Lennox-Gastaut syndrome: symptoms, causes and treatment

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

Early-onset epilepsy includes Lennox-Gastaut syndrome, characterized by frequent, heterogeneous seizures and varying intellectual disability. In this article we will describe what is Lennox-Gastaut syndrome, what are its causes and symptoms and how it is generally treated with medicine.

    What is Lennox-Gastaut syndrome?

    Lennox-Gastaut syndrome is a more severe form of epilepsy than usual it begins during childhood, between 2 and 6 years; however, symptoms may start to appear before or after this period.

    It was first described in 1950 by William G. Lennox and Jean P. Davis through the use of electroencephalography, which allows the analysis of the bioelectric activity of the brain, detecting altered patterns such as those typical of the epilepsy.

    It is a rare disorder that only accounts for 4% of total epilepsy cases. It is more common in men than in women. It is resistant to treatment, although in some cases the intervention may be effective. In half of the cases, the disease worsens over time, while in a quarter the symptoms improve and in 20% they disappear completely.

    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 during epileptic seizures, so it is recommended to put a helmet on children with the disorder. .

    We think there is one relationship between Lennox-Gastaut syndrome and West syndrome, Also known as infantile spasm syndrome, which has similar features and includes the onset of sudden contractions of the muscles of the arms, legs, torso and neck.

      Symptoms of this disorder

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

      In half of the patients, the seizures tend to be long, more than 5 minutes, or to occur with little time separation; we know it as “for the epileptic state” (state of epilepsy). When these symptoms appear, the person usually has listlessness and dizziness and does not respond to external stimulation.

      In the case of Lennox-Gastaut psychomotor development is usually impaired and delayed as a result of brain damage. The same goes for personality and behavior, which are influenced by problems with epilepsy.

      Usual epileptic attacks

      The epileptic seizures that occur in Lennox-Gastaut syndrome can be very different from each other, which makes this disorder special. The most common seizures are tonic, Which consist of periods of muscle stiffness, especially in the limbs. They usually occur at night, while the person is sleeping.

      Myoclonic seizures are also common, that is, those that they cause sudden muscle spasms or contractions. Myoclonic seizures usually happen more easily when the person is tired.

      Tonic, atonic, tonic-clonic seizures, partial complexes and atypical absence are also relatively common in Lennox-Gastaut syndrome, although less than the previous ones. If you want to learn 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 in any case it is not possible to deduce which of them is responsible for the alteration.

      Among the most common causes of this disorder we find the following:

      • Development as a result of West syndrome.
      • Injury or trauma to the brain produced during pregnancy or childbirth.
      • Infections of the brain, such as 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

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

      Valproate (or valproic acid), topiramate, lamotrigine, rufinamide, and felbamate are among the most common anticonvulsant drugs used in the management of epilepsy. Some of them can cause side effects such as viral disease or liver toxicity.

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

      Although until recently, surgery was not believed to be effective in treating this disorder, some recent studies and research have shown that endoventricular callosotomy and vagus nerve stimulation these are two promising interventions.

      Also, in case of epilepsy administration of a ketogenic diet is generally recommended, Which involves eating few carbohydrates and lots of fats. This appears to reduce the likelihood of suffering from epileptic seizures; however, the ketogenic diet does come with some risks, so it should be prescribed by healthcare professionals.

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