West Syndrome – Symptoms, Treatment, Life Expectancy

What is West Syndrome?

West syndrome is referred as infantile spasms that are actually a type of epilepsy. In this condition, the patient experiences a characteristic pattern of seizures. In some cases, the pattern of seizures is like forward to backward bowing due to which the syndrome is often called ‘salaam spasms.’ In 1841, Dr. West first mentioned about the syndrome after he detected the condition in his four month old child.

According to medical records, this type of syndrome is diagnosed in one among 3000 children.  The condition of west syndrome generally arises due to the combination of three different conditions of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypoarrhythmia, and mental retardation.

The condition of west syndrome is more prevent in male child as compare to the female child. The condition is characterized by the occurrence of sudden axial flexion and psychomotor retardation. In some cases, ocular deviation has also been noted. The child suffering from the condition of west syndrome is characterized by abnormal crying pattern and contraction or stiffness of limbs.

The most common reason behind the condition of west syndrome can be Bourneville disease, sequelae of ischemia, meningoencephalitis or genetic deformity caused by the deletion or mutation in the genetic sequence.

Symptoms of West syndrome

In most of the children, the condition of west syndrome arises in the first year of life. Medical data indicates that the chances of occurrence of the syndrome are found to be higher during the period between third and eight months.

In the beginning, the condition does not occur in the cluster that makes the diagnosis of the syndrome difficult.  Sometimes the symptoms of the syndrome are misunderstood by the physicians. It has been noted that during the attack, the child’s body stiffens and often arms or legs flung outward.

During the spasm, there is a pause for a second between each episode. Most of the babies suffering from the syndrome become irritable, and the development gets affected. In few cases, doctors have noted that the infant’s capability of vision gets affected, and they behave as if they cannot see anything. The situation normalizes one the spasm is controlled with improvement in EEG.

Diagnosis of West Syndrome

The diagnosis of west syndrome is little difficult as in the initial stage the condition is confused with other common behavioral problem. Physicians use typical features and EEG patterns to diagnose the presence of the syndrome.

The infants suffering from the west syndrome shows abnormal EEG patterns. However, an abnormality in EEG pattern is also visible when the baby is asleep during the night. In most of the cases, the cause behind the occurrence of west syndrome is unknown. Doctors also recommend several other tests for the infant apart from EEG for the confirmation of west syndrome in a child.

These tests include a blood test, urine test, brain scans, spinal cord test, etc. These tests help in knowing the cause behind the occurrence of west syndrome. The brain scan includes magnetic resonance imaging.

Treatment of West Syndrome

The condition of west syndrome that occurs in infants is curable. The condition can be corrected by proper medication. Steroids or vigabatrin are used to treat the condition of west syndrome. In some cases, doctors also prescribe Nitrazepam and sodium valproate. In most of the cases, steroids are recommended by the doctors.

Prednisolone is given via oral dose( mouth); hydrocortisone is given by injection  and tetracosactide  is given by intra muscular injevtion. Doctors generally prescribe steroids in limited quantity, but it must be given with care as it causes side effects in infants.

Thus to limit the side effects of the steroids, doctors prescribe the medication in limited quantity for a limited period of time. Infant with tuberous sclerosis is given Sabril by the doctor as it proves to be effective. If localized cerebral lesions are detected, then doctors might recommend surgical intervention to treat the condition of west syndrome. Treatment should begin as early as possible to limit the cognitive deficit caused by the epilepsy.

Prognosis and Life expectancy of West Syndrome

The condition of west syndrome is curable with proper medication and in some cases, infants respond to the treatment at early stage. However, in few cases, the treatment does not prove to be helpful in the beginning.

Medical report indicates that in some cases the condition of west syndrome relapses in early childhood after being treated in infants. The reoccurrence of the condition in early childhood is linked with epilepsy and called Lennox-Gastaut syndrome. In such conditions, the child shows symptoms like difficulty in learning and understanding things. The symptoms can be mild or severe depending on the degree of Lennox-Gastaut syndrome.

The premature death rate of infant in west syndrome is found to be 5-31 %. The long term prognosis of the condition of west syndrome is poor. In few children, the prolonged condition of west syndrome causes the increased probability of autism. Pediatrics believes that the children in whom seizures are controlled at early stage, the chances of proper treatment are bright. The prognosis of the condition of west syndrome depends on degree and speed of effective treatment.