The educational and social progress of a child with epilepsy depends not only on seizure control but also on cognitive and behavioral factors. The various epilepsy syndromes of childhood and adolescence differ greatly in terms of cognitive and behavioral outcome. A high proportion of babies who have West syndrome and children who have Dravet syndrome (severe myoclonic epilepsy in infancy) will have long-term cognitive and behavioral problems. The Lennox-Gastaut syndrome also often has a poor prognosis in this regard. Children with the Landau-Kleffner syndrome have a variable prognosis, some regain speech and others have permanent speech impairment. Benign childhood epilepsy with centrotemporal spikes is now recognised as lying on a spectrum with the Landau-Kleffner syndrome: mild cases have few if any cognitive or behavioral problems but others may have quite severe difficulties. People with juvenile myoclonic epilepsy may have characteristics suggesting frontal lobe impairment. The educational and social impairments associated with the epilepsy syndromes of childhood and adolescence are of major importance but they have been the subject of remarkably few well-performed studies. The impairments are not always necessarily permanent and it seems highly likely that the cognitive and behavioural outcome of at least some of these syndromes can be influenced greatly by early effective treatment with either antiepileptic medication or surgery.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1528-1167.2006.00709.x

Cognitive and Behavioral Outcomes of Epileptic Syndromes: Implications for Education and Clinical Practice

Lennox-Gastaut Syndrome (LGS) is a category of severe, disabling epilepsy, characterised by frequent, treatment-resistant seizures and cognitive impairment. EEG (electroencephalography) shows characteristic generalised 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 characterised by a diffuse high voltage slow transient evolving into generalised 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 (SSW) and bursts of generalised paroxysmal fast activity (GPFA)) also have a ‘generalised’ electrical field, suggesting widespread cortical involvement. Recent brain mapping studies have begun to reveal which cortical and subcortical regions are active during these ‘generalised’ 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 conceptualised as a ‘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 a ‘secondary’ network epilepsy because the epileptic manifestations of the disorder reflect the networks being driven, rather than the specific initiating process.

/pdf/conceptualizing-lennox-gastaut-syndrome-as-a-secondary-4arw75qoa0.pdf

Conceptualizing Lennox-Gastaut syndrome as a secondary network epilepsy

No other epilepsy more substantially combines cryptogenicity and intractability than does Lennox-Gastaut Syndrome. Although antecedent neurological conditions have preceded LGS in some patients, others with similar ills fail to develop this syndrome, and precise charting of the pathway between any presumed aetiology and the epilepsy has eluded researchers. Despite these frustrations, LGS has elicited a host of publications over the years, possibly because it combines features of many other epileptic disorders and thereby occupies a crossroads position among them. Lacking a comprehensive experimental model of LGS, this article combines relevant data from several clinical and basic sources in order to formulate a concept of pathogenesis. Data are presented to suggest that the occurrence of factors enhancing excitability during a vulnerable period of cortical and thalamic development may permanently imprint a bilateral, diffuse epileptogenic system upon the mammalian brain.

Pathogenesis of Lennox-Gastaut syndrome: considerations and hypotheses.

Lennox—Gastaut syndrome in adulthood: Clinical and EEG features

In 102 patients with Lennox-Gastaut syndrome (LGS) observed for an average of 16 years (range, 10-20 years), 12 of the patients worked normally, 36 worked part-time or at a sheltered workshop, and most of the remaining 54 were under home care or institutionalization. LGS evolved from West syndrome or from unspecified epilepsies, or as the primary form, mostly in childhood and rarely in adolescence or adulthood. At the worst stage, there were diverse types of generalized seizures, slow spike-and-wave EEG complexes, fast rhythms, and multiple spike-and-wave complexes. Mental subnormality progressively worsened. Characteristic symptoms of LGS continued in one third of patients, and various abortive forms of LGS were seen in the other two thirds, although LGS did not evolve into a localization-related epilepsy during the survey period. The evolution of seizure and EEG epileptic discharges suggests that LGS has a characteristic clinical course as it progresses. The persistent generalized tonic seizures of various magnitude over a long time in the vast majority of patients indicate that the brainstem rather than the cortical mantle is involved as the seat of seizure-generating mechanisms in LGS.

Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study

[Lennox-Gastaut syndrome in the adult].

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[Lennox-Gastaut syndrome in the adult].