West syndrome - Wikipedia, the free encyclopediahttp://en.wikipedia.org/w/index.php?title=West_syndrome&printable=yeof 601/04/2008 12:36 PM
There are known cases in which the spasms occurred for the first time after vaccination againstMeasles, Mumps and Rubella or Tetanus, Pertussis, Diphtheria, Polio, Hepatitis B and Haemophilusinfluenzae Type B. However, stress of any kind is a common trigger for seizures, and theimmunization occurs during the time-frame in which many typical cases become conspicuous. Thereis no causal relationship between immunization and West syndrome, since in many cases Westsyndrome is not recognized as iatrogenic.
West syndrome in Down syndrome babies
On average, West syndrome appears in 1 to 5 per 100 children with Down's syndrome as babies. Whereasthis form of epilepsy is relatively difficult to treat in children who do not have the chromosomal differencesinvolved in Down's syndrome, the syndrome often affects those who do far more mildly and they often reactbetter to medication. The German Down Syndrom InfoCenter (http://www.ds-infocenter.de/) noted in 2003that what was normally a serious epilepsy was in such cases often a relatively benign one.EEG records for Down's syndrome children are often more symmetrical with fewer unusual findings.Although not all children can become entirely free from attacks with medication, children with Down'ssyndrome are less likely to go on to develop Lennox-Gastaut syndrome or other forms of epilepsy than thosewithout additional hereditary material on the 21st chromosome. The reason why it is easier to treat childrenwith Down's syndrome is not known.
When a direct cause cannot be determined but the children has other neurological disorder, the case isreferred to as
West syndrome, where an underlying cause is most likely but even with ourmodern means cannot be detected.Sometimes multiple children within the same family develop West syndrome. In this case it is also referredto as
, in which genetic and sometimes hereditary influences play a role. There are known casesin which West syndrome appears in successive generations in boys; this has to do with X-chromosomalheredity.
Occasionally the syndrome is referred to as
West syndrome, when a cause cannot be determined.Important diagnostic criteria are:Regular development until the onset of the attacks or before the beginning of the therapyno pathological findings in neurological or neuroradiological studiesno evidence of a trigger for the spasmsThose are becoming rare due to modern medicine.
The epileptic seizures which can be observed in infants with West syndrome fall into three categories.Typically, the following triad of attack types appears; while the three types usually appear simultaneously,they also can occur independently of each other:
: Sudden, severe myoclonic convulsions of the entire body or several parts of thebody in split seconds, and the legs in particular are bent (flexor muscle convulsions here are generallymore severe than extensor ones).
: Convulsions of the throat and neck flexor muscles, during which the chin is fitfully