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...
Dyke-Davidoff-Masson syndrome (DDMS) is a rare epilepsy syndrome that is characterized by cerebral hemiatrophy, homolateral skull hyperplasia, hyperpneumatization of the paranasal sinuses, seizures with or without mental retardation, and contralateral hemiparesis. We describe a case of DDMS in a 40-year-old female who had complex partial seizures with occasional secondary generalization since the age of 4 years. Her seizure frequency was 10-20 seizures/month even though she took four antiepileptic drugs. We applied magnetic resonance imaging (MRI), positron emission tomography (PET), functional MRI, and invasive electroencephalography (EEG) to define her epileptogenic and functional zones. Brain MRI showed prominent atrophy in the left frontal dorsal and lateral regions and mild atrophy of the left superior temporal gyrus and left parietal gyri. Interictal PET revealed decreased glucose metabolism in the atrophic regions. Functional MRI demonstrated that the inferior frontal and inferior parieto-occipital regions of the right hemisphere were activated by language testing. Invasive EEG revealed that the left lateral temporal lobe was the sole source of her seizures. Our results imply that the "metabolic border zone" rather than the atrophic region plays an important role in seizure activity, and that reorganization of functional zones occur after cerebral damage early in life.
Fibromyalgia is a chronic inflammatory disease characterized by multifocal pain, fatigue, and cognitive impairment [...]
The clinical features of late adult-onset autosomal dominant myoclonic epilepsy are similar to juvenile myoclonic epilepsy (JME), which is a common generalized epileptic syndrome with a significant hereditary component. But the age of onset, rare association of other seizure patterns, and non-relation of seizure onset to sleepiness suggest that this may be a distinct familial epileptic syndrome different from recognized familial myoclonic epilepsies.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.