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

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

Combination therapy of high-dose pyridoxal phosphate (PAL-P, 40-50 mg/kg/day) and low-dose ACTH beta 1-24-Z (tetracosactide acetate-Zn, Cortrosyn Z, 0.01 mg/kg/day) was instituted in 26 children suffering from West syndrome and related disorders--pretreated without success with high-dose PAL-P alone; 18 with West syndrome (14 with symptomatic and 4 with cryptogenic types), 2 with symptomatic Lennox-Gastaut syndrome, 5 with cerebral palsy with hypsarhythmia or diffuse slow spike-waves and one with myoclonic seizures (secondary generalized epilepsy). Clinical, electroencephalographic and neurochemical investigations were carried out. The results were summarized as follows. 1) Only one of 27 children with West syndrome and related disorders pretreated using high-dose PAL-P alone before ACTH showed a clinically excellent response. 2) Clinical seizures were completely suppressed in 19 of 21 children who initially had seizures (90%) after this combination therapy. 3) Twenty-one of the total 26 children (80%) had disappearance of hypsarhythmia or diffuse slow spike-waves in EEG after this therapy. 4) During PAL-P treatment alone transient increases in liver enzymes occurred in 37 percent. The brain shrinkage of CT and the significant rise in CSF NSE were seen in 95% and 78% after ACTH, respectively. 5) Twenty-three children have been followed for one to 29 months after tapering off of ACTH. No relapses were experienced in 11 of 18 who initially had seizures (61%) and 13 of 23 with hypsarhythmia or diffuse slow spike-waves (57%). 6) Postictal PRL elevations were suppressed during high-dose PAL-P. 7) No significant changes in the CSF levels of HVA and 5-HIAA were seen during this combination therapy. The CSF levels of HVA were significantly lower than the controls. 8) Daily ACTH therapy transiently suppressed the secretion of anterior pituitary hormones (GH, TSH, PRL, LH and FSH) and thyroid hormones (T3 free T3, T4 and free T4). It is recommended that the combination therapy of high-dose PAL-P and low-dose ACTH is a promising new method and should be tried in children with West syndrome and related disorders. The mechanism of action of this combination therapy remains obscure although some information has been obtained from our investigations.
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PMID:Combination treatment of high-dose pyridoxal phosphate and low-dose ACTH in children with West syndrome and related disorders. 170 36

Corpus callosotomy is a well established procedure for the treatment of intractable epilepsy. However, this is the first clinical report of surgical division of the corpus callosum in Japan. Four patients with refractory seizures suffering from Lennox-Gastaut syndrome underwent anterior partial corpus callosotomy. Their seizures consisted of absences, tonic, atonic, tonic-clonic attacks and were characterized by frequent falls. Electroencephalograms showed paroxysms of bilateral synchrony of slow spike and wave complexes. Postoperative follow-up during 12-27 months showed that partial callosotomy reduced the frequency and severity of seizures in all the patients, although they still require antiepileptic medication. This procedure was effective even in patients with mixed cerebral dominance and also in a patient with low intelligence quotient. Postoperatively, disconnection syndrome developed in three patients, which was transient in one and lasting in two.
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PMID:Partial callosotomy for Lennox-Gastaut syndrome--first cases in Japan. 171 Mar 21

Seizures in patients with Lennox-Gastaut syndrome are very resistant to drug therapy. Division of the anterior half of the corpus callosum was performed in two patients with Lennox-Gastaut syndrome incapacitated by frequent atonic seizures leading to sudden falls. Postoperatively, the atonic seizures were completely eliminated and their mental reactivities remarkably improved. It is clear that they have benefited from a lower frequency of seizures and reduced dosages of anticonvulsant medication, which outweight the acquired disabilities.
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PMID:Partial corpus callosotomy beneficial for Lennox-Gastaut syndrome--report of two cases. 172 Feb 13

Band heterotopia, or "double cortex," is a neuronal migration disorder that consists of a symmetrical subcortical neuronal band. The overlying cortex may be normal or macrogyric. We describe two severely mentally retarded girls, aged 14 and 18 years, who had band heterotopia and Lennox-Gastaut syndrome. Band heterotopia was evident in both hemispheres as a subcortical symmetrical layer isointense with gray matter on magnetic resonance T1- and T2-weighted images. Both patients had atonic seizures, atypical absences, and tonic seizures. The electroencephalograms in both cases showed frequent generalized paroxysms and slow background activity. The association of a Lennox-Gastaut syndrome with double cortex in these two patients and in a previously reported autopsy-confirmed case suggests that this malformation may be responsible for other similar cases.
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PMID:Double cortex. A neuronal migration anomaly as a possible cause of Lennox-Gastaut syndrome. 172 65

The relationship between tuberous sclerosis (TS) and epilepsy on one hand and between agenesis of the corpus callosum (ACC) and epilepsy on the other has been recognized for a long time. Until now, a syndrome characterized by TS, ACC and Lennox-Gastaut syndrome (LGS) has not been described in the literature. Three cases of children, all young male patients, who suffer from TS, ACC and LGS, were presented. The ACC was total in two cases and partial in the third. None of the subjects previously had West syndrome. Two cases showed a slight degree of mental deficiency, while in the third it was very pronounced. In all subjects tonic and astatic seizures were frequent. Antiepileptic therapy improved the clinical picture in one case, while in the other two it remained unchanged.
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PMID:Tuberous sclerosis, agenesis of the corpus callosum and Lennox-Gastaut syndrome: mere chance or a new syndrome? 173 24

The correlations between sleep and prolonged epileptic activity are discussed on the basis of the status classification of Gastaut (1983). Little information is available on the interrelation of sleep and the status of tonic-clonic seizures (grand mal status). Most important is the therapeutical management of these cases. Tonic seizures have been reported to occur in large numbers during NREM sleep in patients with Lennox-Gastaut syndrome. A status-like increase is possible. Tonic seizures occur almost exclusively during sleep. Myoclonic status epilepticus arising (a) in the course of primary generalized epilepsy and (b) in the course of encephalopathies, are usually markedly attenuated during sleep. In absence status (petit mal status) synchronized sleep generally fragments the continuous discharge which is replaced by isolated bursts of polyspikes, or polyspike and wave complexes. The absence status can recur upon awaking during the night or in the morning. The abnormal EEG activity of a petit mal status can, however, occasionally persist during the whole night. Improvement as well as activation during sleep have been observed in elementary (= simple) partial status epilepticus; improvement seems to be more frequent. Epilepsia partialis continua may persist or decrease during sleep. An increase as well as decrease of motor phenomena has been observed during the REM stages. 'Epileptic aphasia' of childhood is associated with subclinical bioelectric status epilepticus during sleep. The electrical status epilepticus must be delineated as a separate group. The term encephalopathy related to electrical status epilepticus during slow sleep (ESES) has been proposed on the basis of associated psychic syndromes. This form of status epilepticus disappears during the waking state and during REM sleep. Cases with hypsarrhythmia without clinical signs may also be classified under the group of electrical or bioelectrical status. In some cases, a continuous hypsarrhythmia is observed only during sleep. In this context, one must also mention those patients who demonstrate continuous activation of spikes, or spike and wave potentials (without clinical seizures) during eye closure.
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PMID:Sleep and prolonged epileptic activity (status epilepticus). 176 86


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