Coping with an ill child
West syndrome, otherwise known as infantile spasms, is an uncommon to rare and serious form of epilepsy in infants. It is named after the English physician, William James West (1793-1848), who first described it in an article published in The Lancet in 1841. Other names for it are “Generalized Flexion Epilepsy”, “Infantile Epileptic Encephalopathy”, “Infantile Myoclonic Encephalopathy”, “jackknife convulsions”, “Massive Myoclonia” and “Salaam spasms”. The name “infantile spasms” is used to describe both the seizure type and the syndrome.
The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes (“polyetiology”). The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal.
Prevalence is around 1:4000 to 1:6000. Statistically, boys are more likely to be affected than girls at a ratio of around 3:2. In 45 out of every 50 children affected. In rarer cases, spasms may occur in the first two months or during the second to fourth year of age.
Symptoms: In 90 per cent of children with the condition, infantile spasms occur in the first year of life, typically between 3 – 6 months old. Often, to begin with, the attacks are brief, infrequent and not typical, so it is quite common for the diagnosis to be made late. Frequently, because of the pattern of attacks and the cry that a child gives during or after an attack, they are initially thought to be colic.
The typical pattern is of a sudden flexion (bending forward) in a tonic (stiffening) fashion of the body, arms and legs. Sometimes, however, the episodes are extensor (arching). Usually, they are symmetrical but sometimes one side is affected more than the other.
Typically, each episode lasts a few seconds; there is then a pause for a few seconds followed by a further spasm. While single spasms may occur, infantile spasms usually occur in ‘runs’ of several in a row. It is common for babies who have infantile spasms to become irritable and for their development to slow up or even to go backwards until the spasms are controlled.
The spasms are different in different children, and can be so slight to begin with that they are not noticed or are thought to be due to colic. Each spasm starts suddenly and lasts for less than a few seconds. Typically the arms stretch out, and the head may nod forward and the eyes look upwards. To begin with a baby may experience only one or two spasms at a time, but usually over a period of days or weeks they build up into runs of sometimes several dozen occurring at a few seconds intervals. Many occur as a baby falls asleep or wakes up. Sometimes spasms upset a baby, but in others spasms are followed by a brief smile.
Prognosis: In some children, infantile spasms respond easily to treatment, whereas in others they remain very resistant. Many children unfortunately go on to have other kinds of seizure in later childhood and many have learning difficulties. The prognosis for infantile spasms in both these respects is largely dependent on the underlying cause of the condition.
Diagnosis: The diagnosis of infantile spasms is made by a combination of the typical features with a typical EEG which shows a very disorganised pattern termed ‘hypsarrhythmia’. Infantile spasms, like many other electroclinical syndromes, have lots of different causes though a particular cause cannot be identified in each case.
Most children with infantile spasms will need a number of tests (EEG, brain scans, blood tests, urine tests and, sometimes, spinal fluid tests) in order to try to identify the underlying cause