(Lennox-Gastaut syndrome)

MIM 615369  616346  617113

Prevalence: 15/10.000; represents 1 to 2 % of childhood epilepsies. Severe epileptic encephalopathy of childhood.

It can be cryptogenic or symptomatic of an underlying brain illness such as sequelae of perinatal asphyxia, cortical dysplasia,  tuberous sclerosis, a metabolic disease.

Characteristic triad:

-        different types of seizures: tonic, atonic, myoclonic, atypical absences

-        typical EEG trace: diffuse slow (< 3 Hz) interictal spike wave in the waking EEG and fast rhythmic bursts (10 Hz) during sleep

-        progressive mental retardation  and behavioural disorders.

Treatment: antiepileptic drugs in addition to the symptomatic treatment of the consequences of the polyhandicap.

Anesthetic implications:

epilepsy; severely polyhandicapped patient

References :

-        Dhansura T, Bhorkar N, Pawar P, Gandhi S.
Anaesthetic management in a patient with Lennox-Gastaut syndrome.
Indian J Anaesth 2014; 58: 238-9

Updated: February 2019