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Courtney M. Schusse, Kris Smith and Cornelia Drees

OBJECTIVE

Hemispherectomy is a surgical technique that is established as a standard treatment in appropriately selected patients with drug-resistant epilepsy. It has proven to be successful in pediatric patients with unilateral hemispheric lesions but is underutilized in adults. This study retrospectively evaluated the clinical outcomes after hemispherectomy in adult patients with refractory epilepsy.

METHODS

This study examined 6 cases of hemispherectomy in adult patients at Barrow Neurological Institute. In addition, all case series of hemispherectomy in adult patients were identified through a literature review using MEDLINE and PubMed. Case series of patients older than 18 years were included; reports of patients without clear follow-up duration or method of validated seizure outcome quantification were excluded. Seizure outcome was based on the Engel classification.

RESULTS

A total of 90 cases of adult hemispherectomy were identified, including 6 newly added by Barrow Neurological Institute. Sixty-five patients underwent functional hemispherectomy; 25 patients had anatomical hemispherectomy. Length of follow-up ranged from 9 to 456 months. Seizure freedom was achieved in 80% of patients. The overall morbidity rate was low, with 9 patients (10%) having new or additional postoperative speech or language dysfunction, and 19 patients (21%) reporting some worsening of hemiparesis. No patients lost ambulatory or significant functional ability, and 2 patients had objective ambulatory improvement. Among the 41 patients who underwent additional formal neuropsychological testing postoperatively, overall stability or improvement was seen.

CONCLUSIONS

Hemispherectomy is a valuable surgical tool for properly selected adult patients with pre-existing hemiparesis and intractable epilepsy. In published cases, as well as in this series, the procedure has overall been well tolerated without significant morbidity, and the majority of patients have been rendered free of seizures.

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Cornelia Drees, Kevin Chapman, Erin Prenger, Leslie Baxter, Rama Maganti, Harold Rekate, Andrew Shetter, Maggie Bobrowitz and John F. Kerrigan

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.