Seizure outcome and complications following hypothalamic hamartoma treatment in adults: endoscopic, open, and Gamma Knife procedures

Clinical article

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  • 1 Department of Neurology, University of Colorado School of Medicine, and
  • 2 Department of Pediatric Neurology, Colorado Children's Hospital, Denver, Colorado;
  • 3 Departments of Neuroradiology,
  • 4 Neuropsychology/Neuroimaging Lab,
  • 5 Neurology,
  • 7 Neurosurgery, and
  • 8 Neuroscience, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona;
  • 9 Department of Pediatric Neurology, Phoenix Children's Hospital, Phoenix, Arizona; and
  • 6 Department of Neurosurgery, Chiari Institute Long Island, Great Neck, New York
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Object

This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH).

Methods

Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death.

Results

Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm3. In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS.

Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg).

Conclusions

Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.

Abbreviations used in this paper:AED = antiepileptic drug; EEG = electroencephalography; GKS = Gamma Knife surgery; HH = hypothalamic hamartoma; ILAE = International League Against Epilepsy.

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Contributor Notes

Address correspondence to: Cornelia Drees, M.D., Department of Neurology, University of Colorado School of Medicine, Research Complex 2, Room 5020, 12700 East 19th Avenue, Aurora, Colorado 80045. email: cornelia.drees@ucdenver.edu.

Please include this information when citing this paper: published online June 8, 2012; DOI: 10.3171/2012.5.JNS112256.

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