We report a favorable outcome of multiple subpial transaction (MST) in two patients who had intractable atypical infantile spasms preceded by partial seizures, without any lateralized magnetic resonance imaging (MRI) abnormalities. CASE REPORT Case 1A 22-month-old boy had habitual spasms/jerks since contracting pneumococcal meningoencephalitis at age 14 months. Spasms lasted 1.5 s, but recurred in clusters of 10 to 40, and occasionally up to 100 spasms, with eight to 10 clusters occurring each day. He cried between spasms or after a cluster. Behavior changes, with arousal, extension of the left arm, left deviation of the head, and complex rotation of the eyes were observed before a spasm cluster. Ictal EEG recordings showed a transition of interictal hypsarrhythmic activity into generalized attenuation. During a spasm, the EEG demonstrated high-voltage, bilaterally asynchronous slow waves. Small-amplitude, fast-activity signals emerged between spasms. EEG activity between clusters consisted of random high-voltage slow waves and spikes. Patient's spasms initially responded to adrenocorticotropic hormone (ACTH) and vigabatrin, but relapsed. Although the brain MRI revealed diffuse destruction, right frontotemporal hypometabolism on the interictal positron emission tomography (PET) scan suggested the corresponding epileptogenicity of partial seizures.To delineate the surgical margin, we examined the epileptic activity with a 20-contact subdural grid. Guided by the electrocorticography (ECoG) findings, subpial transections were performed. Extensive areas of cortex were covered by upfront mapping and monitoring during MSTs. The mean number of daily spasms declined to 43.5 ± Accepted November 7, 2005. Address correspondence and reprint requests to Dr. Y-L. Hsin at Department of Neurology, Tzu Chi General Hospital, 707, Chung Yang Road, Sec. 3, Hualien, Taiwan, ROC, 970. E-mail: hsin.yloong@ msa.hinet.net 13.7 and 31.6 ± 15.9 in the 1-and the 6-month postoperative follow-ups, respectively. Developmental level, posture control, motor strategy, and forward reaching ability improved. Case 2A 3-year-old boy, with marked hypotonia and motor spasms since birth, had severe developmental delay and dependence on a positive-pressure oxygen delivery system. The etiology was not determined: chromosomal, metabolic, and brain imaging studies were negative. The mean daily spasm number was 57.0 ± 24.1. The onset of spasms was heralded by upward gaze, facial grimacing, and oral automatisms. Video-EEG showed runs of spikes over the left frontotemporal region. Spasm/jerks lasted 2 s, accompanied by raising of the left arm and flexion of hips and legs. A suppression-burst EEG pattern occurred during a spasm cluster and the interictal state. Although the brain MRI failed to show any abnormality, interictal PET revealed left frontotemporal hypometabolism, corresponding to the surface EEG localization of ictal discharges.Based on presurgical assessments and intraoperative ECoG, MSTs were performed. A small piece of the cortex was sent for pat...
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