Lennox Gastaut Syndrome

Lennox Gastaut syndrome is a form of epilepsy that begins in childhood and is typified by frequent seizures. It is habitually accompanied by a delay in the development as well as behavioral and psychological problems.

Childhood epileptic encephalopathy is a pediatric epilepsy syndrome; the condition is typified by compound types of seizures; mental regression; and anomalous EEG, with generalized slow spike-and-wave discharges. The commonest types of seizure are atonic, tonic-axial, and absence seizures, however, myoclonic, generalized tonic-clonic seizures as well as partial seizures may also be seen.

Different therapeutic approaches are used to manage Lennox Gastaut syndrome: anti epileptic agents, diet and surgery. Unfortunately, the treatment is often ineffective.

Lennox–Gastaut syndrome Causes

1. The primary cause for LGS is idiopathic, i.e.; there isn’t any known cause for the disorder. 2. The underlying causes for symptomatic Lennox Gastaut Syndrome are:

Encephalitis and / or meningitis
Encephalopathy
Trauma
Disease and / or developmental disorder
Malformation of the brain
Tuberous sclerosis
Trauma or injury at birth
Hypoxia ischemia injury
Lesions in the frontal lobe
Hereditary metabolic diseases

3. Cryptogenic is the third etiological factor. It means there is no particular cause; however, there may be a family history of epilepsy.

Lennox Gastaut Syndrome Symptoms

• The symptoms of Lennox Gastaut Syndrome are usually unpredictable and come on in the age group of 2 to 6 years. The initial presenting feature frequently is a head-drop, where, the head flops due to the fact that the neck muscles lose their tone. Over an interval of time, the child may fall down because; many muscles in the body lose their tone. These drop attacks are hazardous, since, the child can injure himself.

• The most frequently seen seizure type is: tonic, (usually nocturnal), the second most frequent is myoclonic seizure, which occurs when the patient is tired.

• Atonic, atypical absence fits and tonic clonic seizures are seen as well.

• Status epilepticus is another noteworthy symptom of Lennox Gastaut Syndrome. This state is typified by faintness, lassitude, apathy and indifference. The child will lose consciousness during the fit, and will suffer from a sequence of atonic and myoclonic seizures, with alternating stiffness and floppiness.

• In addition to these everyday multiple seizures, the child also demonstrates behavioral disorders and issues along with impeded / delayed psycho-motor development.

Lennox Gastaut Syndrome Treatment

Generally, LGS patients are treatment resistant; even so, it does not mean that the treatment is pointless. The following therapy options are used to effectively handle a case of Lennox Gastaut Syndrome:

Medicines administered are:

• anti convulsants
• anesthetics
• steroids like prednosolone
• valproates
• benzodiazepines
• felbamate

Surgery

• vagus nerve stimulation
• corpus callosotomy

Ketogenic diet

Experts advise giving the patient a ketogenic diet that triggers ketosis within the body, a state in which a huge amount of ketones build up.

Intravenous immunoglobulin therapy

Intravenous immunoglobulin therapy is known to diminish the frequency of seizures and also demonstrates an improvement in the EEG.