West Syndrome Topics

• myoclonic encephalopathy
• corticotropin releasing hormone
• seizure type
• neurotransmitter function
• infantile spasms
• brain development
• english physician
• crh
• william james
• heredity
• lancet
• convulsions
• hypotheses
• prognosis
• epilepsy
• medications
• generations

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West Syndrome, West Syndrome

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. Other names for it are "Generalized Flexion Epilepsy", "Infantile Epileptic Encephalopathy", "Infantile Myoclonic Encephalopathy", "jackknife convulsions", "Massive Myoclonia" and "Salaam spasms". The name "infantile spasms" is used to describe both the seizure type and the syndrome, although not all infants with infantile spasms have West syndrome.

It is conjectured that it is a malfunction of the 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. Both hypotheses are supported by the effect of certain medications used to treat West syndrome.

There are known cases in which West syndrome appears in successive generations in boys; this has to do with X-chromosomal heredity.

In general it can be said that the prognosis is worse when the patient does not react as well to therapy and the epileptic over-activity in the brain continues. Treatment differs in each individual case and depends on the cause of the West syndrome (etiological classification) and the state of brain development at the time of the damage.

Source: Wikipedia > West Syndrome