Lennox-Gastaut syndrome

  • Malignant epilepsy.

INCIDENCE

  • This syndrome can also be referred to by its alternative name.
  • A severe form of epilepsy that affects children is called the Lennox-Gastaut syndrome.
  • Although the true prevalence of the condition is unknown, it is estimated that somewhere in the neighborhood of ten percent of children who have epilepsy have this syndrome.
    • To reiterate, it is believed that as many as half of the children who suffer from epileptic seizures that are challenging to treat have this syndrome.
  • Even though specific requirements have been established in order for this particular syndrome to be recognized (for more information, see below), many authorities are of the opinion that this syndrome ought to be categorized as falling under the umbrella term of epilepsy.
  • When discussing prognosis and treatment, on the other hand, it is helpful to think of this syndrome as a distinct entity in its own right.
  • Both males and females are affected in the same way.
  • Between the ages of three and ten years, the characteristics of this syndrome begin to become apparent, most commonly between the ages of four and five years.
  • There is no antenatal diagnosis possible.

CAUSATION

  • Children who already have brain damage are at an increased risk of developing Lennox-Gastaut syndrome.
  • In as many as one quarter of cases, the child will have had infantile spasms in earlier childhood (also known as "salaam attacks"; see West's syndrome for more information).
  • It is a theory that the Lennox-Gastaut syndrome is a later manifestation of the latter syndrome.
    • This theory has been proposed.
  • The child who is exhibiting the typical epileptic pattern of Lennox-Gastaut syndrome may also have other neurological conditions, such as a brain tumor, a previous severe head injury, a sub-dural haematoma, or a congenital condition such as tuberous sclerosis.
  • All of these conditions can cause epilepsy in children.
  • It is estimated that genetic factors could be responsible for a small percentage of cases, which is somewhere around 3%.

CHARACTERISTICS

  • Seizures of varying types, ranging from recurrent "absences" to convulsive fits to non-convulsive "status epilepticus"; the electroencephalogram shows a pattern typical of the condition; the seizures are especially difficult to control and are resistant to many of the known anti-convulsant drugs; unfortunately, the majority of children who have this syndrome will have a learning disability.
  • It can be challenging to diagnose this syndrome because the fits can occur in such a wide variety of unpredictable patterns.
  • Children are susceptible to the most common form of epileptic seizure, which begins with falling to the ground and is then followed by twitching movements.
  • This kind of seizure can occur while a person is sleeping, which can make diagnosis even more difficult.
  • Other children who exhibit the characteristic EEG pattern of Lennox-Gastaut syndrome may experience very frequent attacks (sometimes so frequent as to be almost continuous) of 'absences' characterized by head nodding, eye blinking, a lack of facial expression, and drooling.
  • These children also have the potential to have Lennox-Gastaut syndrome. Still others are susceptible to what are known as "drop attacks," which involve a sudden and unexpected descent to the ground.
    • This latter symptom may or may not be accompanied by twitching movements.
  • In addition to having frequent seizures, the child may also display behavioral issues.
  • These can include many autistic characteristics as well as many bizarre mannerisms, and they can be especially challenging to deal with if they first manifest themselves in a playgroup or early school setting before a definitive diagnosis has been made.
  • As long as the seizures are carried out, the level of intelligence will slowly but surely decline.
  • It is unclear what led to this unfortunate state of affairs.
  • It's possible that the repeated convulsions are what's causing the learning disability, or it could be that both the convulsions and the learning disability stem from the same underlying pathological cause.

INVESTIGATIONS

  • The electroencephalogram will display the pattern that is characteristic of this syndrome.

MANAGEMENT IMPLICATIONS

  • The use of anti-convulsant medication is the first and most important step that must be taken in order to bring the patient's seizures under control.
  • As was mentioned earlier, this can be extremely challenging, and there is no guarantee that it will ever be completely successful.
  • It is essential that the child's medication be monitored and adjusted on a consistent basis.
  • The fits have been attempted to be managed with steroids, but the results have not been entirely satisfactory.
  • A child who suffers from frequent attacks of "dropping" needs to wear a protective helmet that fits properly in order to avoid suffering from frequent head injuries.
  • There have been varying degrees of success when using a ketogenic diet for treating intractable epilepsy in children who have been given the diet.
    • This diet, which is high in fats and triglycerides, must only be given under the supervision of a nutritionist as well as a pediatrician.
    • (Testing of this diet was initiated after it was observed that the child's seizures had a tendency to improve whenever the child was sick with a fever. Therefore, a diet that attempted to simulate the physiological conditions that are present during an illness that causes fever was tried.)
  • Surgical procedures on the brain are currently being evaluated for use on children who have recurrent attacks of the "drop" syndrome.
  • To accommodate both the frequent seizures and the learning disability, the student's educational experience will need to be highly specialized.
  • It is essential to have the awareness that the child's physical state, and consequently their capabilities, can change drastically from one day to the next.
  • It is essential for the authorities in charge of education and health to maintain close communication.
  • In order for the rest of the family to enjoy a vacation without being responsible for the full-time care of a disabled member, it will be necessary to arrange for respite care for the parents.

THE FUTURE

  • Children born with Lennox-Gastaut syndrome will require ongoing medical attention throughout their entire lives.
  • It will be impossible to lead an independent life given the challenges that will need to be overcome in order to keep the seizures under control, as well as the likely decline in intellectual function.