An atypical case of Lennox-Gastaut syndrome not associated with mental retardation: A nosological issue

Shyu, Hann-Yeh; Lin, Ji-Ho; Chen, Chien; Kwan, Shang‐Yeong; Yiu, Chun Hing

doi:10.1016/j.seizure.2011.08.001

Cited by 11 publications

(5 citation statements)

References 10 publications

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“…Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies with childhood onset. It is characterized by the presence of (i) multiple, untreatable seizure types, including tonic seizures, atypical absences and tonic or atonic drop attacks, and (ii) electroencephalographic (EEG) abnormalities, consisting of interictal pattern of slow-spike waves (SSW) < 3 Hz, and paroxysmal fast rhythms (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), mainly during non-REM sleep [1]. At the onset of the disorder, not all the different seizure types can be present [2].…”

Section: Definition Epidemiology and Etiologymentioning

confidence: 99%

“…In cryptogenic cases, the psychomotor development of the child is generally normal before the onset of the disease and then deteriorates. However, the early onset, the presence of tonic seizures, certain EEG patterns and the overuse of antiepileptic drugs (AEDs) are risk factors for ID; on the contrary, the late onset, the presence of subclinical seizures without falls would have a positive role on cognitive functions [11].…”

Section: Cognitive Impairment and Behavioural Disturbancesmentioning

confidence: 99%

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The pharmacological management of Lennox-Gastaut syndrome and critical literature review

Verrotti

Striano

Iapadre

et al. 2018

Seizure

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A B S T R A C TLennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with a prevalence of 1-2% of all patients with epilepsy. It is characterized by multiple pharmaco-resistant seizure types, including tonic, atypical absences and tonic or atonic drop attacks, and the presence of electroencephalographic abnormalities, such as slow-spike waves and paroxysmal fast rhythms. Intellectual disability, behavioural and psychiatric disorders are common comorbidities; these disturbances have a multi-factorial pathogenesis. The selection of the most appropriate drug must be tailored to each patient and guided by the prevalent seizure type. In this paper available pharmacological options are discussed and for each pharmacological agent, current evidence of efficacy and tolerability is provided. Valproic acid represents one of the first-line options in the treatment of LGS. Anyway, other antiepileptic drugs (AEDs) may be considered and added: lamotrigine, rufinamide, topiramate, clobazam can be efficacious. The use of felbamate must be carefully evaluated because of its adverse events. Perampanel, zonisamide, levetiracetam and fenfluramine have shown to be useful in the treatment of selected patients; nevertheless, the lack of RCTs does not allow to recommend their use in a systematic way. Recently, cannabidiol has provided high evidence of efficacy against LGS seizures; however, these data must be confirmed by long-term extensive studies and by trials comparing different AEDs, one to each other.

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Section: Definition Epidemiology and Etiologymentioning

confidence: 99%

Section: Cognitive Impairment and Behavioural Disturbancesmentioning

confidence: 99%

The pharmacological management of Lennox-Gastaut syndrome and critical literature review

Verrotti

Striano

Iapadre

et al. 2018

Seizure

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“…Cognitive impairment in LGS appears related to the age of onset and persistence of seizures. An earlier age of seizure onset (<5 years) has been associated with more severe cognitive impairment, while patients who develop LGS later in life (>9 years) follow a more favorable cognitive course ( 3 , 30 , 32 , 41 – 43 ). In a group of patients with normal mental development before the onset of LGS, Ohtsuka ( 44 ) found that 95.7% (22/23) of patients with persistent seizures showed cognitive impairment after a follow-up period of at least 5 years compared with 12.5% (1/8) of patients who had been seizure free for at least 1 year.…”

Section: Lennox–gastaut Syndrome – Definition and Clinical Featuresmentioning

confidence: 99%

Conceptualizing Lennoxâ€“Gastaut Syndrome as a Secondary Network Epilepsy

et al. 2014

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Lennox–Gastaut Syndrome (LGS) is a category of severe, disabling epilepsy, characterized by frequent, treatment-resistant seizures, and cognitive impairment. Electroencephalography (EEG) shows characteristic generalized epileptic activity that is similar in those with lesional, genetic, or unknown causes, suggesting a common underlying mechanism. The condition typically begins in young children, leaving many severely disabled with recurring seizures throughout their adult life. Scalp EEG of the tonic seizures of LGS is characterized by a diffuse high-voltage slow transient evolving into generalized low-voltage fast activity, likely reflecting sustained fast neuronal firing over a wide cortical area. The typical interictal discharges (runs of slow spike-and-wave and bursts of generalized paroxysmal fast activity) also have a “generalized” electrical field, suggesting widespread cortical involvement. Recent brain mapping studies have begun to reveal which cortical and subcortical regions are active during these “generalized” discharges. In this critical review, we examine findings from neuroimaging studies of LGS and place these in the context of the electrical and clinical features of the syndrome. We suggest that LGS can be conceptualized as “secondary network epilepsy,” where the epileptic activity is expressed through large-scale brain networks, particularly the attention and default-mode networks. Cortical lesions, when present, appear to chronically interact with these networks to produce network instability rather than triggering each individual epileptic discharge. LGS can be considered as “secondary” network epilepsy because the epileptic manifestations of the disorder reflect the networks being driven, rather than the specific initiating process. In this review, we begin with a summation of the clinical manifestations of LGS and what this has revealed about the underlying etiology of the condition. We then undertake a systematic review of the functional neuroimaging literature in LGS, which leads us to conclude that LGS can best be conceptualized as “secondary network epilepsy.”

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“…2,3,17 Up to 10 to 20% of patients with LGS reaches acceptable ranges of intellectual functioning despite variable limitations in daily activities and cases of lacking cognitive impairment have been recently reported. 2,28 Main risk factors for intellectual delay in LGS include earlier age of onset, a previous West syndrome, a symptomatic etiology, and episodes of nonconvulsive status epilepticus. 1,2,17,29…”

Section: Clinical Presentationmentioning

confidence: 99%

Lennox–Gastaut Syndrome: A State of the Art Review

Mastrangelo

2017

Neuropediatrics

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Lennox-Gastaut syndrome (LGS) is a severe age-dependent epileptic encephalopathy usually with onset between 1 and 8 years of age. Functional neuroimaging studies recently introduced the concept of Lennox-Gastaut as "secondary network epilepsy" resulting from dysfunctions of a complex system involving both cortical and subcortical structures (default-mode network, corticoreticular connections, and thalamus). These dysfunctions are produced by different disorders including hypoxic-ischemic encephalopathies, meningoencephalitis, cortical malformations, neurocutaneous disorders, or tumors. The list of etiologies was expanded to pathogenic copy number variants at whole-genome array comparative genomic hybridization associated with late-onset cases or pathogenic mutations involving genes, such as , or. Various clinical trials demonstrated the usefulness of different drugs (including rufinamide, clobazam, lamotrigine, topiramate, or felbamate), ketogenic diet, resective surgery, corpus callosotomy, and vagus nerve stimulation in the treatment of epileptic manifestations. The outcome of LGS often remains disappointing regarding seizure control or cognitive functioning. The realization of animal models, which are still lacking, and the full comprehension of molecular mechanisms involved in epileptogenesis and cognitive impairment would give a relevant support to further improvements in therapeutic strategies for LGS patients.

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An atypical case of Lennox-Gastaut syndrome not associated with mental retardation: A nosological issue

Cited by 11 publications

References 10 publications

The pharmacological management of Lennox-Gastaut syndrome and critical literature review

The pharmacological management of Lennox-Gastaut syndrome and critical literature review

Conceptualizing Lennoxâ€“Gastaut Syndrome as a Secondary Network Epilepsy

Lennox–Gastaut Syndrome: A State of the Art Review

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