West Syndrome

Infancy is a very tender age. The infant goes through many stages of physical and cognitive development. They are growing rapidly, and there are many milestones they need to go through. These milestones and developmental changes are very important. Sometimes, there can be incidences where some children display atypical muscle movements and epileptic episodes. What are these movements? What are the causes of these movements, and how can we treat them?

What is West syndrome?

William James West was the one who discovered a condition in infants that was marked by marked spasms in the body, delays in developmental milestones, and irregular brain wave patterns. This condition is therefore named in his memory and referred to as the "West Syndrome." Three major features characterize this condition.

  • Spasms − Epileptic spasms consist of sudden and acute convulsions of the whole body or several parts. This occurs for a split second. Here the baby bents down from the waist, and the limbs stiffen. The legs also, in most cases, bend. This is most commonly referred to as a "jackknife seizure." Alternatively, some infants may arch their backs, bob their heads, or cross their arms over their bodies like hugging.

  • Developmental Issues − The child can face developmental regression or miss developmental milestones.

  • Hypsarrhythmia − A child with West Syndrome displays highly irregular and chaotic brain wave patterns.

In order to be diagnosed with West Syndrome, all the above three conditions must be present.

Additionally, the child can display other symptoms. Autism and other neurological conditions like cerebral palsy could also exist. Most children experience cognitive and intellectual disabilities for the same reason that causes these spasms. Other major features are −

  • Poor coordination in the child

  • Defects in cognitive abilities.

  • Age of onset

Infant begins to experience these symptoms when they are four to eight months old. West syndrome's onset of infantile spasms normally occurs between 4 and 8 months of age. The youngster may experience brief forward bending of the body and stiffening of the arms and legs during the one− to two−second−long seizures. Some infants extend their arms and legs while arching their backs. The transitions between waking and sleeping and right after eating are when the spasms happen most frequently.

What does Cause West Syndrome?

West Syndrome can be brought on by almost any illness that might cause brain injury. The disorder is known as cryptogenic West syndrome, when a cause is occasionally hypothesized but not proven. A genetic mutation or metabolic issue can also lead to this condition. Prenatal causes that lead to structural abnormalities in the fetus can also lead to this condition.

How is this condition Detected?

The physician does a thorough physical checkup, followed by a medical case history of spasms and other symptoms. Here are some ways to help arrive at a detailed diagnosis.

A child or an infant who experiences spasms is enough of an indicator for parents to visit a doctor. This leads to doctors conducting various imaging tests and scans to find the root of the problem. Several lab findings are carried out to dig deep into the problem. An electroencephalogram test is performed on the child at various sleep and wake cycles. This helps to find irregular brain patterns. CT scans and MRI imaging help to find structural abnormalities. Blood tests help to find out any metabolic abnormalities. Genetic tests help discover genetic irregularities and any history of epilepsy in the family.

What is the Treatment for West Syndrome?

The treatment can be classified into three parts.

  • Drug therapy or psychopharmacological interventions.

  • Surgery.

  • Behavioral intervention and emotional interventions.

Unfortunately, this condition cannot be treated completely. Additionally, the exact cause of this condition is still unknown, which makes the treatment options very different depending on the cause of the condition. Hormones like adrenocorticotropic, corticosteroids, anticonvulsants, and antiepileptics have shown great effectiveness. Natural corticotrophins have relatively fewer side effects compared to synthetic ones. Healthcare professionals may advise surgical removal of damaged brain tissue if drugs are insufficiently successful, particularly in cases involving malformation or tuberous sclerosis complex. This kind of surgery is frequently used to treat various types of epilepsy and typically yields positive results.

The primary goal of the treatment involves controlling the erratic brain wave patterns and getting hold of the seizures. A good treatment approach must include training and assistance for family members, and parents and other caregivers must know how to recognize and handle seizures. Physical treatment, occupational therapy, and speech therapy may be beneficial if the child exhibits developmental delays or regression.

Future Outcomes for Children with West Syndrome

In one of the reports that tried to determine the long−term quality of life of patients with West syndrome, it was found that nearly two−thirds of children with this condition lived an acceptably stable life. This condition must be diagnosed early to provide quality treatment that can prolong life and improve quality of life. When children show normal development before the onset of the spasms, the treatment outcome is better, and they recover quickly. Children without any structural abnormalities also respond well to treatment.

Most infantile spasms disappear by the middle of infancy. While some of these individuals experience a severe form of epilepsy, more than half of children with West syndrome will later experience different seizure forms.

Psychological Impact on the Infant and the Caregivers of West Syndrome

A child with this condition may have to face implications at many levels. Also, living with this condition will require adjustments made by the caregiver. Everything has to be taken care of, from finances to life planning to provide the infant with the best care possible. Many adjustments will have to be made by the caregivers.

The child will require special care. This can get overwhelming for caregivers at times. In many cases, this can cause fear, guilt, panic, and even stigma. This is even though nothing can be done to prevent the condition. Education and awareness are the best ways to cope with this condition. A support group, constant contact with the teachers and healthcare team, and self−care practices can help caregivers cope with this condition.


West syndrome is a rare and uncommon condition usually found in infants and children that causes involuntary and troubling spasms, erratic brain wave patterns, and developmental delays in a child. The causes of this condition can be classified as genetic, metabolic, or cryptogenic. Other reasons that can lead to this condition include a brain injury or structural dysfunction in the brain. The treatment is focused on symptom management as there is no permanent cure for this condition yet. This condition can psychologically and emotionally impact the child and the caregivers. However, a holistic plan and management of this condition can prolong the child's life and give them a better quality of life.

Updated on: 13-Dec-2022


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