Malformation of cortical development and epilepsy. Surgical perspective
DOI:
https://doi.org/10.47924/neurotarget2010390Keywords:
cortical development malformations, refractory epilepsy, cortical dysplasia, dual pathology, surgical proceduresAbstract
Objective: To evaluate the results of surgical treatment in a group of patients with refractory epilepsy associated with cortical development malformations (CDM).
Patients and methods: We performed a retrospective study of 160 patients selected from a total of 581 patients (27.5%) operated for refractory epilepsy associated with some kind of cortical development malformations (CDM), since January 1988 until April 2010, at the National Pediatric Hospital “Juan P Garrahan”, FLENI Institute, Children Hospital “R. Gutierrez” and Hospital “Cosme Argerich”, all of them from Buenos Aires, Argentina. Patients underwent surgery after a complete preoperative evaluation that included: clinical features, scalp EEG, video EEG, CT scan, MRI and neuropsychological evaluation. For the evaluation of postoperative results was used the classification of Engel.
Results: 160 patients were operated with refractory epilepsy associated with some kind of cortical development malformations (CDM); 91 males and 69 women, 124 children and 36 adults. The evolution of epilepsy had an average duration of 4.8 years in boys and 15.1 in adults. There was a wide range of types of seizures. Topographic location of brain injury: temporal, in 72 patients (43 children and 29 adults), and extratemporal in 88 (81 boys and 7 adults). 23 patients (14.4%) required chronic intracranial electrodes implantation. Surgical procedures: 137 surgical resections (65 lesionectomies, 38 standard anterior temporal lobectomies, 12 corticectomies, 10 antero-mesial resections of Spencer, 5 amigdalohippocampectomies, 5 polectomies, 1 lobectomy and 1 anatomic hemispherectomy; 28 disconnecting procedures (10 functional hemispherectomies, 4 hemispherotomies, 2 hemi-hemispherectomies, 3 multiple subpial transection and 2 callosotomies), 1 vagus nerve stimulator (VNS), as the first procedure, and radiosurgery 1. Intraoperative neurophysiological studies: Intra-op ECoG was performed in 60 patients, SSEP were in 5 and cortical stimulation in 2. Surgery under intra-op MRI was performed in 6 patients. Histopathologic findings: Focal cortical dysplasia in 97 patients (60.6%), dysembryoplastic neuroepithelial tumors in 33 (20.6%), tuberous sclerosis in 12 (7.5%), hemimegalencephaly in 10 (6.2%) hypothalamic hamartomas in 5 (3.1%), and alterations of hippocampus in 3 (1.8%). We found 24 cases of “dual pathology” (focal cortical dysplasia associated with temporal mesial sclerosis) and 16 “triple pathologies” (simultaneous presence of focal cortical dysplasia, ganglioglioma and temporal mesial sclerosis) and “associated pathologies” (with low-grade tumors and AVM). Postoperative results according to the Engel classification: Class I are: 120 (75%), class II: 17 (10.6%), class III: 7 (4.3%), class IV: 3 (1.8%) patients. Less than 1 year post-op, 4; not classified, 9 patients.
Discussion and conclusions: The CDM were present in 27.5% (160/581) of patients who underwent surgery for refractory epilepsy. These patients usually present an early onset and unfavorable evolution of epilepsy. However, response to surgical treatment was good with 137/160 (85.6%) in Engel class I and II. In addition, all patients with “double”, “triple” or “associate” pathologies are all in classes I and II of Engel, namely, good and good results. The results suggest that surgery should be considered as an early therapeutic option for epilepsy associated with malformations of cortical development.
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Copyright (c) 2010 Hugo B. Pomata, Marcelo Bartuluchi, Fabiana Lubieniecki, Juan Pociecha, Roberto Caraballo, Esther Cáceres, Claudio Vázquez, César Petre, Carlos D’giano
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