Resective surgery for medically intractable epilepsy associated with schizencephaly

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✓ Four adults with unilateral (three cases) or bilateral (one case) closed schizencephaly, medically intractable epilepsy, and otherwise normal neurological examinations are presented. Three were examined preoperatively with magnetic resonance imaging and one with computerized tomography. Scalp electroencephalographic (EEG) studies in all four patients and electrocorticography via intracranial electrodes in two showed seizure origin in the cleft regions in two patients and in the ipsilateral temporal lobe in the other two. Temporal lobectomy was performed in the two patients with temporal lobe foci. Resection of superficial pachygyria around the cleft was performed in one patient. The authors conclude that the abnormal cortical mantle lining schizencephalic clefts may be epileptogenic. Alternatively, temporal allocortex may become the source of seizures. Therefore, these patients require careful EEG monitoring of the entire ipsilateral hemisphere as well as the cleft region.

Article Information

Address reprint requests to: Daniel L. Silbergeld, M.D., Washington University Epilepsy Program, Department of Neurology and Neurological Surgery (Neurological Surgery), Campus Box 8057, 660 South Euclid Avenue, St. Louis, Missouri 63110-1093.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Left: Sagittal T1-weighted magnetic resonance (MR) image showing a schizencephalic cleft (arrows) extending from the sylvian fissure (arrowheads) into the somatosensory (postcentral) gyrus. Center: Sagittal T1-weighted MR image showing a submerged island of gray matter (arrows) at the medial depth of the schizencephalic cleft. Right: Intraoperative photograph showing sites (numbered 1 to 5) where contralateral face and upper extremity movements were elicited with electrocortical stimulation. The central sulcus (white line), the schizencephalic cleft splitting the postcentral gyrus (white arrows), and the vertical orientation of the temporal gyri and sulci (open arrows) are also viable.

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    Case 1. Ictal electrocorticographic (ECoG) recordings from a left-sided subdural 64-contact electrode grid. The contact in the inferior/anterior corner is numbered 1, the superior/anterior corner is 8, the inferior/posterior corer is 57, and the superior/posterior corner is 64. Ictal ECoG recording from a four-contact subtemporal strip electrode is also shown, with the medial contact numbered 1 and the most lateral 4. These recordings demonstrate electrical seizure onset from the medial subtemporal region.

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    Case 2. Sagittal T1-weighted magnetic resonance image showing a schizencephalic cleft (arrows) lined with thickened gray matter.

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    Case 3. Coronal T1-weighted magnetic resonance image showing bilateral schizencephalic clefts (arrows) lined with thickened gray matter and extending toward the occipital horns of the lateral ventricles.

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    Case 3. Ictal scalp electroencephalographic recordings demonstrating a left temporal onset of seizures (horizontal line).

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    Case 4. Coronal T1-weighted magnetic resonance image showing a schizenccphalic cleft (open arrows) extending to, but not opening into, the ventricle (black arrow).

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