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

Several epileptic syndromes that occur during childhood are characterized by severe treatment-resistant seizures, progressive loss of higher intellectual functions, and characteristic electroencephalographic abnormalities. These catastrophic epileptic syndromes include epileptic encephalopathy with diffuse slow spike waves (Lennox-Gastaut syndrome), West syndrome, progressive myoclonic epilepsies, and electrical status epilepticus during sleep. This article summarizes each syndrome and reviews the most recent information concerning the effectiveness of topiramate with respect to each condition. Suggestions are offered to help clinicians maximize topiramate's efficacy and tolerability in patients suffering with these syndromes. Overall, topiramate is a valuable antiepileptic medication in the treatment of catastrophic pediatric epileptic syndromes.
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PMID:Topiramate in the catastrophic epilepsies of childhood. 1121 52

The diagnosis of Angelman syndrome (AS) can be confirmed by genetic laboratory in about 80% of cases. In 20%, the diagnosis remains clinical, but often there is uncertainty about the correctness of the clinical diagnosis and alternative diagnoses may be investigated. In evaluating individuals for AS in our center since 1989, we have encountered several mimicking conditions, and additional ones have been reported in the literature. Mimicking conditions can be grouped into the areas of chromosome, single gene, and symptom complex anomalies. Microdeletions or microduplications include chromosome regions 2,4,17, 22, and 15. Single gene conditions include methylene tetrahydrofolate reductase deficiency (MTHFR), Rett syndrome, alpha-thalassemia retardation syndrome (ATR-X), and Gurrieri syndrome. Symptom complexes include cerebral palsy, static encephalopathy, Lennox-Gastaut syndrome, autism spectrum disorder, pervasive developmental delay (PDD), and mitochondrial disorders. We present a review of these mimicking disorders to increase the awareness about conditions that can lead to an incorrect clinical diagnosis of AS.
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PMID:Angelman syndrome: mimicking conditions and phenotypes. 1175 74

The subjects were 25 children, including West syndrome, Lennox-Gastaut syndrome, childhood absence epilepsy (CAE) and localization-related epilepsies. Clinical seizures occurred only during waking state in 14 patients (including the cases of West syndrome, CAE and epilepsy with myoclonic absences (EMA)), only during sleeping state (especially during non-REM sleep in five patients, including the case of benign epilepsy with centrotemporal spike), and diffusely during waking and sleeping states in six patients (including the case of early infantile epileptic encephalopathy with suppression burst). These three types were observed in the cases of Lennox-Gastaut syndrome and localization-related epilepsies. Subclinical ictal discharges occurred during REM sleep in West syndrome, and during REM and non-REM sleep especially during non-REM sleep in CAE and EMA.
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PMID:Epileptic seizures and sleep-wake rhythm. 1142 28

A long-term follow-up study of Lennox-Gastaut syndrome (LGS) ( > 10 years) was conducted with 38 patients with severe motor and intellectual disabilities (SMID) to clarify the relationship between the rapid development of dysphagia and epileptic seizures, and to elucidate the long-term evolution of LGS in patients with SMID. Those who showed a relatively favourable seizure outcome were compared to those with a poor seizure outcome. Poor seizure outcome correlated strongly with: (a) an early appearance of dysphagia and additional deterioration of the already retarded mental function; (b) a predominance of atypical absence seizures; and (c) persistent frequent epileptiform discharges during electroencephalographic evaluations. Neither age at seizure onset nor intelligence level at the time of the last examination was correlated with seizure prognosis. Further, seizure prognosis was not related to the aetiology of LGS. Repeated seizures apparently caused development of progressive epileptic encephalopathy, in addition to the underlying severe brain damage. Since development of dysphagia burdens an already severely handicapped patient with intensive medical care, efforts to reduce the seizures and design a long-term care plan are of great importance.
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PMID:Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia. 1143 19

Epileptic encephalopathies are conditions in which neurologic deterioration results mainly from epileptic activity. It can be due to very frequent or severe seizures, or to subcontinuous paroxysmal interictal activity. The former consists mainly of severe myoclonic epilepsy in infancy (SMEN), in which patients exhibit seizures from the middle of the first year of life with repeated episodes of status epilepticus, and migrating partial epilepsy in infancy, in which, from the first trimester of life, partial seizures affect various areas of the cortex randomly and in a subcontinuous fashion. Cases with subcontinuous paroxysmal interictal activity affect newborns with suppression bursts, thus consisting of either Ohtahara syndrome or neonatal myoclonic encephalopathy, and infants with infantile spasms (IS), although rare cases do not start until age 4 years. In childhood, it consists of various types of generalized seizures combined with either slow spike-waves of the Lennox-Gastaut syndrome (LGS) or with myoclonus and 3-Hz spike-waves of myoclonic-astatic epilepsy, and continuous spike-waves in slow sleep (CSWS) combined with various neuropsychological patterns including Landau-Kleffner syndrome, frontal lobe syndrome, orofacial dyspraxia, or negative myoclonus. Management differs for all these syndromes, with the combination of clobazam (CLB) and stiripentol (STP) being promising for SMEN, vigabatrin (VGB) for IS, lamotrigine (LTG) for LGS, and steroids for CSWS. It is important to avoid potential drug-induced worsening by phenobarbital (PB), phenytoin (PHT), carbamazepine (CBZ), tiagabine (TGB), and VGB; in children and especially in infants, treatment with valproate is preferred each time the proper diagnosis is not reached.
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PMID:Epileptic encephalopathy. 1152 Mar 18

Long-term prognosis of epilepsy was investigated on 117 institutionalized patients with severe motor and intellectual disabilities, who were above 15 years of age in 1977, for a 20-year-period from 1977 to 1997. The incidence of epilepsy was 64.1% (75 patients), which was active in 28 patients (37.3%). The patients with the most severe psychomotor disabilities (bedridden and DQ < 20) showed the highest incidence of epilepsy (85.0%). Patients who died during the follow-up period showed higher incidence of active epilepsy (p < 0.01). During the follow-up of 94 surviving patients, persistence, relapse, and onset of seizures were frequent in patients with most severe intellectual disability, whereas those with less severe intellectual disability (20 < DQ < 35) were all seizure-free. Twenty-one patients had active epilepsy; symptomatic partial epilepsy in 17 (81.0%) and generalized epilepsy in 4 (19.0%). Notably, 5 of the 6 patients with persistent frequent seizures had age-dependent epileptic encephalopathy; persistent Lennox-Gastaut syndrome (LGS) (2 patients), severe epilepsy with multiple independent spike foci evolved from West syndrome (WS) and LGS (2 patients), and partial epilepsy with the history of LGS (1 patient).
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PMID:[Epilepsy in patients with severe motor and intellectual disabilities: a long-term follow-up]. 1155 44

Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.
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PMID:Spasms in clusters in epilepsies other than typical West syndrome. 1170 Dec 41

Both West syndrome (WS) and Lennox-Gastaut syndrome (LGS) are associated with various developmental disorders and it has been discussed whether the cerebral cortex or subcortical structures are important in the pathogenesis of both epileptic syndromes. Here we briefly review the literature on the neuropathological findings in WS and LGS, and present our data on immunohistochemical analysis of the brainstem and limbic lesions in autopsy cases of lissencephaly and sequels of hypoxic ischemic encephalopathy (HIE) caused by perinatal asphyxia manifested as both WS and LGS (WS/LGS). Nowadays, the neuroradiological examinations and surgical pathology in WS cases demonstrate dysplastic cerebral lesions more frequently than previously expected. On the other hand, we have delineated the common brainstem lesions such as small size of the tegmentum and spongy state and/or gliosis in the central tegmental tract in a number of WS autopsy cases of various etiologies. Recently, we reported the reduced expression of tyrosine hydroxylase, methionine enkephalin and parvalbumin in the brainstem in autopsy cases of lissencephaly and sequels of HIE manifested as WS/LGS, regardless of the cerebral changes. In the same subjects, we examined the expression of glutamate transporters and calcium-binding proteins in the limbic system by immunohistochemistry. These represent markers of glutamate neurotoxicity and the GABAergic inhibitory neuron system, respectively. The altered expressions of glial glutamate transporters and calcium-binding proteins in the limbic system seemed to reflect temporal lobe sclerosis, irrespective of the past history of WS, and there were no differences in the limbic involvement between the cases manifested as WS/LGS and disease controls of sequels of HIE not manifested as WS/LGS. It is more likely that the brainstem lesions contribute to the pathogenesis of WS and/or LGS more than the heterogeneous limbic lesions in these cases.
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PMID:Neuropathology of the limbic system and brainstem in West syndrome. 1170 Dec 47

Children with infantile epileptic encephalopathies comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were retrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental status and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, long-term development and final seizure status were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic encephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent relapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, compared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic encephalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal development at onset of spasms. Microcephaly was associated with delayed development but did not influence final seizure outcome. Final seizure outcome was poor in children with delayed development at onset (adjusted odds ratio [OR]=4), delay in diagnosis >12 months (OR=2.27) and in children with Lennox-Gastaut syndrome (OR=4.75). ACTH/prednisolone and antiepileptic treatment versus antiepileptics alone showed a good final seizure response in 36.6% versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR=23.4) and abnormal electroencephalogram (OR=7.46). Perinatal factors constituted one third of symptomatic West syndrome. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy implications. Delay in diagnosis was common and negatively influenced final seizure outcome.
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PMID:West syndrome and other infantile epileptic encephalopathies--Indian hospital experience. 1189 Nov 7

Thyrotropin-releasing hormone (TRH) has been successfully used for treating children with neurologic disorders including epilepsy. The effectiveness of TRH and a TRH analog has been reported in West syndrome, Lennox-Gastaut syndrome, and early infantile epileptic encephalopathy that were intractable to anticonvulsants and adrenocorticotrophic hormone (ACTH). However, the peptide has not been widely studied as a treatment of intractable epilepsy outside Japan. TRH is safe in children and effective in some cases of West syndrome and Lennox-Gastaut syndrome. TRH is considered as a possible new strategy for treating West syndrome and Lennox-Gastaut syndrome prior to ACTH therapy, especially for the patient with an infection, immunosuppression, or severe organic lesions in the brain. The mechanisms of its antiepileptic action may differ from those of other antiepileptic drugs. One possibility is that TRH may act as an antiepileptic through a kynurenine mechanism, considering that kynurenic acid acts as an antagonist on the N-methyl-D-aspartate receptor complex.
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PMID:Thyrotropin-releasing hormone: role in the treatment of West syndrome and related epileptic encephalopathies. 1170 Dec 74


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