Infantile epileptic spasms syndrome is a rare seizure disorder, previously referred to as West Syndrome. This syndrome mainly affects those in their first year of life, typically between 4-8 months of age but the onset and diagnosis can be outside these parameters.
The spasms (seizures) typically happen during wakefulness and look like a sudden bending forward of the body with stiffening of the arms and legs. These are brief – and over in seconds. Some infants arch their backs as they extend their arms and legs. The spasms tend to cluster when the infant wakes up, and they can have a few, to up to hundreds of seizures per day.
Cause
There are many different causes of infantile spasms, which can happen before or after birth.
Diagnosis
Infants with this type of epilepsy will present with:
- Seizures known as infantile or epileptic spasms
- Developmental delays and cognitive problems or regression
- A specific brain wave pattern on EEG called hypsarrhythmia
Early diagnosis is important for infantile epileptic spasms because early treatment affects prognosis and outcomes.
Treatment
Treatment is firstly commenced with specific medications known to act upon these seizures. These medications can be very effective but also have significant adverse side effects, which your doctor will discuss.
When these medical treatments do not improve the epileptic spasms, doctors may consider trying the ketogenic diet as well.
If there are brain lesions causing the spasms, the seizures may decrease if the lesions are removed by surgery.
It is very important for treatment to start as soon as possible. If spasms (and hypsarrhythmia) continue, they can have negative effects on the developing brain.
Prognosis
The outlook varies, and mostly depends on what is causing the epileptic spasms.
Many infants have neurological impairment before spasms begin and will have intellectual disabilities later in life.
Infantile spasms usually stop by age 4 or 5 but many children will develop other types of seizures or syndromes, like Lennox-Gastaut syndrome, which may not respond well to medication.
Outcomes may be better if:
- Treatment is started quickly after the epileptic spasms start
- There is normal development before the spasms started
- There is no known cause for their spasms found.
