West syndrome is an epilepsy syndrome characteristic of the childhood population. West's syndrome as childhood epilepsy is quite a serious disease, which results, among others, from from the fact that it can lead to considerable intellectual delay. The risk of mental dysfunction increases the later the treatment of West's syndrome is started - so what are the symptoms of this disease and what should prompt parents to visit a neurologist?

West syndromeis one of the types of childhood epilepsy. The name of this unit comes from the author of the first description of it, published in 1841 in the Lancet - William James West.

In his publication, an English doctor described a case of a patient who was very close to him - this description concerned the son of W.J. Vesta.

According to statistics, West syndrome occurs in 1 in 3500 children, boys suffer from this disease slightly more often. The illness usually begins between three and twelve months of age.

West syndrome: causes

Any conditions associated with organic changes in the brain can actually lead to West syndrome - pathologies of this kind may occur in the prenatal period, as well as in the perinatal period, or at different times after the birth of a child. The most common disorders that are associated with West syndrome are:

  • hydrocephalus
  • microcephaly
  • polymikrogyria
  • Sturge-Weber syndrome
  • tuberous sclerosis
  • congenital infections (e.g. cytomegalovirus)
  • hypoxic ischemic encephalopathy
  • intracranial hemorrhages
  • meningitis and its consequences
  • maple syrup disease
  • phenylketonuria
  • Down syndrome
  • mitochondrial encephalopathies
  • cerebral vessel malformations
  • head injuries

Even if a child does suffer from any of the diseases listed above, it is not always possible to find out why a small patient develops epileptic seizures.

The hypotheses of scientists currently focus mainly on disturbances in the number of neurotransmitters - the neurotransmitter GABA attracts the most attention. Other theories concern one of the hormones, namely hypothalamic corticoliberin(CRH).

It is possible that the production of this substance in excessive amounts may lead to disturbances in the electrical activity of the brain and related epileptic seizures.
Sometimes the child is not able to detect any defects in the brain, and it still suffers from West Syndrome. In such situations, the disease is referred to as idiopathic. In fact, it is quite often impossible to establish the causes of West's syndrome - up to 30% of patients with this disease are diagnosed with the idiopathic form of this syndrome.

West syndrome: symptoms

The most characteristic of West syndrome areflexion epileptic seizures . During such an attack, there is a sudden, generalized contraction of the flexor muscles.

  • Epilepsy symptoms in children and adults. How to recognize epilepsy?

The effect of such a situation is that in a child lying on its back, the head is bent, along with the torso, forward. There is also noticeable bending of the lower limbs to the body, while the child's upper limbs are most often thrown forward.

The hallmark of the seizures that appear in a child with West syndrome is that they usually occur in series. Most often, the seizures occur when the child wakes up or falls asleep.

In West's syndrome, however, the problem is not only epileptic seizures - seizures may be accompanied or followed by such ailments as:

  • disturbance of consciousness
  • drooling
  • vomiting
  • flushing
  • psychomotor restlessness
  • significant increase in sweating

Another problem is characteristic of West's team. Namely, quite often (especially in the case of the lack of treatment or failure in its implementation) the child's psychomotor development may be inhibited.

West syndrome: diagnostics

The most important thing in the case of suspicion of West's syndrome in a child is an electroencephalographic examination (EEG). The most favorable situation is when the test is performed both while the child is awake and when the child is asleep.

A deviation in the EEG, characteristic of West syndrome, is a hypsarithmic record (i.e. one in which, in addition to the irregularity of the recording of brain wave activity, many sharp waves, sometimes referred to as spikes) are noticeable.
In the diagnosis of West syndrome many other tests can also be performed to find the pathology responsible for the disease.

For this purpose can be performedimaging tests, such as magnetic resonance imaging or computed tomography of the head - these tests are very important because, according to statistics, even 8 out of 10 children with West syndrome have noticeable deviations in neuroimaging tests.

Laboratory tests such as blood counts, urine tests or analysis of the cerebrospinal fluid collected during puncture are also used.

West syndrome: treatment

Treatment of West's syndrome is based primarily on pharmacotherapy. The therapy should be implemented as soon as possible, because the greater the delay between the onset of the disease and the commencement of its treatment, the greater the risk that the child will experience a psychomotor delay.

  • Treatment of epilepsy: drug treatment, surgery and side effects

Drugs such as vigabatrin and synthetic corticotropin derivatives (ACTH) are the most commonly used in the treatment of West syndrome. Other preparations, such as derivatives of valproic acid, drugs from the benzodiazepine group or lamotrigine, are used less frequently.

Rarely used, but one of the treatment options for West's syndrome, are epilepsy surgery.

Another effect that may be effective in children with West syndrome is the use of a ketogenic diet (high fat and protein restriction).

  • Ketogenic Diet (Low Carbohydrate Diet)

There is indeed evidence that the ketogenic diet may have beneficial effects in this childhood epilepsy, however, it should only be introduced after consulting a doctor and only after obtaining his consent.

Other treatments for epilepsy:

  • Stimulation of the vagus nerve in the treatment of epilepsy
  • Telemedicine in the treatment of epilepsy

West syndrome: prognosis

In the case of West's syndrome, the prognosis is rather poor. Statistically, about 5% of children die before the age of five.

A variety of problems can be encountered in surviving children. Even 90% of them experience cognitive impairment, while significant mental retardation can be found in up to 70% of patients.

Mental disorders in the form of autistic behavior or excessive psychomotor activity are also common. Another problem with West syndrome is that in a significant proportion (even 6 out of 10) of patients, epileptic seizures will also occur in their adult life.

Almost half of the patients convert West's syndrome into another type of childhood epilepsy syndrome, which is;

  • Lennox-Gastaut syndrome

Quite an interesting phenomenon, although still incomprehensible to medicine, is that children with idiopathic West syndrome have a better prognosis than those who have been diagnosed with some pathology related to the structures of the nervous system.

More information about packages in children:

  • Epilepsy syndromes
  • Childhood epilepsy with absences (pycnolepsy, Friedman syndrome)
  • Roland's Epilepsy

What else do I need to know about epilepsy?

  • SEIZURES - what to do and how to help the patient
  • Seizures: types
  • Status epilepticus: causes, symptoms, treatment
  • Psychogenic pseudo-epileptic seizures

Category:

Neurology