive functioning prior to surgery were correlated with postsurgical cognitive status. Patients who had mental retardation but were testable generally showed the greatest gains., Conclusions: Despite the great variability in level of cognitive impairment in patients with HH and refractory epilepsy, level of intelligence may show mild to moderate improvements postsurgery if no surgical complications occur. The variables that predict cognitive outcome are not fully delineated, but testable individuals with the greatest presurgical cognitive impairment and those with the shortest duration of epilepsy appear to make the greatest gains in intellectual functioning., Classification of Evidence: This study provides Class IV evidence that single surgical resection for HH was associated with improvement in some subset measures of intellectual functioning, but not memory. Factors that predict better outcomes cannot be determined.

We evaluated health-related quality of life in patients with hypothalamic hamartoma, to see how it differs from that of children with more common neurologic disorders. We used the PedsQL 4.0, along with the Child Behavior Checklist, Hague Seizure Severity Scale, and Side Effects Scale, to evaluate presurgical patients with hypothalamic hamartoma and epilepsy (n = 21). The results were compared with those of age-matched cohorts with migraine (n = 19) and Benign Epilepsy with Central Temporal Spikes (n = 11). In comparison with the migraine group, the patients with hypothalamic hamartoma had decreased health-related quality of life across all domains of the PedsQL 4.0. Compared with the benign epilepsy group, the hypothalamic hamartoma cohort has a significantly lower score in School Function. Comorbid psychomotor retardation was predictive of lower quality of life. Research examining the efficacy of recently developed surgical treatments for hypothalamic hamartoma should include health-related quality of life as an outcome measure.

Objective: The goal of the work described here was to examine the relationship between intellectual test performance in patients with hypothalamic hamartoma (HH) with refractory epilepsy and their seizure histories, as well as the size and neuroradiographic anatomical features of the HH. It was predicted that the level of estimated intelligence and the pattern of intellectual test performance would significantly correlate with the size of the HH and neuroanatomical features., Method: In this cross-sectional design study, 49 patients with HH between the ages of 5 and 55 years were classified by age at time of examination, as well as pattern of performance on the Wechsler intelligence scales. All patients were included in data analysis irrespective of their ability to participate in psychometric testing. Patients with a prior history of neurosurgical treatment were excluded., Results: For those patients functionally capable of participating in cognitive testing (n=42), a summary index score, which estimated level of intellectual function (composed of the Vocabulary, Block Design, and Coding subtests of the Wechsler intelligence scales), was significantly correlated only with number of antiepileptic drugs (AEDs) the patient was taking at the time of evaluation (r=-0.66, n=38, P=0.05). In contrast, a categorization method addressing the pattern of intellectual test performance (including those patients who were not functionally capable of participating in cognitive testing, n=49) was significantly correlated with number of AEDs (r=+0.35, n=48, P=0.01), size of HH (r=+0.38, n=49, P=0.01), presence of precocious puberty (PP: r=+0.41, n=49, P=0.01), and anatomical classification of HH (r=+0.39, n=49, P=0.01)., Conclusions: The findings confirm the wide range of cognitive functioning in the population of patients with HH and refractory epilepsy, and suggest that multiple variables are correlated with intellectual test performance in patients with HH with refractory epilepsy. Although the present cross-sectional design study does not answer the question of whether or not epilepsy severity produces lower intelligence in this patient population, number of AEDs and neuroanatomical features of the HH lesion are identified as being significantly related to cognitive performance in this patient sample.