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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 1:3200 to 1:3500 of live births. Statistically, boys are more likely to be affected than girls at a ratio of around 56:44. In 45 out of every 50 children affected, the spasms appear for the first time between the third and the twelfth month of age. In rarer cases, spasms may occur in the first two months or during the second to fourth year of age.
It is still unknown which bio-chemical mechanisms lead to the occurrence of West syndrome. It is conjectured that it is a malfunction of the neurotransmitter function, or more precisely, a malfunction in the regulation of the GABA transmission process. Another possibility being researched is a hyper-production of the Corticotropin-releasing hormone (CRH). It is possible that more than one factor is involved. Both hypotheses are supported by the effect of certain medications used to treat West syndrome.
If a cause presents itself, the syndrome is referred to as symptomatic West syndrome, as the attacks manifest as a symptom of another anomaly. These are the possible causes being considered:
On average, West syndrome appears in 1 to 5 per 100 children with Down’s syndrome as babies. Whereas this form of epilepsy is relatively difficult to treat in children who do not have the chromosomal differences involved in Down’s syndrome, the syndrome often affects those who do far more mildly and they often react better to medication. The German Down Syndrom InfoCenter noted in 2003 that what was normally a serious epilepsy was in such cases often a relatively benign one.
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