Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0238111 (Lennox-Gastaut syndrome)
 861 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between January 1992 and June 1995, 160 patients were presurgically evaluated for medically refractory epilepsy by the Epilepsy Monitoring and Surgery Team at the University Hospital of Gent. All these patients underwent a comprehensive presurgical evaluation, including extensive neurological history and examination, video-EEG monitoring of interictal EEG and habitual seizures, CT and optimum MR. In a large subgroup of these patients a comprehensive neuro-psychological examination and interictal 18FDG-PET were performed. After the non-invasive phase of the presurgical evaluation, a bilateral carotid angiography and intracarotid amytal procedure was planned in 27 patients to establish hemispheric language dominance and bilateral memory function. After proper selection, 14 patients underwent invasive video-EEG monitoring with intracranial implantation of parenchymal and/or subdural electrodes to further document the area of seizure onset. From the initial group of 160 potential surgical candidates, 40 patients (20 M, 20 F) with mean age of 31 years (range: 2 months-55 years) and mean duration of uncontrolled seizures of 16 years (range: 2 months-47 years) eventually underwent a surgical procedure. 30/40 patients were on high dose antiepileptic polytherapy. Optimum MR detected structural abnormalities, confined to a limited brain area, in 39 patients. These abnormalities were of space-occupying nature in 21 cases; an atrophic lesion was suspected in 17 patients. Structural abnormalities were most frequently located in the temporal lobe (n = 26) and the frontal lobe (n = 7). Video-EEG monitoring documented complex partial seizures in 32 patients with occasional secondary generalisation in 14. In most of these patients, seizures could be subclassified as being of temporal lobe origin based on clinical and EEG criteria. Two patients had only simple partial seizures. One patient with Sturge-Weber syndrome and a strictly unilateral angioma had hemiconvulsions. A mentally retarded patient with Lennox-Gastaut syndrome had different types of seizures. After non-invasive and invasive exploration, the area of seizure onset could be determined in all patients. Standard or modified temporal lobectomy +/- hippocampectomy were the most commonly performed procedures (n = 26). In 5 patients complete lesionectomies were performed for epileptogenic structural lesions in and outside the temporal lobe. In 2 patients only partial lesionectomies were possible; in 5 patients only biopsies could be performed. Anterior 2/3 callosotomy and hemispherectomy were each performed in one patient. Postsurgical seizure control, after average follow-up of 20 months (range: 6-40 months), was excellent in 27 patients who became seizure-free. In these patients antiepileptic therapy was tapered 2 years after surgery. An additional 4 patients continue to experience non-disabling simple partial seizures only. Patients in whom only biopsies or partial lesionectomies were performed have poor seizure control. Three patients died as a result of the intrinsic malignancy of their space-occupying lesion. Two patients who are seizure free experienced a moderate postoperative hemiparesis with subtotal recovery. Overall quality of life was substantially improved both in patients who became entirely seizure free or who experienced a very significant reduction in seizure frequency. Presurgical evaluation and epilepsy surgery are a labour intensive but rewarding therapeutic alternative for patients with medically refractory epilepsy. Besides providing therapeutic efficacy, comprehensive presurgical evaluation and epilepsy surgery allow for fruitful clinical neurological research.
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PMID:Epilepsy surgery in Belgium, the Flemish experience. 866 30

Drug-resistant epilepsy, not associated with acute brain complications or central nervous system leukaemic involvement, can develop in patients treated for acute lymphocytic leukaemia during childhood. It has been postulated that this rare complication may be due to CNS oncological treatment neurotoxicity, related to intrathecal drugs, such as methotrexate, and brain radiotherapy. We report four patients who developed drug-resistant epilepsy sometime after receiving treatment for acute lymphocytic leukaemia. All patients were female and received intrathecal methotrexate. One received additional intrathecal cytarabine, and two concomitant brain radiotherapy. Two developed Lennox-Gastaut type syndrome, one multifocal epilepsy, and one focal epilepsy related to a radiotherapy-induced cavernous angioma. The development of drug-resistant epilepsy after treatment for acute lymphocytic leukaemia is a rare complication that may vary, from focal epilepsy to an epileptic encephalopathy. This may appear even years after the treatment has finished and is most likely associated with treatment-related neurotoxicity.
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PMID:Drug-resistant epilepsy after treatment for childhood acute lymphocytic leukaemia: from focal epilepsy to Lennox-Gastaut syndrome. 2793 21