11 Oct 2012 : West Syndrome
THE ROAD AHEAD
West Syndrome
11 October 2012
Author : Aswinkumar

Introduction
It is very recently that we noticed the incidence of West syndrome in Adarsh. It so happened that with the arrival  of Dr.Neena Shilen, a Developmental Pediatrcian, we are now able to identify specific  developmental as well as genetical disorders, which otherwise remained undetected and therefore not  treated. One of such developmental disorder is West syndrome which is described below in detail. It is a typical epilepsy   associated developmental disorder which can be effectively treated medically as well as therapeutically. Medical treatment with Sabril (vigabatrin) found very effective in controlling severe epilepsy in West syndrome. Therapeutic measures provided simultaneously with the medical treatment to compensate delay in motor development. We now have 4 children in Adarsh ,who are under treatment of Dr.Neena. and who are in our Early Intervention Section.

Definition
West syndrome or West's Syndrome is an uncommon to rare epileptic disorder in infants. It is named after the English physician, William James West (1793–1848), who first described it in an article published in The Lancet in 1841. The original case actually described his own son, James Edwin West (1820–1840). Other names for it are "Generalized Flexion Epilepsy", "Infantile Epileptic Encephalopathy", "Infantile Myoclonic Encephalopathy", "jackknife convulsions", "Massive Myoclonia" and "Salaam spasms". The term "infantile spasms" can be used to describe the specific seizure manifestation in the syndrome, but is also used as a synonym for the syndrome itself. The syndrome is age-related, generally occurring between the third and the twelfth month of a child , generally manifesting around the fifth month. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal.

Prevalence
Prevalence is around 1:3200 to 1:3500 of live births. Statistically, boys are more likely to be affected than girls

Causes
It is still unknown which bio-chemical mechanisms lead to the occurrence of West syndrome. It is conjectured that it is a malfunction of neurotransmitter function, or more precisely, a malfunction in the regulation of the GABA transmission process. Another possibility being researched is a hyper-production of the Corticotropin-releasing hormone (CRH). It is possible that more than one factor is involved. 

Treatment
As the condition occurs due to the involvement of brain damage, there is no medical treatment. But the associated condition like Epilepsy can be treated using medicines. Once the medical condition is stable, child can be provided with adequate therapies and training to make her/him independent in adult life.

Tags: West syndrome- a bri


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