GC: n
S: NORD – https://rarediseases.org/rare-diseases/west-syndrome/ (last access: 6 November 2015); MD – http://emedicine.medscape.com/article/1176431-overview (last access: 6 November 2015).
N: 1. The syndrome’s namesake, Dr W J West, gave the first detailed description of infantile spasms, which occurred in his own child.
2. In a letter to the editor of The Lancet in 1841, West described the events as “bobbings” that “cause a complete heaving of the head forward towards his knees, and then immediately relaxing into the upright position … These bowings and relaxings would be repeated alternately at intervals of a few seconds, and repeated from 10 to 20 or more times at each attack, which would not continue more than 2 or 3 minutes; he sometimes has 2, 3 or more attacks in the day.”
3. The eponym West syndrome was created in the early 1960s by Drs. Gastaut, Poirier, and Pampiglione.
4. West syndrome is a type of epilepsy characterized by spasms, abnormal brain wave patterns called hypsarrhythmia and sometimes mental retardation. The spasms that occur may range from violent jackknife or “salaam” movements where the whole body bends in half, or they may be no more than a mild twitching of the shoulder or eye changes.
5. The most common disorder responsible for West syndrome is tuberous sclerosis complex. (TSC). TSC is an autosomal dominant genetic condition associated with seizures, eye, heart and kidney tumors and skin findings.
S: 1, 2 & 3 MD – http://emedicine.medscape.com/article/1176431-overview (last access: 6 November 2015). 4 & 5. NORD – https://rarediseases.org/rare-diseases/west-syndrome/ (last access: 6 November 2015).
SYN: epileptic spasms (context), infantile spasms (context).
S: NORD – https://rarediseases.org/rare-diseases/west-syndrome/ (last access: 6 November 2015)
