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

A clinico-electroencephalographic study on 14 cases of the early-infantile epileptic encephalopathy with suppression-burst (EIEE) including long-term follow-up studies for one year 8 months to 12 years 2 months disclosed the specificity of EIEE in its developmental aspects. With age, clinical evolution from EIEE to the West syndrome was observed in as many as 10 cases, among which two cases showed further transition to the Lennox-Gastaut syndrome. Electroencephalographically, suppression-burst pattern gradually began to disappear from age of 3 months and disappeared by 6 months in all the cases, transforming to hypsarhythmia in 10 cases from 2 to 6 months of age, showing further transition to diffuse slow spike-and-waves in 2 cases at one year and one month and at 3 years and one month of age, respectively. Changing pattern of EEG were classifiable into two types which strongly related to the prognosis. These findings indicated EIEE to be an independent epileptic syndrome as the earliest form of the age-dependent epileptic encephalopathy.
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PMID:The early-infantile epileptic encephalopathy with suppression-burst: developmental aspects. 343 12

Eleven infants with neonatal onset of intractable epilepsy are described, who showed the clinical and electroencephalographic features of Ohtahara syndrome. With time, transition to West and Lennox-Gastaut syndromes occurred. No cause could be found in eight cases. All nine survivors are severely mentally and physically handicapped and continue to have seizures. Early infantile epileptic encephalopathy represents the earliest of the age-dependent epileptic encephalopathies.
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PMID:Early infantile epileptic encephalopathy with suppression burst: Ohtahara syndrome. 367 31

We review the development of the classification of the epilepsies. Primary epilepsies are relatively benign, usually age-limited syndromes without clinical or radiologic evidence of brain lesions, and are related to a heritable constitutional predisposition to epilepsy. They usually respond well to antiepileptic drugs. The biochemical correlates of primary generalized epilepsy have been demonstrated in animals and man and have been related to diffuse cortical hyperexcitability, which has been linked to this disorder. The pathophysiology of the primary partial or focal epilepsies is poorly understood but does not appear to depend on focal brain lesions. We suggest that these are due to relatively localized areas of cortical hyperexcitability confined to isolated corticothalamic sectors and depend on interrelations between a constitutional cortical hyperexcitability and normal cortical maturation. The secondary epilepsies are associated with clinical and radiologic evidence of brain lesions and are often resistant to anticonvulsants. Secondary generalized epilepsies, with an associated diffuse encephalopathy, are typified by the Lennox-Gastaut syndrome. The presentation of the secondary partial epilepsies depends on the site of the lesion. The pathogenesis of epilepsy is multifactorial, and a preexisting constitutional predisposition can interact with an acquired diffuse or focal encephalopathy, facilitating the clinical expression of one or the other.
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PMID:Classification of the epilepsies. 405 19

Twelve patients with perinatal anoxic encephalopathy, 1 with encephalitis, and 1 with Down's syndrome had startle epilepsy. Hemiparesis, startle-induced seizures involving the hemiparetic side, focal electroencephalographic abnormalities, and unilateral lesions evident on computed tomographic scan were found in 6 patients, all of whom responded favorably to carbamazepine. The remaining 8 patients had severe intellectual impairment, bilateral motor deficits, generalized startle-induced seizures, diffuse and lasting electroencephalographic abnormalities, and widespread cerebral lesions evident on computed tomographic scan. Of these, 2 patients with Lennox-Gastaut syndrome responded to clonazepam, 4 others responded to valproic acid, and 2 others, with predominantly focal hemispheric lesions, improved on a regimen of carbamazepine.
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PMID:Startle epilepsy: a clinical study. 646 64

From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxysmal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or better neuropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatology). The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.
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PMID:Secondary late-onset Lennox-Gastaut syndrome: a critical view. 646 53

Two patients with childhood epileptic encephalopathy were studied by positron emission tomography before and after corpus callosotomy. Preoperatively, both patients showed in the temporal lobe unilateral hypometabolism that is characteristic of interictal epileptic foci. Postoperatively, the first patient had no seizures by the time of scanning, and his temporal lobe metabolism was bilaterally symmetric. Seizure control in the second patient did not improve by the time of scanning, and unilateral temporal hypometabolism persisted. This finding suggests a temporal lobe focus in two patients with Lennox-Gastaut syndrome.
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PMID:Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). 698 84

Electroclinical aspects of the West syndrome are studied in their atypical forms: clinical and EEG characteristics, partial forms, Aicardi syndrome. Etiological investigations (especially CT scan) are necessary to distinguish the more frequent 'secondary forms' from the 'primary forms'. The etiological factors are: (i) Prenatal causes (hereditary affections, chromosome-related etiology, fetal etiology; (ii) Perinatal causes (prematurity, traumatic delivery); (iii) Postnatal causes (infectious diseases). The evolutive aspects and prognosis are different for 'primary' and 'secondary' forms. The time of commencement of the mode of therapy and its duration are important factors in relation to the prognosis of 'primary forms'. A pre-existing encephalopathy obviously leads to a very poor prognosis. A typical Lennox-Gastaut syndrome can be observed in infants. However, atypical forms are more frequent. These too have a poor prognosis because they are usually secondary to cerebral lesions.
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PMID:[Epileptic evolutive encephalopathies in infants (West tsyndromee and Lennox-Gastaut syndrom) (author's transl)]. 734

Early infantile epileptic encephalopathy (EIEE) with burst-suppression (Ohtaharas syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalised epilepsy. We present 4 cases of EIEE fulfilling the classic diagnostic criteria, excluding the cases with segmentary and erratic myoclonic, characteristic features of Early Myoclonic Encephalopathy. All the patients were females. Pregnancy and delivery were normal and no other cases were observed in the families. One of the patients had a normal twin brother. Seizures began in all the cases before the 10th day of life. The initial EEG showed burst-suppression pattern, lasting for an average of 2.3 months of age (1.5-4 months). Seizures were initially resistant to therapy, although 2 cases showed partial response. None of the patients evolved to West syndrome. A case died at 5 months of age. No etiologic factors were found in two cases, but hemimegalencephaly was detected in the other two patients; in one of them seizures disappeared after corticectomy. We believe EIEE constitutes a type of epileptic encephalopaty which can be distinguished from other types of early onset epilepsy and should be included in the International Classification of Epilepsies with the West and Lennox-Gastaut syndromes.
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PMID:[Early infantile epileptic encephalopathy]. 749 79

The severe epilepsies of childhood are described briefly and information available on the efficacy of newly developed antiepileptic drugs (AEDs) in their control is reviewed. Therapeutic advances are awaited for early infantile epileptic encephalopathy, early myoclonic encephalopathy, progressive myoclonus epilepsies and Kojewnikow syndrome. West syndrome may respond to vigabatrin, and less predictably to lamotrigine. Lamotrigine can be helpful for severe myoclonic epilepsy and myoclonic absences. Astatic seizures may be dramatically controlled by lamotrigine, whereas vigabatrin may worsen myoclonic attacks. In the Lennox-Gastaut syndrome, the efficacy of felbamate has been demonstrated by a controlled trial; vigabatrin and lamotrigine can also be helpful. Non-idiopathic partial and secondary generalized epilepsies are responsive to vigabatrin in a useful percentage of cases, and some children improve with felbamate, lamotrigine or striripentol. A trial which compares the efficacies of the newer AEDs against each other could provide very useful information for the clinician.
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PMID:Management issues in severe childhood epilepsies. 758 57

One of the most challenging areas in nosology is in the field of severe generalized epilepsy of early childhood. This is certainly true in the case of Lennox-Gastaut syndrome (LGS), an age-related epileptogenic encephalopathy which comprises several types of generalized seizures including tonic seizures, atypical absence seizures and frequent status epilepticus. EEG shows generalized slow spike waves, and as the disease progresses, cognitive functions deteriorate. LGS is listed in the 1989 classification of the International League Against Epilepsy alongside epilepsy with myoclonic astatic seizures and West's syndrome. A number of variants or atypical forms have been proposed. As a result, differential diagnosis presents a major challenge and includes specific generalized epilepsies, i.e., metabolic or inflammatory; secondarily generalized epilepsies, i.e., those arising from the frontal lobe; and severe forms of idiopathic generalized epilepsy, i.e., Doose syndrome. Antiepileptic drug (AED) treatment of LGS has been disappointing. Results obtained from anterior callosotomy have been promising, but only a small number of patients have been evaluated. Although the syndrome is rare, the severe nature and intractability of LGS emphasizes the need for the development of specific AEDs which would completely modify the quality of life for these patients.
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PMID:The Lennox-Gastaut syndrome. 824 77


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