The Lennox‐Gastaut Syndrome

Dulac, Olivier; Nguyen, Thi-Minh

doi:10.1111/j.1528-1157.1993.tb04593.x

Cited by 137 publications

(124 citation statements)

References 63 publications

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“…Lennox-Gastaut syndrome (LGS) is a challenging area for nosology (38). As we were not sure which diagnostic criteria had been retained, all possible cases were categorized only as symptomatic generalized epilepsy.…”

Section: Discussionmentioning

confidence: 99%

Newly Diagnosed Unprovoked Epileptic Seizures: Presentation at Diagnosis in CAROLE Study

Jallon¹,

Loiseau²,

Loiseau³

2001

Epilepsia

213

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Summary:Purpose: We describe first unprovoked seizures and newly diagnosed epilepsies at initial presentation, with a special emphasis on epilepsy syndromes, in a large cohort recruited in the mid-1990s in France.Methods: The French Foundation for Research on Epilepsy set up a network to conduct a prospective study of patients with newly diagnosed unprovoked seizures. Information was provided by 243 child or adult neurologists. Four neurologists classified each case according to the International League Against Epilepsy (ILAE) criteria. First-seizure patients and patients with previously undiagnosed seizures were compared.Results: Between May 1, 1995, and June 30, 1996, 1,942 patients aged from 1 month to 95 years were identified: 926 (47.7%) with a single seizure and 1,016 (52.3%) with newly diagnosed epilepsy. All but 17 patients had EEGs. In the firstseizure and newly-diagnosed-epilepsy groups, neuroimaging studies were performed in 78.2 and 68.3% of patients, and medication prescribed in 54.1 and 89.6%, respectively. There were significant differences between the two groups with respect to age at onset and diagnosis, sex, etiology, several specific syndromes, as well as the type and presentation of initial seizure. In patients for whom the first seizure was convulsive, only sex, multiple seizures in a day or status epilepticus, and cryptogenic localization-related syndrome differed between the two groups.Conclusions: Approximately half of patients who first came to attention for an unprovoked seizure already met epidemiologic criteria for epilepsy. There were significant differences between the types of patients with a first seizure and those with newly diagnosed epilepsy. One or several seizures at diagnosis did not influence the diagnostic assessment of the patients but had a strong influence on the initiation of treatment.

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Section: Discussionmentioning

confidence: 99%

Newly Diagnosed Unprovoked Epileptic Seizures: Presentation at Diagnosis in CAROLE Study

Jallon¹,

Loiseau²,

Loiseau³

2001

Epilepsia

213

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“…Collectively, these were termed "ranks of epilepsy." Patterns of seizures rather than single seizure types were chosen since astatic seizures, which may be due to myoclonic, myoclonic-atonic, atonic, and tonic (rigid falls) seizures (1,8,9,10) were frequently mentioned. Moreover, distinguishing these seizures from one another may be difficult without video-EEG monitoring (1,8,9), which was not feasible in the present study due to its retrospective nature.…”

Section: Methodsmentioning

confidence: 99%

“…The transitional forms of epilepsy in the continuum are Doose syndrome (DS), also known as myoclonic astatic epilepsy (MAE) and myoclonic variant of LGS (MVLGS) (1,6). However, due to a lack of proper pathophysiologic models and biologic markers, some investigators (7,8) consider these epilepsies separate entities.…”

mentioning

confidence: 99%

Community‐Based Study of Lennox‐Gastaut Syndrome

Heiskala

1997

Epilepsia

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Summary: Purpose: Before 1986, the spectrum of childhood epilepsies, including Lennox-Gastaut syndrome (LGS) and Doose syndrome (DS), known collectively as "epilepsia myoclonica astatica," was believed to represent a single disease. More recently, some investigators have considered these syndromes to be parts of a continuum. To clarify these theories, neurobiologic factors of the syndromes were studied to determine which qualities were shared and which were unique.Methods: A retrospective (1975)(1976)(1977)(1978)(1979)(1980)(1981)(1982)(1983)(1984)(1985), communitybased (Helsinki metropolitan area and the province of Uusimaa) study was designed to seek children with features of LGS and DS. It was assumed that recall bias and the selection of documented history would be similar throughout the group. Ranks of increasing pathology were assigned to different seizure types, EEG results, and drug treatments. A similar procedure was applied to epidemiologic data. Spearman rank-order correlations were calculated to determine which features correlated with LGS and which correlated with less severe epilepsy.Results: The survey comprised 75 patients with broadly defined LGS. The annual incidence was 2 in 100,000 children aged 0 to 14 years. Prenatal or perinatal abnormalities did not correlate with severity of epilepsy. As compared with the relatively favorable ranks, the severe epilepsy ranks were more often associated with an early onset of epilepsy, an infectious disease at the onset, delayed development before epilepsy, abnormalities in neurologic or neuroradiologic examinations, and a deteriorating course of the condition.Conclusions: Patients with LGS are more likely than patients with less severe epilepsy to have a younger age at onset of epilepsy, an infection or both, and a deteriorating course of the condition.

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“…13 Dulac also mentioned that steroids may be effective in patients with LGS, particularly if steroid therapy is commenced shortly after the onset of cryptogenic LGS. 14,15 However, because of the high relapse rate, the use of steroids is usually limited to times when episodes of nonconvulsive status or periods of frequent seizures occur. 14,15 We were fortunate in that 5 of 17 patients who were seizure free and then relapsed still had residual seizurecontrol values of 450% at the final follow-up.…”

Section: Article In Pressmentioning

confidence: 99%

“…14,15 However, because of the high relapse rate, the use of steroids is usually limited to times when episodes of nonconvulsive status or periods of frequent seizures occur. 14,15 We were fortunate in that 5 of 17 patients who were seizure free and then relapsed still had residual seizurecontrol values of 450% at the final follow-up. However, we cannot explain why such good outcomes were observed in these patients and not in others.…”

Section: Article In Pressmentioning

confidence: 99%