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Lennox Gastaut Syndrome: An Overview

 

            
             Lennox-Gastaut Syndrome (LGS) was initially outlined from the results of studies into the encephalopathy (brain function/disorder) of `petit-mal' seizures by Lennox and Davis (1950), and was later expanded to its now widely accepted form as a syndrome by Gastaut, Roger, Soulayrol, Tassinari, Regis, Dravet, Bernard, Pinscard, and Saint-Jean (1966).
             LGS is described as having three interrelated symptoms, consisting of the following:.
             1. Epileptic seizures (axial tonic, atypical absences, atonic seizures, and less frequently - myoclonias, generalised tonic-clonic, and partial seizures).
             2. Electroencephalogram (EEG) abnormalities. (slow spike-wave in the awake EEG, and rapid burst rhythms during sleep).
             3. Slowing in mental development with personality disorders.
             LGS describes one of the most severe forms of childhood epilepsies. An extensive review of the literature suggests that LGS usually develops between the ages of 1 and 8 years, affects more males than females, with half having no known cause and the remainder having various aetiologys (causing factors) affecting the brain and directly causing epilepsy.
             This article examines the clinical manifestations of the LGS, detailing the seizure types, the implications for the individual's mental development, and the various attempts to manage the epilepsy through pharmacological (ie drugs) and other means.
             Seizures in LGS.
             LGS is characterised by repeated, brief seizures, often responsible for multiple falls, with the length and type of seizures being somewhat idiosyncratic (individual in nature), but of a clearly defined group of seizure types.
             The seizures which occur in LGS are clearly defined, although there are several factors which influence the predominance of one type over the others - for example: age (younger children tend to have only one type of seizure, with other types appearing as their age increases); and state of consciousness (atypical absences with falls and amyotonias more frequent if the child is not stimulated, and tonic seizures are always observed during sleep).


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