Lennox-Gastaut syndrome: a comprehensive review

@article{AsadiPooya2017LennoxGastautSA,
  title={Lennox-Gastaut syndrome: a comprehensive review},
  author={Ali Akbar Asadi-Pooya},
  journal={Neurological Sciences},
  year={2017},
  volume={39},
  pages={403-414}
}
Lennox-Gastaut syndrome (LGS) is considered an epileptic encephalopathy and is defined by a triad of multiple drug-resistant seizure types, a specific EEG pattern showing bursts of slow spike-wave complexes or generalized paroxysmal fast activity, and intellectual disability. The prevalence of LGS is estimated between 1 and 2% of all patients with epilepsy. The etiology of LGS is often divided into two groups: identifiable (genetic-structural-metabolic) in 65 to 75% of the patients and LGS of… 

Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies

TLDR
Six treatments are specifically indicated as adjunct therapies for the treatment of seizures associated with LGS in the US: lamotrigine, clobazam, rufinamide, topiramate, felbamate and most recently cannabidiol, which have demonstrated reductions in drop seizures in 15%–68% of patients across trials.

The Curious Case of Lennox-Gastaut Syndrome: Treatment-Resistant Seizures in a Patient With Autism Spectrum Disease With Lennox-Gastaut Syndrome

TLDR
An eight-year-old boy presenting to the emergency department with seizures and mental retardation is reported, diagnosed with LGS with concomitant ASD and successfully been treated for his treatment-resistant seizures.

Adjunctive Rufinamide in Children with Lennox-Gastaut Syndrome: A Literature Review

TLDR
The use of adjunctive RFN in children and adolescents with LGS and its efficacy and safety profile, based on a systematic literature review, shows a very favorable profile in terms of adverse events and drug-interactions.

[Why the diagnosis of Lennox-Gastaut syndrome is a rare one?]

TLDR
D diagnosis of the syndrome is complicated by its polyetiology (clinical and EEG manifestations can vary significantly), the evolution of seizure types and EEG characteristics as the patient matures, the presence of other epileptic syndromes similar to Lennox-Gastaut syndrome.

Characteristics of Genetic Variations Associated With Lennox-Gastaut Syndrome in Korean Families

TLDR
To identify causative mutations of Lennox-Gastaut syndrome, the whole-exome sequencing data of 17 unrelated Korean families, including patients with LGS and LGS-like epilepsy without brain abnormalities, was analyzed using the Genome Analysis Toolkit and 14 mutations in 14 genes were identified as causes.

Anti-seizure medications for Lennox-Gastaut syndrome.

TLDR
This review included 11 trials using add-on ASMs for LGS in children, adolescents and adults and found high-certainty evidence that 72 more people per 1000 (confidence interval (CI) 4 more to 351 more) had adverse events (AE) leading to study discontinuation following treatment with ASMs, compared to two studies with no trials of ASM monotherapy.
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References

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Lennox‐Gastaut syndrome (LGS) is a rare epileptic encephalopathy with a peak age of onset of 3–5 years of age. Reported prevalence rates for LGS vary widely from 1–10% of all childhood epilepsies.

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TLDR
An improvement in the management of Lennox-Gastaut syndrome requires a better understanding of the pathophysiology of this disorder and the development of animal models in which to test new compounds.

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TLDR
Rufinamide was an effective and well-tolerated treatment for seizures associated with Lennox–Gastaut syndrome and had a greater improvement in seizure severity than placebo.

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TLDR
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TLDR
It is shown that Lennox-Gastaut syndrome is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment with standard antiepilepsy drugs.

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TLDR
Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects, and cannabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation.

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TLDR
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