Infantile spasms (West syndrome).

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.

Lots of different brain disorders create the same symptom of West Syndrome. This alone makes diagnosis difficult, it also serves to make locating the right information quite difficult. For those looking for further website information about this condition, other useful search terms have been:

• Salaam Seizure
• Salaam Attack
• Salaam Tic
• Hypsarrythmia
• West Syndrome
• Infantile Spasms

These search terms tend to lead onto a great many other websites of support groups, personal pages of those who have been cured and those with ongoing struggles to tell. Also, there will be sites of further research studies being performed and information of doctors around the globe who may be of assistance. Each case is unique in its own way, what works for one may not work for all, but there is so much to lose for not searching and so much to gain.

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, 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.

The epileptic seizures which can be observed in infants with West syndrome fall into three categories. Typically, the following triad of attack types appears; while the three types usually appear simultaneously, they also can occur independently of each other:

• Lightning attacks: Sudden, severe myoclonic convulsions of the entire body or several parts of the body in split seconds, and the legs in particular are bent (flexor muscle convulsions here are generally more severe than extensor ones).
• Nodding attacks: Convulsions of the throat and neck flexor muscles, during which the chin is fitfully jerked towards the breast or the head is drawn inward.
• Salaam or jackknife attacks: a flexor spasm with rapid bending of the head and torso forward and simultaneous raising and bending of the arms while partially drawing the hands together in front of the chest and/or flailing. If one imagined this act in slow motion, it would appear similar to the oriental ceremonial greeting (Salaam), from which this type of attack derives its name.

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