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Lennox-Gastaut syndrome: symptoms, causes and treatment

Lennox-Gastaut syndrome: symptoms, causes and treatment

November 27, 2021

Epilepsy is a neurological disorder characterized by the appearance of episodes of abnormal electrical activity in the brain that cause convulsions and mental absences, among other symptoms. It is due to alterations in the morphology or the functioning of the nervous system, especially of the encephalon.

Among the early onset epilepsies we find the Lennox-Gastaut syndrome, characterized by frequent and heterogeneous seizures and variable intellectual disability. In this article we will describe What is the Lennox-Gastaut syndrome, what are its causes and symptoms and how it is usually treated from medicine.

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What is Lennox-Gastaut syndrome?

Lennox-Gastaut syndrome is a very serious form of epilepsy that usually It starts during childhood, between 2 and 6 years of life ; however, symptoms may begin before or after this period.


It was described in 1950 by William G. Lennox and Jean P. Davis thanks to the use of electroencephalography, which allows analyzing the brain's bioelectrical activity, detecting altered patterns such as those typical of epilepsy.

It is an infrequent disorder that accounts for only 4% of total cases of epilepsy. It is more common in males than in females. It is resistant to treatment, although in some cases the intervention can be effective. In half of the cases the disease gets worse over time, while in one quarter the symptoms improve and in 20% disappear altogether .

Between 3 and 7% of children diagnosed with this syndrome die between 8 and 10 years after diagnosis, usually due to accidents: it is very common for falls to occur when seizures occur, so it is advisable to wear a helmet to children with the disorder.


It is believed that there is a relationship between Lennox-Gastaut syndrome and West syndrome , also known as infantile spasm syndrome, which has similar characteristics and includes the appearance of abrupt contractions of the muscles of the arms, legs, torso and neck.

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Symptoms of this disorder

This syndrome is characterized by the presence of three main signs: the appearance of recurrent and varied epileptic seizures, the slowing down of brain electrical activity and moderate or severe intellectual disability. He also has problems with memory and learning, as well as with motor alterations.

In half of the patients the crises tend to be long, lasting more than 5 minutes, or they occur with little temporal separation; we know this as "status epilepticus" (state of epilepsy). When these symptoms occur, the person is usually apathetic and dizzy, and does not respond to external stimulation.


In the cases of Lennox-Gastaut psychomotor development is usually altered and delayed as a consequence of cerebral involvement. The same happens with personality and behavior, which are influenced by epileptic problems.

Common epileptic seizures

The epileptic seizures that occur in the Lennox-Gastaut syndrome can be very different between them, something that makes this disorder peculiar. The most frequent crises are tonic , which consist of periods of muscular rigidity, especially in the extremities. They tend to occur during the night, while the person sleeps.

Myoclonic epileptic seizures are also common, that is, those that cause spasms or abrupt muscle contractions . Myoclonic seizures tend to occur more easily when the person is tired.

Tonic, atonic, tonic-clonic, partial complex and atypical absence crises are also relatively frequent in the Lennox-Gastaut syndrome, although to a lesser extent than the previous ones. If you want to know more about the different types of epilepsy you can read this article.

Causes and factors that favor it

There are several causal factors that can explain the development of Lennox-Gastaut syndrome, although not in all cases it is possible to deduce which of them is responsible for the alteration.

Among the most frequent causes of this alteration We find the following:

  • Development as a result of West syndrome.
  • Injuries or traumatisms in the brain produced during pregnancy or childbirth.
  • Infections in the brain, like encephalitis, meningitis, toxoplasmosis or rubella.
  • Malformations of the cerebral cortex (cortical dysplasia).
  • Hereditary metabolic diseases.
  • Presence of tumors in the brain due to tuberous sclerosis.
  • Lack of oxygen during birth (perinatal hypoxia) .

Treatment

The Lennox-Gastaut syndrome is very difficult to treat: unlike most types of epilepsy, this disorder usually occurs resistance to pharmacological treatment with anticonvulsants .

Among the most used anticonvulsant medications in the management of epilepsy are valproate (or valproic acid), topiramate, lamotrigine, rufinamide and felbamate. Some of them can produce side effects such as viral diseases or liver toxicity.

Benzodiazepines such as clobazam and clonazepam are also commonly used. However, the efficacy of any of these drugs in the Lennox-Gastaut syndrome has not been definitively demonstrated.

Although until recently it was believed that surgery was not effective in treating this disorder, some recent research and studies have found that Endoventricular callosotomy and vagus nerve stimulation they are two promising interventions.

Also, in cases of epilepsy the administration of a ketogenic diet is usually recommended , consisting of eating few carbohydrates and many fats. This seems to reduce the likelihood of having epileptic seizures; However, the ketogenic diet carries certain risks, so it must be prescribed by medical professionals.


Pediatric Epilepsy Syndromes – Mayo Clinic (November 2021).


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