Infantile spasm is a type of seizure seen in an epilepsy syndrome during the infant and early childhood years. This syndrome is known as West Syndrome. An EEG is done to confirm the diagnosis and will show a chaotic brain wave pattern, hypsarrythmia.
The spasms can begin in the first year of life, between 3 and 12 months of age. Although, in most children, spasms stop by age 2 – 3 years, about half of children will begin to have other types of seizures. These can occur before, or at the time the spasms start.
What are the spasms?
They are clusters (or groups) of brief seizures that consist of a sudden bending forward of the body either at the waist or neck. This is followed by the legs being stiff or the jerking forward of both the arms and legs. These spasms are sometimes called “jackknife seizures”. Some spasms consist of arching of the body torso. Each spasm can be brief (between 1 and 5 seconds) but can occur in clusters of 2 – 100 spasms at a time. It is common for the baby to cry out after a spasm or be crabby between spasms and when they stop. Spasms occur more often when the child is drowsy and falling asleep or upon waking from sleep. They rarely occur during sleep. Spasms may occur many times per day. Sometimes spasms are confused with colic, but the cramps of colic do not occur in clusters.
What causes the spasms?
It is a rare form of epilepsy that affects only one baby out of three thousand. In about 20%of the cases, the cause is not known and the baby develops in a normal way.
In the other cases, there are many reasons that could cause this type of epilepsy:
- Brain malformations
- Brain injuries or infections
- Chromosome abnormalities
- Metabolic disorders
- Syndromes like tuberous sclerosis that involve the brain, skin, and heart
What tests will be done?
- An EEG or video EEG will be done to confirm the diagnosis. EEG will be done again some weeks later to see if the treatment is helping.
- MRI scan of the brain
- Blood tests for metabolic disorders
What is the treatment?
The treatments for infantile spasms include steroids by mouth, hormone shots (ACTH), or medicines used to control other types of seizures.
ACTH is often used as the first treatment. It is a hormone made by the pituitary gland. It tells the adrenal glands to make and release more cortisol, which acts much like a steroid. It is more helpful than a steroid, but it must be given as a shot, once daily for the first few weeks, then slowly tapered until it is stopped.
Steroids and ACTH have side effects but can be safely managed:
- Increased appetite
- High blood pressure
- Kidney problems
- Increased risk of infection
- Increased risk for bleeding in the stomach
- Puffiness from extra fluid. This can make the face look fuller
- High glucose level in the blood
Some children will need seizure medicine such as Vigabatrin®, Depakote® or Topamax® along with the steroids.
What is the prognosis?
The future course of the disorder and the child’s development depend on the cause of the seizures. Even with treatment of the spasms, most children will have delays in speech and motor skills. Many go on to have other forms of epilepsy. When a child has developed in a normal way before the onset of the spasms, there is a better chance of
- stopping the seizures with medicine.
- normal or mildly delayed speech and motor skills and learning.
Many doctors feel that the quicker the seizures are treated and controlled, the better the results will be. Children who are not treated often have frequent spasms for many years.
Some children may later have autism or a syndrome called Lennox-Gastaut.
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Last Updated: 01/28/2013
Copyright © 01/28/2013 University of Wisconsin Hospitals and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6925
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