West Syndrome

What is West Syndrome?

This is a very rare to uncommon epileptic syndrome in infants. It was first described in medical literature by an English physician W.J. West who described this syndrome first in his own son. Other names for this syndrome include:


  • Infantile Epileptic Encephalopathy
  • Generalized Flexion Epilepsy
  • Jackknife convulsions
  • Infantile Myoclonic Encephalopathy
  • Massive Myoclonia
  • Salaam spasms

The name “infantile spasms” may be used to designate the precise seizure exhibition in the syndrome but it may also be used as an alternative expression for the syndrome itself. This syndrome is a triad of a pathognomonic EEG pattern – called hypsarrhythmia, mental retardation, and infantile spasms

This syndrome is related to age, usually happening amid the 3rd and the 12th month, usually displaying about the 5th month. There are various causes.

Symptoms

Symptoms for this syndrome include:

Infantile spasms

More often with this sort of seizure the baby will suddenly bend frontward at the waist and the arms, legs and body get stiff. This is often referred to as “jackknife” seizure. With selected infants they might bob their heads, or arch their backs, or cross arms across their body as if they are embracing themselves. The spasms last only several seconds occurring in groups of anywhere from two (2) to one hundred (100) spasms at one time. Some babies have loads of these groups of spasms in a day. These spasms more frequently occur after the baby wakes up in the morning or after a nap.

Hypsarrhythmia

An EEG or electroencephalogram records electrical action in the brain as brain waves. An infant with this syndrome will have chaotic, abnormal patterns of brain waves referred to as hypsarrhythmia.

Mental retardation

This is caused by the brain injury which caused the syndrome. Other symptoms can be evident because of the disorder that is underlying the causes of this syndrome. Other disorder neurologically for instance cerebral palsy can also be present.

This is known as the triad of symptoms.

Causes

West syndrome has many causes for instance:

  • Severe brain injury at birth
  • Brain malformations
  • Metabolic disease
  • Chromosomal abnormalities
  • Genetic condition referred to as Tuberous Sclerosis

In about twenty-five (25) of children affected, the cause cannot be established.

Treatment

Infantile spasms are normally treated either with a course of Prednisolone – oral steroid – or often a course of ACTH – making the body create extra steroid. If other kinds of seizures occur there are a huge number of other anti-epileptic medications that can be tried. With this syndrome, as in all types of epilepsy, the medication’s effect on a specific child cannot be foretold in advance so they may need to try several and some children may need more than one medication at a time. Dependent on the underlying cause, surgery of the brain might also be a possibility for a small number of children. As with all children having disabilities with learning, education and therapies – physio/speech/occupational – should be scheduled for the child as this will ensure that he/she is assisted in reaching their full potential as well as live life to the fullest, however severe or mild their problems are.

Prognosis

It isn’t possible to make a general prognosis for development due to the unpredictability of causes, the differing sorts of etiology and symptoms. Every case needs to be individually considered.

The prognosis for those with idiopathic syndrome is more positive than those with the symptomatic or cryptogenic forms.

Idiopathic cases are less likely to show indications of problems with development before the attacks start, the attacks can be easily and effectively treated and there is a lower rate of relapse.

Those with this type of syndrome are less likely to go on to cultivate other forms of epilepsy; around 3 in every 5 progressing at the same rate as children who are healthy.

In other cases, treatment of West syndrome is difficult and results of therapy dissatisfying.

About half of all children can become totally free of attacks with the aid of medication. Treatment is satisfactory in about 3 out of 10 cases with 1 in every 25 showing motoric and cognitive development evolving more or less normally.

Up to 90% suffer severe cognitive and physical impairments even if treatment is successful. This is not because of epileptic fits but rather the causes behind them such as cerebral anomalies or degree of severity of their location.

Life Expectancy

Five out of every 100 with West syndrome do not survive beyond 5 years age – some due to the syndrome and others because of medication.

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