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Lennox-Gastaut syndrome is an epileptic syndrome that typically begins in childhood and is associated not only with epileptic seizures, but also with intellectual disorders. Lennox-Gastaut syndrome is unfortunately a serious disease, the treatment of which is often associated with various difficulties. What causes this disease and what are the treatment options?

Lennox-Gastaut syndrome(abbreviatedLGS , derived from the English nameLennox-Gastaut syndrome) is one of the types of childhood epilepsy. The first symptoms of the disease may appear in children of all ages, but most often the disease begins between the ages of 2 and 6. Lennox-Gastaut syndrome can affect children of both sexes, but boys are about twice as likely to suffer from it. According to statistics, LGS accounts for up to 4% of all epilepsy in children.

Lennox-Gastaut syndrome: causes

In general, there are two types of Lennox-Gastaut syndrome. The first is sometimes referred to as primary or idiopathic - LGS is defined as such when it is not possible to find the cause of the disease in a child. Much more common, accounting for up to 80% of all cases of Lennox-Gastaut syndrome, is the secondary form, i.e. the one in which the patient can find some neurological abnormalities. In such a situation, the causes of Lennox-Gastaut syndrome may be:

  • encephalopathy
  • tuberous sclerosis
  • Congenital metabolic diseases that damage nerve cells
  • encephalitis or meningitis
  • brain damage related to ischemia or hypoxia (e.g. those occurring during childbirth)
  • toxoplasmosis
  • head injuries (especially those in which the frontal lobes of the brain are damaged)

In some, not fully known way, another childhood epilepsy syndrome is associated with Lennox-Gastaut syndrome - West syndrome. It turns out that West syndrome precedes the development of Lennox-Gastaut syndrome in up to 40% of all LGS patients.

Lennox-Gastaut syndrome: symptoms

For the Lennox-Gastaut syndrome, the triad of problems they are is the most characteristicExtremely frequent seizures, varying degrees of intellectual disturbances and EEG deviations typical of LGS. The range of possible seizure types in LGS patients is probably the largest of any other epilepsy syndromes. Most often, in the course of Lennox-Gastaut syndrome, tonic seizures occur, which are especially likely to occur at night. The second most frequent type of seizures in LGS are myoclonic seizures, which tend to occur especially when the patient with LGS is overworked. Other seizures in the course of Lennox-Gastaut syndrome are:

  • atonic
  • focal points
  • partially-folded
  • unusual absences
  • tonic-clonic

A significant difficulty in the case of Lennox-Gestaut syndrome is how often a child may experience epileptic seizures - in the course of an individual they may occur up to 100 times a day. The risk of status epilepticus is also quite high in patients with LGS - according to statistics, it occurs even in half of patients with Lennox-Gastaut syndrome. Intellectual disorders in children with Lennox-Gastaut syndrome vary in severity. They are primarily caused by frequent seizures - in children suffering from LGS, seizures appear many times a day. The consequences of such a phenomenon are disorders of psychomotor development, speech disorders, behavioral disorders or problems with social functioning.

Lennox-Gastaut syndrome: diagnosis

Electroencephalography (EEG), performed both at rest and during sleep, plays a key role in the diagnosis of Lennox-Gastaut syndrome. The most common deviation found in this study is a slowing of the basic brain wave activity, as well as paroxysmal discharges of irregular slow waves and the appearance of spike-wave syndromes with a frequency of 2-2.5 Hz. If a child is suspected of having LGS, other tests are also performed. mainly imaging tests. Magnetic resonance imaging of the head or computed tomography of the head are used to search for a potential cause of Lennox-Gastaut syndrome - thanks to such examinations it is possible to detect, for example, ischemic foci in the child's brain.

Lennox-Gastaut syndrome: treatment

Pharmacotherapy is the mainstay of treatment for Lennox-Gastaut syndrome. The first-line drugs in the treatment of the disease include:

  • rufinamid
  • walproiniany
  • benzodiazepines (especiallyclonazepam, nitrazepam and clobazam)
  • felbamat

Other drugs that are also used to treat Lennox-Gastaut syndrome are:

  • topiramate
  • zonisamid
  • lamotrygina

It is not uncommon to achieve improvement in Lennox-Gastaut syndrome through single drug therapy, therefore, different combinations of antiepileptic drugs may be used in different patients. Generally, LGS belongs to the group of drug-resistant epilepsy, and therefore, sometimes it is implemented in patients with effects other than pharmacotherapy. Dietary changes can be helpful in some patients with refractory epilepsy, including those with Lennox-Gastaut syndrome. Particular attention is drawn to the ketogenic diet, in which you should consume large amounts of fatty compounds with the simultaneous limitation of protein substances and carbohydrates. The ketogenic diet can actually help LGS patients, but it should never be used on its own - it should always be done only after consulting a doctor, and be under your constant care while maintaining this diet. when the patient's condition is not improved by other methods, surgical treatment may be used. The most common procedures performed in this case are the implantation of the vagus nerve stimulator and the surgery involving cutting the commissure of the great brain (called calosotomy).

Lennox-Gastaut syndrome: prognosis

The prognosis for LGS is rather poor. Even more than 3/4 of patients with this unit, despite many years of treatment, may not be able to live independently in adulthood and may require care from other people. The prognosis is worse, e.g. those in whom LGS was preceded by West's syndrome or whose disease started early. The prognosis is also worse the more often the patient experiences seizures. Lennox-Gastaut syndrome also poses a risk of experiencing injuries - various types of accidents occur among patients with this epilepsy syndrome much more often than in the general population.

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Category:

Neurology