Magazine article The Exceptional Parent

Infantile Spasms-A Seizure Disorder of Infancy

Magazine article The Exceptional Parent

Infantile Spasms-A Seizure Disorder of Infancy

Article excerpt

Infantile spasms (IS) are rare, found primarily in children under the age of one year. The condition usually begins at 3 to 7 months of age and has a very specific EEG or brain wave pattern. IS occurs in approximately 28 to 30 percent of infants with epilepsy. Causes include tuberous sclerosis, brain malformations such as lissencephaly, cerebral hypoxic events, and such head traumas as subdural hematomas.

MP was the second child born to a healthy 26-year-old woman. He did well in the first months of life. One morning, when MP was 5 months, he developed cold symptoms; he awoke fussy and cranky and did not feed well. He was dropped off at daycare as usual. Shortly thereafter, his condition worsened and he was rushed to a children's hospital. He was examined by a pediatrician and pediatric neurologist. It appeared as if MP had "shaken baby syndrome" with resultant significant brain injuries. MP was admitted to the pediatric ICU and placed into a barbiturate coma. This treatment shut down most of his brain activity and was designed to reduce stress on the injured brain, in the hope that over time there would be significant recovery from the injuries.

MP spent two weeks in the artificial coma; the barbiturate treatment was gradually withdrawn and he slowly regained consciousness. A detailed neurological examination found weakness of his left side, significant loss of vision and reduced tone in all four extremities. More important, MP began to show signs of epileptic (seizure) activity. Initially he had brief periods of reduced responsiveness with subtle abnormal eye movements, but he soon began to have contractions of his body at the waist. A pediatric neurologist evaluated the boy with an EEG and a brain imaging study. MP was started on phenobarbital, an anti-seizure medication. When he continued to show evidence of seizures, the medication was switched to clonazepam.

Infants with IS are frequently thought to have colic, since the disorder often begins with subtle symptoms and crying during and after the attacks. Then the child develops typical patterns of sudden flexion (bending forward) in a tonic (stiffening) fashion of the body, arms and legs. A minority of children with IS have extensor episodes (in which the body arches backwards). In the majority of children the spasms can be mixed--sometimes flexion and sometimes extensor. While most episodes are symmetric, some children exhibit marked differences between the sides of their bodies and thus may have predominantly right- or left-sided symptoms. This usually is the result of asymmetric brain abnormalities.

The characteristic attack starts with a sudden onset of a tonic (stiffening) seizure that is bilateral and symmetric. Spasms may vary, from massive contractions of the large muscle groups to contractions of only the neck and abdominal muscles. Children may have a mixture of spasms. The attacks tend to occur in clusters of 5 to 10 spasms; each may last up to a minute, with brief intervals between each spasm. A cry may precede or follow the seizure. The seizure may take place during sleep or while awake, but tends to occur most often when the child is drowsy or immediately upon awakening. Afterwards, the child is often irritable. Increasing frequency of episodes is likely to slow development, which may regress until the spasms are controlled.

During the next few weeks, MP had at least five seizures per day and became increasingly irritable, with frequent and prolonged crying spells, interrupted sleep, and decreased appetite. MP's development seemed to stop and then regress, as he became less alert. A subsequent evaluation by his pediatric neurologist confirmed these observations. A repeat EEG demonstrated characteristic hypsarrhythmia (disruption of the normal brain wave pattern). An MRI scan of the brain was reviewed and found to show reduced brain size on the right, with significant damage in the occipital region (area associated with vision found in the back of the brain). …

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