Treatment & prognosis West Syndrome

Because of the manifest underlying causes of West Syndrome, and the possible outcomes and complications, it’s not unusual for a multi-discipline approach to be adopted. This could include paediatricians, surgeons and neurologists as well as other healthcare professionals.

At present there are two primary courses of treatment that can be adopted for any infant diagnosed with West Syndrome:

  1. steroid therapy either via adrenocorticotropin [ACTH] or prednisolone, corticotrophin and hydrocortisone
  2. anti-convulsants such as vigabatrin

Most infants respond well to treatment but it’s not uncommon for West Syndrome to become refractory, thus necessitating a change in therapy at a later date to maintain control.

Although treatments for West Syndrome can be effective, they are not without risk and in every case there needs to be careful monitoring and the balancing of possible adverse effects against the potential benefits.

Some of the side effects which are regularly encountered in varying degrees of severity include:

  1. steroids - hypertension, immunosuppression, agitation/irritation, gastric problems, glycosuria
  2. vigabatrin - MRI abnormalities (transient hyper-intensity of deep structures), irritability, visual field defects which are irreversible

Many factors will be considered by the physicians when deciding on the optimum treatment and each case will be judged on the individual merits. Steroid therapy is often believed to be more effective in the short term, achieving superior control within two weeks of commencement. However, in the longer term, when compared with vigabatrin over a year, the relative effectiveness was the same. The responsiveness of any infant diagnosed with either cortical dysplasia or tuberous sclerosis is generally better with vigabatrin compared to steroids.

For those infants where vigabatrin is not successful, and steroid use is either ineffective or contraindicated, other anti-convulsants may be tried including:

  1. valproic acid
  2. benzodiazepines
  3. rufinamide
  4. topiramate
  5. zonisamide

A ketogenic diet has also been used in the treatment of infantile spasms in tandem with medication.

For cases where a manifestation of tuberous sclerosis is causing the spasms, or where there is a malformation, surgery be used. However, this should be considered as a last resort for treatment because of the associated risks.

Prognosis

Although West Syndrome appears to spontaneously resolve by the age of 4 there is some evidence to suggest that the infants affected may go on to experience a developmental delay. This is particularly the case in those who have an underlying brain injury or disorder, where the development delay has a greater likelihood of being moderate to severe.

Where there was no pre-existing brain condition, the prognosis is brighter, with 10-20% of infants going on to develop normally without any delay. Of those that do experience a development delay, there is a strong likelihood that this delay will be mild.

The development delays that can be seen often include psychomotor retardation and poor co-ordination in varying degrees of severity.

There is a a greater chance that any child who experiences infantile spasms will go on to to be diagnosed with autism.

Irrespective of prior brain injury or not, current medical research suggests that the more quickly the spasms are brought under control, the better the outcome will be. Prompt diagnosis and effective treatment is therefore seen as key to a preferential prognosis.

Aside from global delays, there is an increased risk of developing a different form of epilepsy. Between one in three and one in five children with West Syndrome go on to be diagnosed with Lennox-Gastaut Syndrome. This is a classification of epilepsy which is particularly refractory, with mixed types of seizures and an associated intellectual disability. Lennox-Gastaut Syndrome is usually severe and life-long, although a small number of children affected may outgrow it. 

A further third of infants diagnosed with West Syndrome will go on to be diagnosed with a different form of epilepsy which presents at a later age.

The prognosis for West Syndrome will be affected by any underlying condition, genetic or otherwise, and it’s essential that this is accurately diagnosed and treated as necessary.

The infants for where there is no clear underlying cause for West Syndrome have the best prognosis. Typically a third of infants diagnosed with West Syndrome make a full recovery, and most of these fall within this group.

Introduction to West Syndrome

Causes of West Syndrome

Diagnosis of West Syndrome

Treatment & prognosis of West Syndrome