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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Lennox-Gastaut syndrome (LGS) is a severe epileptic disorder, characterized by developmental delay, typical electroencephalographic pattern and poor control of seizures. This epileptic syndrome generally presents in early childhood but exceptionally starts at the age of 5 years or later. We describe a patient who was classified as late-onset LGS, with evidence of mild hydrocephalus on CT. MRI added new diagnostic information by demonstrating laminar heterotopic grey matter.
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PMID:Laminar heterotopic grey matter (double cortex) in a patient with late onset Lennox-Gastaut syndrome. 144 56

We report eight sporadic cases of typical Angelman syndrome (AS) associated with chromosome 15q12 deletion. Age at first visit was 3-35 months (average 18 months), and follow-up period was 4-20 years (average 14.1 years). The characteristic features of epilepsy in AS are (a) seizure onset in early childhood (8 of 8); (b) evolution of seizure type with age (8 of 8); (c) EEG abnormality changes from high-voltage slow bursts (HVS) in infancy to diffuse spike and waves in middle childhood (4 of 5); (d) atypical absence seizures (8 of 8), often occurring as atypical absence status (4 of 8); and (e) diminution of seizure discharges and clinical seizures after puberty (7 of 7). We believe that AS may frequently exist in the intractable epilepsies of childhood with severe mental retardation. We stress the importance of AS as one of the main etiologic background diseases of the intractable epilepsies with infantile onset such as West syndrome, Lennox-Gastaut syndrome, and others.
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PMID:Epilepsy in Angelman syndrome associated with chromosome 15q deletion. 146 68

We report our experience with lamotrigine add-on therapy in the treatment of 11 patients with Lennox-Gastaut syndrome. Lamotrigine is a novel antiepileptic drug, chemically unrelated to the major anticonvulsants in current use. Ten patients experienced a > 50% reduction in seizure frequency, 1 patient experienced no change in seizure frequency. All patients tolerated lamotrigine satisfactorily and no side-effects were reported.
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PMID:Lamotrigine as an add-on drug in the management of Lennox-Gastaut syndrome. 149 Apr 94

The most severe epilepsies that affect neonates, infants, and children include Ohtahara, West, and Lennox-Gastaut syndromes. These three syndromes display considerable similarities and transitional features in their clinical symptoms, seizure phenomena, and electroencephalographic abnormalities. This review examines the similarities and differences between these three syndromes and the other severe epilepsies of infancy and childhood, and discusses the hypothesis that the three disorders form a continuum of epileptic encephalopathies that have a predictable age-related evolution.
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PMID:The age-dependent epileptic encephalopathies. 155 55

Triphasic waves are usually thought of as indicating a metabolic encephalopathy. Recent investigations have added nonmetabolic etiologies to the differential diagnosis of triphasic waves. Seizures are not generally thought of as associated with triphasic waves. Similarities in the appearance of records with encephalopathies and continuous triphasic waves and those of some patients with the Lennox-Gastaut syndrome have been noted. We presented a case which suggests that the presence of TW in a patient with a metabolic encephalopathy might suggest petit mal status epilepticus.
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PMID:Triphasic waves and spike wave stupor. 158 47

The epileptic encephalopathies of infancy and childhood include West syndrome, Lennox-Gastaut syndrome, severe myoclonic epilepsy of early childhood and related syndromes. The exact limits of these syndromes are still a subject of debate. The clinical features include several types of brief, frequently repeated seizures associated with mental retardation. The causes are multiple and brain malformations and dysplasias play an important role. Treatment remains unsatisfactory and the place of surgery seems limited.
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PMID:Epileptic encephalopathies of early childhood. 162 62

Two children, 13 and 14 years old, presented an intractable epilepsy of Lennox-Gastaut. In front of dangerous tonic and atonic epileptic crisis, a corpus callosotomy was performed. Corpus callosotomy is becoming a more widely used procedure in the treatment of intractable epilepsy as Lennox-Gastaut syndrome, or frontal epilepsy. However, there have been very few series that have reported results in children. It is not a complete but a partial, callosal section including approximately the anterior two thirds of the callosum. After this surgery the two children improved dramatically because the dangerous tonic and atonic crisis disappeared. Secondly we observed improvement of pre-surgical psychiatric troubles, that is a data not developed in the literature. Before surgery, the two children had a frontal syndrome with hyperkinesia, distractibility, aggressiveness, alexithymia, loss of the program of ideas. During the two months after the section of the anterior two thirds of the corpus callosum, we observed a progressive improvement of the frontal syndrome, with possibility to learn new praxies. The intellectual quotient was not altered and associative functions, depending of the posterior third of the corpus callosum were spared. Anti-epileptic medications were not stopped. We think that the improvement of the frontal syndrome is due to reduction of seizures. Therefore, we insist on the interest of the section of the anterior two thirds of the corpus callosum as treatment of tonic and atonic seizures but also as treatment of psychiatric symptoms depending of a frontal syndrome.
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PMID:[Effects of callosotomy in the treatment of intractable epilepsies in children on psychiatric disorders]. 163 4

Induced microseizures (IMS) were observed in a 5-month-old girl with symptomatic West syndrome. The seizures occurred following the suppression of infantile spasms with adrenocorticotropic hormone therapy and disappeared following the cessation of clonazepam administration. The ictal manifestations consisted of periods of irregular respiration, and respiratory arrest lasting for several seconds which often involved opening of the eyes and mild extension of the neck corresponding with the diffuse fast wave bursts in EEG activity observed during sleep. These seizures were thought to be equivalent to the IMS in Lennox-Gastaut syndrome, which have never been reported before in patients with West syndrome.
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PMID:Induced microseizures in West syndrome. 165 8

A 10-year-old girl with developmental delay, mental retardation and intractable seizures is reported. She manifested clinically as Lennox-Gastaut syndrome with unknown etiology before admission. Brain MRI revealed a band-like lesion over the bilateral subcortical region being later recognized as band-form heterotopia. This type of heterotopia may be overlooked easily due to its symmetrical distribution and mild degree of cortical convolutional anomaly. As it is often associated with intractable seizures and psychomotor retardation, the prognosis is poor. This patient responded poor to ketogenic diet and anticonvulsants. We present this case and review the related articles.
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PMID:[Lennox-Gastaut syndrome with band form heterotopia: a case report]. 165 45

The generalized repetitive fast discharge (GRFD) is an ictal pattern representing the EEG counterpart of tonic seizures occurring mainly in Lennox-Gastaut syndrome (LGS) during slow-wave sleep. The history of terminology, electromorphology, correlations with sleep, ictal clinical correlations and associations with different epileptic syndromes as well as the clinical significance of the pattern is described reviewing the pertinent literature and our own experiences. The physiopathogenesis from both the electrophysiological and pharmacological aspects is discussed in the framework of a concept according to which GRFD is considered as a malignant derivative of an existing slow spike-wave mechanism, due to the permanent or momentary breakdown of the GABA-ergic inhibitory process. In observations performed on some patients we found a paradoxical GRFD-eliciting effect of BDZ drugs and hexobarbiturate after chronic treatment with BDZ agents and/or barbiturate, and a GRFD-blocking effect of Anexate (Flumazenil), a BDZ antagonist on the pattern, appearing either spontaneously in slow-wave sleep or elicited by diazepam or barbiturate. Our findings support the assumption that BDZ (Barbiturate) GABA-Chloride Ionophore Complex plays an important role, both in the development of and possibly in the therapeutic approach to, the GRFD phenomena. Some hypotheses about the role played by the complex based on these observations are put forward.
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PMID:Runs of rapid spikes in sleep: a characteristic EEG expression of generalized malignant epileptic encephalopathies. A conceptual review with new pharmacological data. 166 48


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