West syndrome is a little-known condition that is still the subject of deeper studies to accurately determine its causes and forms of treatment. Its first mention occurred in 1841 by the physician Willian James West, who therefore gave it his name. Through a letter to a well-known newspaper of the time, Dr. West presented to the world the problem of constant and daily spasms that his son suffered from. Due to the number of daily attacks, his son developed intellectual disability despite all the controls and treatments started at the time.
Considered a neurological syndrome, it began to be studied more in depth in 1949 by monitoring babies who had the seizures. During this period, it was called infantile epileptic spasms and divided into two groups that indicate the causes. The cryptogenic group, which is of unknown cause, where the baby is born completely normal until the first occurrence of spasms; and the symptomatic group, where abnormal neuropsychomotor development is already present, as well as other changes confirmed by neurological exams or imaging tests such as magnetic resonance imaging or computed tomography.
Over the years and through various studies, by 1991 more hypotheses and treatment results had been reached. In 80% of the analyzed cases it was identified as a secondary syndrome, which depends on other problems such as neonatal anoxia, toxoplasmosis, birth trauma and encephalitis, Bourneville tuberous sclerosis, and Aicardi syndrome in order to occur. Another highly relevant fact that was pointed out is that there are more cases in baby boys than in girls. It affects 2 boys for every 1 girl, and the cases occur within the first year of life, mainly between the 4th and 7th month of life.
Symptoms and Treatment of West Syndrome
The main symptoms of West syndrome are constant muscle spasms that can suddenly begin as sudden neck movements to spasms affecting the entire body, so they are divided into flexor, extensor, or mixed spasms, which determine the type of spasms the baby experiences. The most common are the mixed type, where the child has spasms throughout the body, moving the neck, trunk, and other limbs.
Children who suffer from West Syndrome usually present intellectual disability and psychological changes in up to 70% of cases, but the chances can be reduced when the condition is properly and early treated. Few children with the syndrome have normal cognitive development; however, the seizures tend to decrease or disappear completely with age, around 4 or 5 years old.
To conclude the diagnosis of West Syndrome it is necessary not only to perform a physical exam and check the symptoms, but to analyze the entire clinical history and occurrences since birth. The most valuable exam requested to confirm the diagnosis is the electroencephalogram, which will display all the records of brain activity. Other tests to aid the understanding of the syndrome, such as a complete blood count, liver function tests, urine test and kidney function tests, are also important.
Once the diagnosis is established, the focus of treatment for West Syndrome is to minimize the number of daily spasms with as few medications as possible, aiming that the child does not suffer from the side effects of treatment drugs. Treatment is usually based on antiepileptic drugs and follow-up with physical therapists to minimize the risk of bodily deformities, as well as to lessen the sequelae caused by the spasms.
See Also: Down Syndrome – Types and How to Deal
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