Search

Purpose: Hypothalamic hamartomas (HHs) are a malformation of the ventral hypothalamus and tuber cinereum, associated with gelastic seizures and epilepsy. We sought to determine the spectrum of electroencephalography (EEG) abnormalities in a large cohort of HH patients., Methods: Data was collected for HH patients undergoing evaluation between 2003 and 2007. Data included seizure history, prior treatment, and results of diagnostic studies. After informed consent, data were entered into a database., Key Findings: We reviewed 133 HH patients. Mean age at time of data analysis was 15.7 years (59.4% male). Most patients had gelastic (77%) and/or complex partial seizures (58%). Records for 102 EEG studies on 73 patients were reviewed. Interictal epileptiform abnormalities were seen in 77%, localizing predominately to the temporal and frontal regions. Records for 104 video-EEG (VEEG) studies on 65 patients were reviewed. Of 584 gelastic seizures (GS) captured, no ictal EEG change was noted in 438 (75%). Of GS with localizing features, 89% suggested onset from the temporal and/or frontal regions. There were 160 complex partial seizures (CPS). For those with localizing features, 100% localized to the temporal and/or frontal head regions. EEG and VEEG findings correlated with the side of HH attachment. VEEG did not influence outcome., Significance: EEG features in HH patients are diverse. The majority of gelastic seizures fail to demonstrate change in the EEG. The lack of EEG changes with many clinical seizures, and the false localization seen in those events with an ictal change suggest the utility of EEG is limited in the evaluation of these patients., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: This study aims to examine the outcomes of ten patients after orbitozygomatic (OZ) pterional surgery in cases of refractory epilepsy caused by hypothalamic hamartomas (HH)., Methods: Ten patients with HH and treatment-resistant epilepsy (mean age 18.3 years, range 0.7 to 42.7) underwent HH resection with an OZ approach (n = 8) or an OZ approach combined with a transventricular endoscopic approach (n = 2). Follow-up for the patients ranged from 0.5 to 6.2 years (mean 3.1). Outcomes were prospectively monitored with the use of a proprietary database., Results: Four patients (40%) are seizure-free, and four (40%) have had greater than 50% reduction in seizures. One patient had no significant change in seizure frequency, and one patient died unexpectedly 2.8 years after surgery. Six patients had total or near-total HH resection (98-100% of HH lesion volume). Of these, four of six (66%) were seizure-free, and two had at least greater than 50% reduction in seizures. Residual complications include diabetes insipidus (n = 1), poikilothermia (n = 1), visual field deficit (n = 1), and hemiparesis (n = 1). Eight families (80%) reported improved quality of life., Conclusions: Patients with treatment-resistant epilepsy and tumors with an inferior or horizontal plane of attachment to the hypothalamus should continue to be approached from below. Those with both intrahypothalamic and parahypothalamic components may require approaches from above and below, either simultaneously or staged. For appropriately selected patients, the success of controlling seizures with an OZ is comparable to results utilizing transcallosal or transventricular approaches. The likelihood of controlling seizures appears to correlate with extent of resection.

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.

Background: Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection., Methods: Between October 2003 and April 2005, 42 consecutive patients with refractory epilepsy who underwent endoscopic resection of HH were studied prospectively. The endoscope was held by an articulated pneumatic arm and tracked with a frameless stereotactic neuronavigation system. Data collection and follow-up were performed by personal interview. Five patients were excluded. The remaining 37 patients (22 males, 15 females; median age 11.8 years; range 8 months to 55 years) had frequent and usually multiple types of seizures., Results: Postoperative MRI confirmed 100% resection of the HH from the hypothalamus in 12 patients. At last follow-up (median 21 months; range 13-28 months), 18 (48.6%) patients were seizure free. Seizures were reduced more than 90% in 26 patients (70.3%) and by 50% to 90% in 8 patients (21.6%). Overall, the mean postoperative stay was shorter in the endoscopic patients compared with our previously reported patients who underwent transcallosal resection (mean 4.1 days vs 7.7 days, respectively; p = 0.0006). The main complications were permanent short-term memory loss in 3 patients and small thalamic infarcts in 11 patients (asymptomatic in 9)., Conclusions: Endoscopic resection of hypothalamic hamartoma (HH) is a safe and effective treatment for seizures. Its efficacy seems to be comparable to that of transcallosal resection of HH, but postoperative recovery time is significantly shorter.