Appendix 15 (B) - Infantile Spasms (IS)
Infantile spasms (IS) is a rare condition that occurs in young children, usually under the age of one. The condition is also known as West syndrome. Around 400 children a year are diagnosed with infantile spasms in the UK. The average age of onset is around four months, but some children may experience spasms as early as one month, and a few may begin as late as two years.
A child experiencing infantile spasms (IS) seizures has a chaotic brainwave pattern, detectable on an EEG, which can be associated with loss of skills and brain damage. It is incredibly important, therefore, to recognise that a child has IS as soon as possible, because medication can be given that may control the spasms.
The longer the spasms continue before they are treated and controlled, the greater the risk that development will be affected. If spasms are stopped quickly, children have a better chance of good development.
What does a spasm look like?
Infantile spasms often have a very subtle appearance that makes it difficult to identify as a serious problem. The spasms can look similar to common disorders such as colic or reflux, or a baby’s normal ‘startle’ reflex.
The typical pattern is a sudden bending forward and stiffening of the body, arms and legs. Sometimes, the episodes are different, with the arms and legs being flung outwards. Usually, they affect both sides of the body equally. Typically, each episode lasts just one or two seconds, followed by a pause for a few seconds, then a further spasm. However, a child having infantile spasms may just have little head drops that do not appear to be anything serious.
What should you do?
If a child is thought to have infantile spasms, they need urgent referral to a paediatrician, and probably discussion with, or review by, a neurologist. If you suspect a child is having IS seizures, try to capture them on film and go to your nearest children’s A&E department.
To diagnose IS in a child with suspicious movement patterns, their brainwaves need to be assessed with an EEG test (electroencephalogram). The EEG is a test that measures electrical activity in the brain by placing a series of small electrodes on the child’s scalp and connecting them to a computer. It often involves taking a video of the child’s movements at the same time (video-EEG) to correlate any seizure episodes or movements that might cause an artefact on the trace.
How can infantile spasms be treated?
Infantile spasms do not generally respond to the types of anti-epileptic drugs (AEDs) used to treat most other forms of epilepsy. Two types of medication have proved to be most effective and they are likely to be recommended, singly or in combination, as the initial treatment.
Hormonal treatment with ACTH or prednisolone
Treatment with vigabatrin (Sabril)
Other options for the treatment and management of IS include a ketogenic diet, epilepsy surgery and other AEDs. More information about the treatment of IS can be found in this guide:
https://ukinfantilespasmstrust.org/ukistwp/wp-content/uploads/2018/11/UKIST-Information-Guide.pdf
https://ukinfantilespasmstrust.org/
Online support group
Parents worried about infantile spasms can access advice and support through our Facebook support group
