What do infantile spasms look like?
The first description of infantile spasms was by English Physician, Dr. W. J. West, more than 170 years ago. His description is as accurate today as it was then and is very poignant since he was describing his son.
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Infantile spasms can start by with suble head bobbing that can be confused with other normal baby behaviors. The crying out may be confused with colic. However, as time went on, Dr. West noted that the spasms became more and more apparent. Infantile spasms can appear in different ways:
- “Flexor spasms” or jackknife seizures - head drops down and body flexes like a jackknife.
- Extensor spasm- these can look like a cheerleader motion, with an arching of the head and back, and a straightening of the legs.
- In either case, one or both arms may fling out in a motion that mimics a startle response (also called a Moro reflex).
- Clusters of spasms may happen repeatedly during the day but most often occur upon awakening in the morning or after a nap.
Infantiles spasms that occur with Tuberous Sclerosis Complex (TSC) may present differently.
- The spasms are very often a mixed type, with both flexion and extension movements.
- Features such as head turning and eye deviation (turning away) may be present causing the seizures to look like they are arising from one area of the brain.
- Or the seizures may affect the two sides of the body unequally; in some cases, only one side of the body may be affected.
- These different symptoms may cause children with infantile spasms from TSC to be misdiagnosed, leading to a delay in proper treatment.
What is West Syndrome?
Some children with infantile spasms may also be said to have West Syndrome. West Syndrome is actually a combination of epileptic spasms combined with a particular EEG pattern called hypsarrhythmia. Thus, West Syndrome is a subgroup of infantile spasms. Children with TSC (and who have other causes of infantile spasms) have been documented to have infantile spasms without the presence of hypsarrhythmia.
What type of behavioral or developmental problems occur?
Once children begin to have infantile spasms, they often fail to meet new milestones and may even regress, losing intellectual and/or physical skills previously learned. Dr. West clearly described the consequences of IS in his son:
When infantile spasms begin, parents may notice that the child loses interest in people and objects in the their environment. Social interaction may lessen, smiling may cease, sleep may become disrupted, and the child may seem irritable or indifferent to their surroundings. A child who had learned to sit may stop sitting or even lose the ability to roll over. A child who had been babbling happily may become silent or fussy.