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Neurology 2002;58:1338-1347
© 2002 American Academy of Neurology

Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy

A. Palmini, MD PhD, C. Chandler, MD, F. Andermann, MD FRCP(C), J. Costa da Costa, MD PhD, E. Paglioli–Neto, MD MSc, C. Polkey, MD, B. Rosenblatt, MD FRCP(C), J. Montes, MD FRCS(C), J. V. Martínez, MD, J. P. Farmer, MD FRCS(C), B. Sinclair, MD FRCP(C), K. Aronyk, MD FRCS(C), E. Paglioli, MD PhD, L. Coutinho, MD PhD, S. Raupp, MD and M. Portuguez, PhD

From the Porto Alegre Epilepsy Surgery Program (Drs. Palmini, Costa da Costa, Paglioli–Neto, Martinez, Paglioli, Coutinho, Raupp, and Portuguez), Hospital São Lucas da PUCRS, Brazil; the Department of Neurology and Neurosurgery (Dr. Andermann), Montreal Neurological Institute, McGill University, Quebec, Canada; the Department of Neurosurgery (Drs. Chandler and Polkey), King’s College Hospital, London, United Kingdom; the Department of Neurology and Neurosurgery (Drs. Rosenblatt and Montes), Montreal Children’s Hospital, McGill University; and the University of Alberta Hospital (Drs. Sinclair and Aronyk), Edmonton, Canada.

Address correspondence and reprint requests to Dr. André Palmini, Serviço de Neurologia, Hospital São Lucas da PUCRS, Avenida Ipiranga 6690 #220, 90610-000, Porto Alegre, RS, Brazil; e-mail: apalmini{at}conex.com.br

Background: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently.

Objective: To report a series of 13 patients in whom the hamartoma itself was resected.

Methods: All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y).

Results: Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate.

Conclusion: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.




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