Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Flunarizine (FLN) has been suggested as an add-on treatment in drug-resistant epilepsy patients. In view of the discordant experiences and of the paucity of controlled trials in children, we studied its effectiveness in 20 patients aged 6 to 18 years (10 males and 10 females), affected by drug-resistant epilepsy. 14 had symptomatic generalized epilepsy (the Lennox-Gastaut syndrome in 10; other forms in 4); 3 had cryptogenic generalized epilepsy (the Lennox-Gastaut syndrome in 2; myoclonic absences epilepsy in 1); 3 had symptomatic partial epilepsy (temporal lobe epilepsy). 7 of them were withdrawn: only 1 because of side effects. An initial four-month baseline pretrial period was followed by two four-month periods of administration of FLN or a placebo, under double blind conditions, in a randomized sequence. Preexisting antiepileptic (AEDs) medication was maintained at a constant dose throughout the study. FLN was administered as drops in a single evening dose of 5 mg (patients less than 10 years) or 10 mg. (patients greater than 10 years). During the pretrial phase, after phase 1 and phase 2, a waking EEG was recorded and blood samples were taken for hematology, hepatic-function tests, and AED serum levels. The evaluation of the activity of FLN was based on the total number of seizures. A 30-60% reduction in seizure frequency was found in 5 out of the 13 patients completing the trial (no changes occurred in the remainders). This result did not appear to be due to changes in the plasma levels of the AEDs. No significant differences were seen in the EEG paroxysmal activity in the three phases of the study. Side effects were rare. The serum FLN levels ranged between 16.4 and 109 ng/ml. It seems that the antiepileptic properties of FLN need further validation, particularly in childhood.
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PMID:Double-blind placebo-controlled trial of flunarizine as add-on therapy in refractory childhood epilepsy. 195 68

Among 62 children with myoclonic epilepsy who had first seizures between 1 and 10 years, without clinical or radiological evidence of brain lesion, we selected the 16 patients who had exhibited several types of fits and had stopped having seizures for over two years. First seizures occurred between 18 months and 4 years, and they were generalized clonic, tonic-clonic or tonic. After a mean 3 months' period, patients started also to have absence and myoclonic fits. During the period with various types of seizures, that lasted a mean 10 months, patients were ataxic and hyperkinetic, and 11 of them suffered myoclonic absence status for several hours or days. The EEG showed a high voltage rhythmic slow-wave activity with spikes, differing from the slow spike wave tracing of the Lennox-Gastaut syndrome, and there was no photosensitivity. The mean duration of the epilepsy was 1 year and 4 months and the last seizures were convulsive, occurring mainly during sleep. The clinical and EEG pattern, the high familial incidence are shared by the Doose syndrome, of which the present series seems to be a subgroup, as are other well-defined syndromes: benign and severe myoclonic epilepsies of infancy.
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PMID:["Benign" form of myoclonic epilepsy in children]. 211 71

Medium-chain triglycerides (MCT) are an important component of an enteral ketogenic diet for seizure control. Previously, it was difficult to maintain ketosis when parenteral (iv) nutrition therapy was necessary. The use of iv MCT in a 5-year-old girl with Lennox-Gastaut syndrome who had diarrhea and dehydration is reported. Conventional 20% iv fat emulsion (long-chain triglycerides, LCT) and dextrose free hyperalimentation (HAL) in a 4:1 ketogenic ratio did not maintain adequate ketosis during bowel rest. Compassionate use of iv MCT (Clintec Nutrition) infused as a 70:30 MCT/LCT ratio plus HAL maintained moderate ketosis. Seizures were well controlled during the iv MCT regimen, which allowed normal daily functioning. Complications included abnormal liver function tests and severe iron deficiency anemia of unknown etiology. Serum triglyceride and cholesterol levels increased to 1717 mg/dl and 614 mg/dl, respectively, but decreased with a reduction of lipid infusion and use of an antihyperlipemic drug. Nutritional status was maintained. In this case, iv MCT proved to be a relatively safe and effective short-term method of continuing parenteral nutrition while maintaining ketosis for seizure control.
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PMID:Use of parenteral medium-chain triglyceride emulsion for maintaining seizure control in a 5-year-old girl with intractable diarrhea. 212 29

A long-term follow-up study of 89 patients of Lennox-Gastaut syndrome (LGS) disclosed the persistent occurrence of seizures in 68 patients (76.4%) and severe mental defect in 48 (53.9%). An analysis of the correlation between the mental and seizure prognoses confirmed that the persistence of minor seizures could result in mental deterioration. An examination of the evolutional changes in EEG demonstrated that diffuse slow spike-waves characteristic of LGS gradually disappeared, while focal epileptic discharges, especially multifocal spikes, appeared in spite of the persistence of minor seizures. The diagnostic criteria were satisfied in only 31 (47.0%) of 66 patients with the persistence of minor seizures. Patients with multiple independent spike foci and minor seizures were considered to belong to a specific type of epilepsy, namely the severe epilepsy with multiple independent spike foci (the severe epilepsy with MISF). The seizure and mental prognoses were poorer in patients who evolved into the severe epilepsy with MISF than others.
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PMID:Long-term prognosis of the Lennox-Gastaut syndrome. 212 84

The Lennox-Gastaut syndrome (LGS), although described earlier, was first accepted in 1966 as a form of severe childhood epileptic encephalopathy, characterized by refractory epileptic seizures of diverse types, typical EEG abnormalities and slow mental development. This condition, however, is surrounded by controversy as the clinical criteria used by various authors to define it are quite different. It is very likely that this eponym has been used to harbour distinct epileptic conditions, all of which have in common a slow spike and wave complex in their EEG recording. Despite the fact that one of the hallmarks of LGS is an onset in early childhood, to add to the confusion, some cases of a LGS-like condition with onset in adulthood have been described. We report here one such case: a 28 year old man whose LGS-like condition started after severe head trauma at the age of 20. In addition we briefly review the difficulties in making this diagnosis, using this case to illustrate these aspects.
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PMID:[Lennox-Gastaut syndrome of late onset? Report of a case]. 212 96

Clinical and electroencephalographic studies were performed to elucidate the characteristics of epilepsy in severely handicapped children. The subjects were 56 severely mentally and physically handicapped children whose ictal seizures were documented by simultaneous EEG-VTR monitoring. Seizure types were infantile spasms in 17 (30.4%), atypical absence in 5 (8.9%), myoclonic seizures in 3 (5.4%), generalized tonic-clonic seizures in 2 (3.6%), secondary generalized partial seizures in 11 (19.6%), and undetermined in 4 (7.1%). Epilepsy types were also classified by the ictal seizure types and clinical courses: West syndrome in 27 (48.1%), Lennox-Gastaut syndrome (LGS) (at onset) in 5 (8.9%), partial epilepsy in 13 (23.2%) and others (at onset) in 11 (19.8%). Among 29 cases with West syndrome, 22 (81%) developed LGS, and among 11 cases with others group, 64% developed LGS. After all, LGS appeared in 60.7% of all 56 severe epileptic children associated with severe physical and mental handicaps.
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PMID:[Epilepsy in severely handicapped children]. 213 74

The antiepileptic effect of intravenous immunoglobulin (Sandoglobulin, Sandoz) was investigated in Lennox-Gastaut syndrome by an add-on, placebo-controlled, single-blind trial. Ten patients, aged 4-14 years, with insufficient response to conventional anticonvulsive therapy received placebo and Sandoglobulin 400 mg/kg two times each with an interval of two weeks. The washout period was four weeks and the total observation period 14 weeks, during which parents daily registered number and type of seizures. EEG, in vitro lymphocyte transformation tests and concentrations of immunoglobulins including IgG subclasses were evaluated before and after active treatment. Two children showed an immediate reduction in their high-frequency and invariable seizure activity from 42% to 100% and a less abnormal EEG. In addition, general well-being and intellectual performance was improved. The strongest response was observed in one child with a concomitant finding of a low level of IgG2, the only abnormal immunologic test in this study. The remaining 8 children, who had either a high or a low but variable seizure frequency showed no immediate change as EEG and their general condition was unaffected. We conclude that intravenous immunoglobulin had an immediate and pronounced effect on break-through seizure activity and a simultaneous neurophysiologic effect in 20% of our patients with Lennox-Gastaut syndrome. The effect was not confined to patients with immunologic abnormalities.
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PMID:Intravenous immunoglobulin: a single-blind trial in children with Lennox-Gastaut syndrome. 219 42

Below is report on our experience using a relatively new benzodiazepine as treatment for refractory epilepsy in 30 children. All of the patients had a severely fixed encephalopathy; 17 presented mixed epilepsy, 9 had Lennox-Gastaut syndrome and 4 others, West Syndrome. The seizures were totally controlled in 20% of the children; in a proportion superior to 75%, seizures were decreased in 46% of the patients. There were positive results in 6% of the cases and side-effects were seen in 13%. The usefulness of the clobazam as a co-adjuvant medication in the control of refractory epileptic seizures was demonstrated since the number of seizures decreased from 15 to 3 per day.
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PMID:[Usefulness of clobazam in the treatment of refractory epilepsy in children]. 227 46

Triple bromide elixir was used as an adjunctive antiepileptic drug in 11 children whose seizure disorders were intractable to other antiepileptic therapy. The patients' ages ranged from 2 to 17 years. The seizure disorders treated included photosensitive epilepsy (one case), acquired epileptic aphasia (one case), Lennox-Gastaut syndrome (three cases), and symptomatic localization-related epilepsies (six cases). Two patients' seizures completely stopped with bromide therapy. Four patients had a significant and sustained improvement on bromide therapy, while three more had a transient improvement. In these six patients with complete or significant control, the mean therapeutic dose was 33 mg bromide/kg daily, and the mean therapeutic serum concentration was 14.1 mmol/L (range, 4 to 30.5 mmol/L). The combination of bromide with valproate appeared to be particularly effective in these patients. Toxicity was minimal, and in only one patient was the medication stopped, because of anorexia and weight loss. Given the low cost, long half-life, and minimal toxicity when serum bromide concentrations are followed, bromide therapy should be considered as adjunctive antiepileptic drug therapy for patients whose seizures are intractable to other drugs.
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PMID:Bromide therapy for pediatric seizure disorder intractable to other antiepileptic drugs. 229 42

Cognitive functions of patients showing slow spike-wave discharge in EEG without obvious absence seizures were investigated using tachistoscopic stimuli triggered by spike-waves. Response times obtained during spike-waves and during non-spike-waves were compared for three tests (tapping, simple reaction, and morphological discrimination). We present one case of Lennox-Gastaut syndrome in which the test results could be statistically confirmed. The tapping test was interrupted by the occurrence of spike-waves. Response times during spike-waves were more delayed than during non-spike-waves in both the simple reaction tests and morphological discrimination tests. The differences in response times measured during spike-waves and during non-spike-waves were significantly greater in the morphological discrimination test than in the simple reaction test. The positive correlation between the length of spike-waves and response time in the discrimination test was significantly more pronounced than in the reaction test. These results were also confirmed in two other patients with Lennox-Gastaut syndrome. We conclude that spike-wave discharges may impair cognitive processing more intrinsically than motor functions.
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PMID:Visual cognitive disturbance during spike-wave discharges. 230 12


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