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Epilepsy / Syndromes

What is West Syndrome?

What is West Syndrome?

Epileptic spasms, also known as infantile spasms, juvenile spasms, or West syndrome[ is an uncommon-to-rare epileptic disorder in infants, children and adults. 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 (1840–1860).  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. West syndrome in modern usage is the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia), and developmental regression[4] – although the international definition requires only two out of these three elements.

The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal.

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