Presenting typically between four and eight months of age, although it can start earlier or later too, West Syndrome is the name given to a specific type of infantile spasm which falls within the epileptic spectrum.
West Syndrome is an umbrella term used to describe the presentation of a certain type of infantile spasm for which there can be a number of different causes or contributing factors.
A type of epilepsy which was named after the physician who first described it (Dr William James West in 1840) West Syndrome is a type of infantile epilepsy which has very distinctive features.
The spasms typically commence during the first 12 months after birth but usually cease by the time the child has reached the age of 4 years. Although West Syndrome resolves by 4 years, it’s not uncommon for the child to go on to develop alternate forms of epilepsy later in life.
The seizures in West Syndrome are very characteristic, lasting just 1-2 seconds each, but occurring in a series, thus differentiating them from other childhood conditions. Spasms very rarely occur during sleep, often taking place shortly after waking or after feeding.
Each spasm presents as an initial jerk followed by a noticeable spasticity which rapidly resolves. During the spasm, the body can jackknife, with the knees being pulled up to the chest, the arms flung out and the torso forced forward. An alternate presentation which is less common involves the entire body straightening, with the legs stiffening and extending and the head being thrown back.
In each series of seizures, the spasms occur approximately every 5-15 seconds with each series lasting 10-20 minutes. There may be several series of spasms every day.
However, some types of West Syndrome spasm can also be more subtle, with symptoms confined to the cervical spine or other isolated muscle regions within the body. Each spasm can even be limited to abnormalities in eye movement, making it much more difficult to identify.
The differential diagnosis is colic, but as West Syndrome presents as a series of spasms and colic does not, the latter is relatively easy to rule out.
The infant may also appear to lose some of the skills they have already acquired, such as sitting or rolling over, and they may stop babbling. Eye contact may also cease or reduce.
Introduction to West Syndrome
Causes of West Syndrome
Diagnosis of West Syndrome
Treatment & prognosis of West Syndrome