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Query: UMLS:C0238111 (Lennox-Gastaut syndrome)
 861 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a group of rhesus monkeys (Macaca mulatta) inoculated intracerebrally and intravenously with a strain (Enage strain rhesus L6 56) of kuru already passaged in rhesus monkeys, 1 monkey presented the typical EEG pattern of epileptogenic encephalopathy reminiscent of the Lennox-Gastaut syndrome. This observation provides no direct evidence for the viral origin of epilepsies of this type. It does, however, show that it is possible to induce an epileptogenic encephalopathy by an unconventional infectious agent.
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PMID:Appearance of EEG changes reminiscent of a secondary generalized epilepsy in a rhesus monkey inoculated with a strain of kuru. 9 16

Cclinical and electroencephalographic aspects of twenty seven (27) patients with Lennox-Gastaut syndrome were studied (20 without previous West syndrome, group A, and 7 with this antecedent, group B). The epileptic seizures were characterized through descriptions made by relatives who were in close contact with the patients, and also by direct observation by the author in the clinic. The direct observation was possible due to high frequency rates of the seizures. Different clinical patterns were observed in patients who had and who had not previous history of West syndrome. The analysis of these differences permitted the identification of two groups of patients, although both of them had sharp and slow waves in the EEG. The clinical picture of each group was interpreted as the result of the stage of cerebral maturation at the time the diffuse epileptic encephalopathy occurred.
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PMID:[Comparison between the epileptic manifestation of the Lennox-Gastaut syndrome with and without previous West syndrome]. 82 82

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 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

The examination of cerebrospinal fluid has provided useful information for diagnosis of CNS infections. The progress of analytical technology has brought the possibility to detect very small amounts of chemical substances. I thought that new information from brain should be obtained by using modern analytical technology for several substances in CSF. Free amino acid pattern, glutamine, homocarnosine, glutamic acid decarboxylase (GAD), neuron specific enolase (NSE) and 2',5'-oligoadenylic acid synthetase (2-5 A) in CSF have been examined for information of brain injury and dysfunctions. The results are as follows. 1) The individual difference and constancy of free amino acid pattern in CSF were found in children without any neurological diseases. 2) The levels of free amino acids in CSF increased in the acute phase of bacterial meningitis. 3) High levels of glutamine in CSF of children with acute bacterial meningitis were normalized during the recovery phase. 4) A marked imbalance of free amino acids in CSF was found in children with Lennox-Gastaut syndrome. 5) A decrease of homocarnosine levels in CSF was related with the degree of unconsciousness in children suffering from neurological diseases. 6) High GAD activities in CSF were observed in the acute phase of aseptic meningitis and after intrathecal injection of methotrexate for the therapy against meningeal leukemia. 7) High NSE activities in CSF were found in the acute phase of bacterial meningitis, intracranial hemorrhage, encephalopathy and encephalitis. 8) High 2-5A activities CSF were measured in the acute phase of mumps meningitis with subsequent decreases during the recovery phase. These results suggest that several substances in CSF are useful as markers of brain injury and dysfunction.
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PMID:[Cerebrospinal fluid as informative source of the brain]. 201 95

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

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

Remarkable progress has been accomplished in epileptology in recent years. The remission rate in childhood epilepsy has been improved by the introduction of new technologies and better diagnostic as well as therapeutic methods. Investigation of prognosis is an important field of epileptology, because prognostic improvement reflects its overall progress. A long-term follow-up study of childhood epilepsy was undertaken in the Okayama University Hospital. Ten to 15 years of follow-up was possible in 730 of 1,295 patients who were first diagnosed at ages below 15 years, from 1968 to 1971. The 3-year remission rate amounted to 82.0% and 5-year remission was obtained in 79.1%. These high rates of remission indicate the favorable prognosis of childhood epilepsy. On the other hand, cases of intractable epilepsy also amounted to a considerable number. Intractable epilepsy consisted mainly of age-dependent epileptic encephalopathy (Ohtahara's syndrome, West syndrome and Lennox-Gastaut syndrome) and severe myoclonic epilepsy in infancy. Development of effective therapy for these intractable epileptic syndromes will be an important subject of future studies.
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PMID:Clinical course and prognosis of childhood epilepsy. 250 21

Therapy with synthetic ACTH (zinc tetracosactide) in children affected by epileptic encephalopathy is often associated with a large number of infectious complications. We studied the phagocytic activity of polymorphonuclear leucocytes (PMN) in 9 children with West or Lennox-Gastaut syndrome, measuring PMN superoxide anion production during the phagocytosis of particles of Zymosan and after phorbol myristate acetate (PMA) stimulation. The test was performed before, during and after therapy with zinc tetracosactide (0.02 mg/kg/day for 15 days). At the same time plasma immunoglobulins, C3, C4, C3 activator and cortisol were determined. During treatment PMN phagocytic function was significantly reduced but returned to normal levels after suspension of therapy. The other hematological parameters considered remained within the normal range. During the follow-up of the patients we observed 15 infectious episodes (3 mucocutaneous candidiasis, 2 enterocolitis, 4 urinary tract infections, 1 otitis media, 3 bronchiolitis, 2 pneumonia). One of the patients died of a bilateral pneumonia. Three children were treated with ACTH on alternating days. In these patients PMN phagocytic activity was less impaired and 2 infectious episodes rapidly resolved. Alternate day ACTH therapy seems to be preferable.
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PMID:Impairment of polymorphonuclear leucocyte function during therapy with synthetic ACTH in children affected by epileptic encephalopathies. 300 25

Key manifestations helpful in diagnosing Rett syndrome include progressive loss of previously acquired psychomotor skills, apraxia with loss of use of hands and legs, and "handwashing" automatisms. Four types of clinical presentation can be described: a neurodegenerative disorder, an autistic syndrome, a Lennox-Gastaut syndrome, and a chronic encephalopathy. Carbamazepine currently appears to be the anticonvulsant of choice. The mild lactic and pyruvic acidosis along with the ultrastructural abnormalities of mitochondria in brain and liver biopsies point to a generalized disorder of energy metabolism.
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PMID:Clinical recognition of Rett syndrome. 308 71


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