West syndrome, which is also known as infantile spasm, is a rare neurologic syndrome which is often seen in infants. The symptoms of this rare syndrome manifests during 3 to 12 months with an episode of epileptic spasm which occurs due to be uncontrolled brain activity. Such brain activity lasts for few seconds, however, is seen in clusters lasting for about 5 to 15 minutes. Such episodes are usually seen during awakening or feeding time, with sudden involuntary contractions of the head, neck, and trunk and/or uncontrolled extension of the legs and/or arms. However, the duration and intensity of the episode along with the muscle group affected varies on infants.
Triadic symptoms: Abnormal EEG (brain wave) pattern, infantile spasms and intellectual disability.
Along with the triadic features, other symptoms like crankiness, regression of the attained skills, change in sleep patterns and delayed developmental pattern are also common among them.
Causes of WS: The major cause of WS occur due to any brain insults such as trauma, brain malformations (ex. cortical dysplasia), infections, genetic abnormalities, metabolic disease etc. However among 5%, the cause is idiopathic.
Types of WS: According to the cause, there are 3 types of WS
- Symptomatic: The reason is due to any known brain disorder.
- Cryptogenic: The cause for the WS is unknown but due to brain insult
- Idiopathic: When a sudden regression is noticed, without any brain insult
Diagnosis:
WS are diagnosed with:
- Electroencephalography (EEG) which records the electrical activity of the brain
- Brain scans such as CT and MRI scan
- Blood tests, urine tests and lumbar puncture
- Genetic testing
Once the child is diagnosed with WS, it is important for them to provide rehabilitation services like speech therapy, occupational therapy and other services, as they have developmental delay. Thus, the role of early and long term multidisciplinary treatments are crucial for them. These programs helps them in attaining their developmental milestones and in improving the outcome for the child, families and the society. Thereby helps in leading an independent life.
