Literature DB >> 21669558

All children who experience epileptic falls do not necessarily have Lennox-Gastaut syndrome... but many do.

Alexis Arzimanoglou1, Trevor Resnick.   

Abstract

Lennox-Gastaut syndrome (LGS) is a severe, chronic, epileptic encephalopathy, primarily with childhood onset, which is characterised by a triad of features: multiple seizure types, including tonic seizures that may appear late in the course of the disorder; abnormal EEG features with slow spike-wave discharges; and cognitive impairment. Recognition of LGS is problematic, since the seizure types and EEG features that characterise it are not pathognomonic and often change over time. Furthermore, although seizures associated with LGS may occur de novo, the appearance of core LGS seizures may be preceded by prolonged periods of other seizure types, including myoclonic seizures, partial seizures, or infantile spasms. This has led some authors to postulate that a continuum between LGS and other types of childhood epilepsy may exist. Accurate diagnosis requires careful assessment of both clinical and EEG features, in order to distinguish LGS from other childhood epilepsy syndromes, such as atypical benign partial epilepsy of childhood, Dravet syndrome or epilepsies with predominantly myoclonic-astatic seizures. Since there is no biological marker that can be used to confirm diagnosis of LGS and because of the multiple aetiologies that could lead to its development, early referral to a specialist team may prove to be crucial for facilitating both diagnosis and management. Such an approach ensures that patients receive the appropriate treatment at the correct time, providing the best opportunity for the clinical course and overall prognosis to be improved. Effective management of LGS requires regular reappraisal of the evolving symptoms and features, and adjustment of the treatment accordingly.

Entities:  

Mesh:

Year:  2011        PMID: 21669558     DOI: 10.1684/epd.2011.0422

Source DB:  PubMed          Journal:  Epileptic Disord        ISSN: 1294-9361            Impact factor:   1.819


  13 in total

Review 1.  Epileptic encephalopathies: new genes and new pathways.

Authors:  Sahar Esmaeeli Nieh; Elliott H Sherr
Journal:  Neurotherapeutics       Date:  2014-10       Impact factor: 7.620

Review 2.  Clobazam : in patients with Lennox-Gastaut syndrome.

Authors:  Lily P H Yang; Lesley J Scott
Journal:  CNS Drugs       Date:  2012-11       Impact factor: 5.749

Review 3.  Treatment of epileptic encephalopathies.

Authors:  Amy McTague; J Helen Cross
Journal:  CNS Drugs       Date:  2013-03       Impact factor: 5.749

Review 4.  Comprehensive overview: efficacy, tolerability, and cost-effectiveness of clobazam in Lennox-Gastaut syndrome.

Authors:  Michele A Faulkner
Journal:  Ther Clin Risk Manag       Date:  2015-06-08       Impact factor: 2.423

5.  Treatment of Adults with Lennox-Gastaut Syndrome: Further Analysis of Efficacy and Safety/Tolerability of Rufinamide.

Authors:  Rob McMurray; Pasquale Striano
Journal:  Neurol Ther       Date:  2016-02-10

6.  Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature.

Authors:  Jesus Eric Piña-Garza; Steve Chung; Georgia D Montouris; Rodney A Radtke; Trevor Resnick; Robert T Wechsler
Journal:  Epilepsy Behav Case Rep       Date:  2016-02-11

Review 7.  Expert Opinion on the Management of Lennox-Gastaut Syndrome: Treatment Algorithms and Practical Considerations.

Authors:  J Helen Cross; Stéphane Auvin; Mercè Falip; Pasquale Striano; Alexis Arzimanoglou
Journal:  Front Neurol       Date:  2017-09-29       Impact factor: 4.003

8.  Novel seizure outcomes in patients with Lennox-Gastaut syndrome: Post hoc analysis of seizure-free days in rufinamide Study 303.

Authors:  Stéphane Auvin; Betsy Williams; Rob McMurray; Dinesh Kumar; Carlos Perdomo; Manoj Malhotra
Journal:  Epilepsia Open       Date:  2019-03-13

9.  Antiepileptogenic effects of rapamycin in a model of infantile spasms due to structural lesions.

Authors:  Ozlem Akman; Stephen W Briggs; Wenzhu B Mowrey; Solomon L Moshé; Aristea S Galanopoulou
Journal:  Epilepsia       Date:  2021-07-02       Impact factor: 6.740

10.  Stable dosages of clobazam for Lennox-Gastaut syndrome are associated with sustained drop-seizure and total-seizure improvements over 3 years.

Authors:  Joan A Conry; Yu-Tze Ng; Lydia Kernitsky; Wendy G Mitchell; Rima Veidemanis; Rebecca Drummond; Jouko Isojarvi; Deborah Lee; Juliann M Paolicchi
Journal:  Epilepsia       Date:  2014-03-01       Impact factor: 5.864

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