Is it IS?
Infantile spasms is a kind of epilepsy. It usually begins in children who are less than one year old.
Other names for infantile spasms include:
- West syndrome
- Epileptic spasms
- Infantile spasms syndrome
Children with infantile spasms typically have clusters of short seizures. They often exhibit developmental problems.
A diagnosis of infantile spasms can be made by looking at a child’s movements and performing an electroencephalogram (EEG) to evaluate their brainwaves. Diagnosing and treating infantile spasms is an urgent matter. If not treated, the disorder can permanently harm development. The main treatments include hormonal therapy and vigabatrin, an anti-seizure medicine.
Infantile spasms may occur in children without prior medical problems. However, most children get infantile spasms after the diagnosis of another brain disorder. The long-term outcome for children with infantile spasms varies a lot. It depends on the cause of the disorder. Some children with rapid diagnosis and successful treatment will have a normal health outcome. However, many children will have poor outcomes.
Poor outcomes include:
- Continued seizures and other forms of epilepsy
- Developmental problems
Infantile spasms is a severe form of epilepsy. The term “epilepsy” refers to diseases in which a patient has recurrent seizures. A seizure is an event in which a patient has a change in behavior, such as shaking or loss of awareness. Seizures are caused by abnormal electrical activity in the brain. Infantile spasms is a severe form of epilepsy. In children with infantile spasms, seizures are also called “spasms.” Infantile spasms is considered severe because of the developmental problems that often occur with the disease.
SIGNS AND SYMPTOMS
The symptoms of infantile spasms can vary.
In some children, infantile spasms will begin without any prior diagnoses or developmental problems. However, in most children, infantile spasms will begin after the diagnosis of another brain disorder. For instance, it can begin after a stroke. Or it can begin after the onset of another form of epilepsy. Some children will already have severe developmental problems when infantile spasms begin.
Typical features of infantile spasms include the following:
Each spasm (seizure) lasts about 1 second. It often looks like the child is briefly startled. The spasms typically repeat in a cluster. In other words, a single spasm will happen every 5 to 15 seconds over a total span of several minutes.
The movement during a spasm usually includes a quick wide-eyed stare, dropping of the head, and raising of the shoulders and arms. The entire seizure lasts about 1 second. However, there are many possible movements that can happen with a spasm. It is important to note that in an individual child, all of the spasms usually look identical. Examples may be seen here.
Clusters of spasms may occur once per day. They may occur several times per day. Clusters are most common several minutes after waking. In most cases, there is no warning that spasms are about to happen. However, some parents report that they can predict a cluster is about to happen. They notice a change in their child’s behavior, such as being oddly vigilant or scared. During a cluster of spasms, some children will cry momentarily after each spasm. Others may seem distressed throughout a cluster. Still, others will briefly smile or giggle after each spasm. When a cluster is over, children usually go back to their normal behavior.
Around the time that infantile spasms begin, it is common to observe new developmental problems. These may include a loss of specific “skills.” A child may stop babbling, rolling, sitting, or reaching for objects. Many parents will report that their child seems less alert or engaged. There may be a loss of visual fixation and tracking.
Other types of seizures may occur before or after the onset of infantile spasms. In some cases, multiple types of seizures can coexist with infantile spasms.
This is a chaotic brain-wave abnormality. It can be detected with a lab test. Hypsarrhythmia can strongly suggest that a child has infantile spasms. However, not all children with infantile spasms have this condition.
There are hundreds of possible causes of infantile spasms. Almost any disease or disorder of the brain can cause it.
Three known types of causes can lead to infantile spasms:
- Abnormalities in the structure of the brain that are present before birth
- Acquired injuries to the brain after birth
- Genetic disorders
The most common causes of infantile spasms include:
- Tuberous sclerosis complex
- Hypoxic-ischemic encephalopathy
- Down syndrome, also called trisomy 21
- Stroke or bleeding in the brain – read more about perinatal stroke and childhood stroke
- Brain malformations such as focal cortical dysplasia, polymicrogyria, hemimegalencephaly, and lissencephaly
- CDKL5 deficiency disorder
- Duplication 15q syndrome, or Dup15q
- Aicardi syndrome
Some children have normal development before infantile spasms, and no cause is found. These children are termed cryptogenic.
The long-term outlook for children with infantile spasms varies a lot.
On one hand, most children with infantile spasms do not do well long-term. There are two main reasons for this.
Treatment of infantile spasms is often only temporarily successful. Many children relapse.
Infantile spasms often occurs in children who already have severe brain diseases. These other diseases can carry their own risks. They may cause developmental problems. They may also cause other forms of epilepsy.
On the other hand, normal development and a normal life is possible for some children with infantile spasms. Overall, about 20% of children with infantile spasms will develop normally. A child can develop normally if:
- Infantile spasms is treated quickly and successfully
- A child does not have another severe brain disorder