Tuesday, April 28, 2015

West Syndrome





          West Syndrome often referred as Infantile Spasm (IS) is named after it was 1st described by Dr. William James West in 1840s when he observed the spasms in his 4-month kid.


          Spasms means sudden jerks which one can't control followed by stiffening of the muscles. Infantile Spasms are characterized by sudden flinging of arms in outward direction, knees pulled up and body bending in forward direction. Sometimes the head is thrown back as body and legs stiffen in straight position. Each spasm lasts only for a second or 2 but it comes in clusters; which means once the child gets a spasm it lasts for few seconds then it is repeated again after few moments. Infantile Spasms is common after waking up and rarely occurs in sleep.

          Infants between 3 to 12 months of age are usually affected by infantile spasms. Infants having suffered any kind of brain injury or brain damage are ones who are usually affected. But in some cases, infants who are developing normally too suffer from West Syndrome.

          Usually doctor recommends an EEG for infants with infantile spasms. EEG with unusual pattern called hypsarrhythmia pattern is helpful in confirming the diagnosis.


Hypsarrhythmia is an abnormal pattern, consisting of high amplitude and irregular waves and spikes in a background of chaotic and disorganized activity seen on EEG, and frequently encountered in an infant diagnosed with infantile spasms, although it can be found in other conditions. In simpler terms, it is very chaotic and disorganized brain electrical activity with no recognizable pattern, whereas a normal EEG shows clear separation between each signal and visible pattern.

          Children with West syndrome usually show developmental delay, have vision problems, and in children who are normal, show losing of skills which they have already developed or mastered.


          Like other epileptic disorders, infantile spasms are also treated with anti-epileptic medications. 



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