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From the Departments of Neurology and Neurosurgery (Drs. Guerreiro, F. Andermann, Bastos, Dubeau, Snipes, Olivier, and Rasmussen) and Human Genetics (Dr. E. Andermann), and the Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada; Porto Alegre Epilepsy Surgery Program (Dr. Palmini), Hospital São Lucas da PUCRS, Porto Alegre, Brazil; and Division of Neurology and Neurosurgery (Drs. Hwang, Hoffman, and Otsubo), Hospital for Sick Children, University of Toronto, Ontario, Canada.
Dr. Guerreiro is currently affiliated with Campinas University (UNICAMP), Campinas, São Paulo, Brazil.
Address correspondence to Dr. Frederick Andermann, Montreal Neurological Hospital and Institute, 3801 University Street, H3A 2B4, Montreal, Quebec, Canada.
Background: Seizures in patients with tuberous sclerosis complex (TSC) are often intractable to antiepileptic medications and searching investigation may provide evidence that surgical treatment can be considered.
Objective: To review the results of investigation and surgical therapy, a treatment modality not generally considered in patients with medically refractory seizures and TSC.
Methods: We report 18 patients (9 male) with TSC who underwent surgical treatment of medically refractory epilepsy. Twelve patients had a well-localized epileptogenic lesion and were treated by lesionectomy or focal resection. Resections were: 7 frontal, 4 temporal, 1 frontotemporal, 1 occipital, and 1 frontoparietal. Four patients underwent more than one operation. Six patients had corpus callosotomy (CC).
Results: Follow-up ranged from 1 month to 47 years. Outcome of the patients treated by resection was excellent in 7 (5 were seizure-free and 2 had auras only), good in 1, fair in 3, and 1 was lost to follow-up. Best outcome was obtained in patients who had focal seizures and good imaging and EEG correlation, although they might have multiple seizure types, other imaging abnormalities, and multifocal or generalized EEG findings. When there was no such correlation, CC was found to be an option as five patients had at least some improvement and only one showed no change.
Conclusion: Surgical treatment of patients with TSC and intractable epilepsy is most effective when a single tuber or epileptogenic area can be identified as the source of seizures and resected. This may be possible even when other tubers or diffuse EEG abnormalities are present. In patients with unlocalizable epileptic abnormalities, palliation may be obtained by CC.
Dr. Guerreiro was supported by FAPESP and UNICAMP, Brazil.
Presented in part at the annual meeting of the American Epilepsy Society, Boston, December 1997.
Received February 9, 1998. Accepted in final form August 1, 1998.
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