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Lennox-Gastaut syndrome is one form of epilepsy disorder and also called as childhood epileptic encephalopathy or myoclonic-astatic epilepsy. In 1770 Dr. Tissot was first person who describe Lennox-Gastaut syndrome. Many types of seizure are involved in Lennox-Gastaut syndrome because of which it is difficult to control. Most frequent seizures are tonic, atonic and absence seizures but there are also chances for myoclonic, generalized tonic-clonic and partial seizures. It is responsible for mental retardation and behavioral problem. EEG shows a slow spike-and-wave pattern. Most of cases of Lennox-Gastaut syndrome also show non-convulsive status epilepticus. It usually affects childhood between one and eight years of age and peak age is between 3-5 years of age. Chances are slightly more in male than female.
Types of seizure in Lennox-Gastaut syndrome
Different types of seizure involved in Lennox-Gastaut syndrome are as follows
- Tonic seizure-95% chances of occurrence. It includes stiffening of body and breathing problems.
- Myoclonic seizure-sudden jerking movements
- Atonic seizure-loss of consciousness and muscle tone.
- Atypical absence seizure-staring spells
- Tonic-clonic seizures-muscle stiffening followed by jerking movements.
What causes Lennox-Gastaut syndrome?
In 30-35 cases causes are unknown. Some well known causes are given below.
- Brain injury, brain infections such as encephalitis, meningitis, toxoplasmosis, rubella and genetic brain diseases
- Central nervous system infection or metabolic conditions
- In 20% cases West syndrome is responsible for development of Lennox-Gastaut syndrome.
Lesions of the frontal lobe
How to treat Lennox-Gastaut syndrome
Treatment for Lennox-Gastaut syndrome is very difficult because it involves several types of seizures. Investigation for effective treatment is still going on.
- Anticonvulsant, anesthetics medication is used for treatment of LGS.
- Sodium valproate, phenytoin, phenobarbital, lamotrigine, clonazepam, frisium, topiramate and zarontin are used in combination with each other.
- Steroids such as ACTH, immunoglobulins and prednisolone are also used with little side effect.
- Ketogenic diet is rich in fat and less in carbohydrate because of which fat is source of energy which is helpful to reduce seizure.
- Surgery involves following two things
- Vagus nerve stimulation-A small device placed inside chest which sends electric energy to the brain through the left vagus nerve.
- Corpus callosotomy-Surgery involves cutting the front two-thirds of fibre bundle.
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