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)

Twenty-four medically refractory seizure patients, who did not qualify for excisional surgery, had anterior two-thirds corpus callosum section. Three to 11 years' postoperative follow-up suggests that this procedure can (1) lateralize a frontal lobe focus, which may lead to subsequent localized excision and (2) significantly reduce seizure frequency and severity in 75% of the patients without giving any permanent neurologic deficits. Patients with an ictal focus confined to one frontal lobe did best (8/8 improved), followed by patients with secondarily generalized seizures and multifocal bilateral foci (5/6 improved). Patients with mental retardation benefited less frequently (5/10 improved), but 4/4 from this group with ictal falls associated with Lennox-Gastaut syndrome did benefit. In this series, the improvements following the anterior partial section were lasting if present at 1 year of follow-up. Anterior corpus callosum section should be considered as a diagnostic (lateralizing) and therapeutic option in appropriately defined medically refractory patients who do not qualify for excisional surgery.
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PMID:Results of anterior corpus callosum section in 24 patients with medically intractable seizures. 339 67

Fifteen (12.7%) among 118 cases of the West syndrome were effectively treated by high-dose pyridoxal phosphate (PAL-P). 1) Clinical seizures were completely suppressed in 12 cases with PAL-P alone, and in 3 cases by an addition of PAL-P to the previously poorly-effective regimen. At the follow-up, 12 cases have continued to be free from seizures, while two cases relapsed into the Lennox-Gastaut syndrome, and one died. 2) Electroencephalographically hypsarhythmia disappeared by PAL-P in all 15 effective cases. 3) Effective daily dose of PAL-P was 30 to 400 mg. 4) Notably, PAL-P was effective even in the cases with obvious organic brain pathology, such as tuberous sclerosis, porencephaly, holoprosencephaly, postmeningitis, besides 5 idiopathic cases. 5) Efficacy of PAL-P was significantly higher in idiopathic cases than symptomatic cases; 35.7% vs 9.6%. 6) Response to PAL-P was not predictable by any laboratory data nor clinical features. 7) Prognosis of PAL-P responsive cases was favorable; as many as 6 cases developed normally among 14 cases followed-up. Treatment with a high-dose PAL-P should be tried in all cases of the West syndrome at first.
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PMID:Treatment of the West syndrome with high-dose pyridoxal phosphate. 343 17

Nine children with Lennox-Gastaut syndrome, aged 2-14 years, were studied by overnight polygraphy for one night. Percentage of sleep period time (SPT) for stage rapid eye movement (REM) and REM density during REM sleep decreased in Lennox-Gastaut syndrome as compared with control. Alpha rhythm was seen in only 3 cases and sleep spindles in only 6 cases. The effect of sleep-wake or REM-non-REM (NREM) sleep rhythm on the rate of generalized epileptiform discharges varied with the individual. Ictal discharges with or without clinical tonic seizures observed in 5 children appeared during NREM sleep and awakening in the morning, and in 2 of these cases they also occurred frequently during the NREM sleep of the first sleep cycle. Subclinical ictal discharges were also seen during REM sleep in the early morning in one case who was 2 years old. The Lennox-Gastaut syndrome is assumed to involved a considerable degree of brain stem dysfunction.
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PMID:Overnight polygraphic study of Lennox-Gastaut syndrome. 344 24

We measured local cerebral glucose utilization in 19 patients with Lennox-Gastaut syndrome (LG), partial seizures (PS), atypical and classical phenylketonuria (PKU), Leigh disease, and subacute sclerosing panencephalitis (SSPE), using positron emission tomography (PET). The mean values of regional glucose utilization in interictal scans of LG were significantly reduced in all brain regions when compared with that of PS (P less than 0.005). PET studies of glucose utilization in LG revealed more widespread hypometabolism than in PS. Two siblings with dihydropteridine reductase deficiency, a patient with classical PKU, and a boy with cytochrome c oxidase deficiency showed reduced glucose utilization in the caudate and putamen. A marked decrease in glucose utilization was found in the cortical gray matter of a patient with rapidly progressive SSPE, despite relatively preserved utilization in the caudate and putamen. The PET study of a patient with slowly progressive SSPE revealed patterns and values of glucose utilization similar to those of the control. Thus, PET provided a useful clue toward understanding brain dysfunction in LG, PS, PKU, Leigh disease, and SSPE.
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PMID:Cerebral glucose utilization in pediatric neurological disorders determined by positron emission tomography. 349 26

The authors present two siblings suffering from Lennox-Gastaut syndrome. One of them also had the Dandy-Walker malformation. His seizures were difficult to control with anticonvulsant drugs, and somnolence and cerebellar ataxia easily occurred during administration of low dose anticonvulsants. On the other hand, his brother did not have this malformation, and his seizures were easily controlled. The relationship of seizure control to the Dandy-Walker malformation is discussed.
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PMID:Lennox-gastaut syndrome with and without Dandy-Walker malformation. 350 1

Forty-nine children and adolescents whose seizures reportedly worsened while receiving carbamazepine (CBZ) were studied retrospectively. Twenty-six patients met criteria for excellent documentation of carbamazepine-exacerbated seizures. Four epileptic syndromes were particularly affected: childhood absence epilepsy; focal symptomatic, frontal lobe epilepsy; Lennox-Gastaut syndrome; and severe myoclonic epilepsy of infancy. Eight of the 26 patients developed new-onset absence seizures and three patients with established absence epilepsy experienced absence status. Other seizure types, including atonic, tonic-clonic, and myoclonic, developed in eight patients treated with CBZ, and new generalized spike-and-wave discharges were observed in electroencephalograms of nine patients. CBZ is a widely used, effective antiepileptic drug, particularly for partial or partial complex seizures; however, if uncontrolled, generalized seizures occur after CBZ is prescribed for children or adolescents with absence or mixed seizures, a trial of CBZ discontinuation is warranted. The data reported here do not permit calculation of the incidence of this phenomenon.
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PMID:Carbamazepine-exacerbated epilepsy in children and adolescents. 350 8

We describe the failure of an intravenous benzodiazepine to control non-convulsive status epilepticus occurring in six patients with the Lennox-Gastaut syndrome. In one patient the benzodiazepine induced a paradoxical response with clinical and electroencephalographic seizures.
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PMID:Non-convulsive status epilepticus resistant to benzodiazepines. 354 98

Atonic seizures, atypical absence seizures, myoclonic seizures, tonic seizures, and infantile spasms are among the most difficult to control in children. Certain 1,4-benzodiazepines may be effective against these seizure types. Thus, clonazepam has been shown to reduce the frequency of absence, atypical absence, myoclonic, atonic, and complex partial seizures in children. Furthermore, both clonazepam and nitrazepam have been shown to be effective in the treatment of infantile spasms. However, their usefulness is limited by a deleterious effect on neurologic function, particularly on cognition. Controlled studies in animals and in adult patients and volunteers have demonstrated that clobazam has less neurotoxicity than 1,4-benzodiazepines and, in addition, may possess a psychotropic effect. Uncontrolled studies of clobazam in children suggest that this drug may be effective in the treatment of partial seizures, startle-induced seizures, infantile spasms, and Lennox-Gastaut syndrome. We studied the effect of clobazam in children whose seizures were resistant to most other antiepileptic drugs. Mental retardation was present in 80% of the children, and 62.5% had Lennox-Gastaut syndrome. Tolerance to clobazam developed in approximately one-third of patients, but this was frequently only partial and often responded to an increase in dosage. Of 50 children studied for a minimum of 3 months, seizures were controlled completely in 10 and frequency of seizures was reduced by more than 50% in a further 17. In most cases, parents observed a striking improvement in neurologic function, particularly in alertness, concentration, and balance. Thus, clobazam may be of value in the treatment of those seizures which are most difficult to control in children.
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PMID:Benzodiazepines in the treatment of children with epilepsy. 374 24

Serial polysomnograms were performed on 11 children with primary Lennox-Gastaut syndrome (LGS), 6 control children with other seizure disorders, and 12 who were developmentally normal. Five LGS children had abnormal polysomnograms with either complete absence or marked reduction of REM sleep; the other six LGS children had only a mild reduction of REM sleep. The percentage of REM in LGS children was less than in the controls with other seizure disorders (p less than 0.05) or the normal children (p less than 0.005). The scatter of REM percentages in LGS may imply heterogeneity of the syndrome, perhaps related to the severity of brainstem dysfunction or neurochemical derangement.
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PMID:Sleep patterns in the Lennox-Gastaut syndrome. 374 89

The authors have followed six children with atypical epilepsies but a favorable evolution, consisting in minor motor seizures of the myoclonic-astatic type (with diffuse slow spike-waves on the electroencephalogram) together with clinical and EEG features seen in benign focal epilepsy of childhood (BFEC), an association recently reported by Aicardi and Chevrie (1982). The maintenance of a normal neurological function despite severe epilepsy, the absence of tonic seizures and the marked activation of the spike discharges during sleep were described by these authors as important characteristics suggesting a good prognosis. The purpose of this study was to see if these children indeed represent a particular subgroup of idiopathic epilepsy and to draw attention to a special clinical and EEG combination indicating a possibly favorable ultimate outcome, in children usually diagnosed as suffering from Lennox-Gastaut syndrome. The six cases closely resembled the group described by Aicardi and Chevrie (1982), although the clinical and EEG features of BFEC were not as striking as in their cases. Also transient mental deterioration occurred during the active seizure periods. The therapeutic benefit of the various drugs tried was difficult to assess, but the behavior was often perturbed by medication. Although it is not possible to decide at the present time if these cases represent a particular epileptic syndrome, the special combination of clinical and EEG features seems characteristic enough to justify prospective studies of similar cases in the future.
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PMID:Combined myoclonic-astatic and "benign" focal epilepsy of childhood ("atypical benign partial epilepsy of childhood"). A separate syndrome? 376 71


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