World West Syndrome Day Date in the current year: April 10, 2024

Contrary to a common misconception, epilepsy is not a single disease; it is an umbrella term for a group of neurological disorders characterized by recurring epileptic seizures that are caused by an abnormal, excessive, and synchronized electrical discharge in the neurons. Some of these disorders are relatively common, while others are uncommon-to-rare; West syndrome falls into the second category.

West syndrome was named after English surgeon and apothecary William James West, who first described in an 1841 article published by The Lancet. The object of the description was West’s third son, James Edwin, who began to have seizures at four months of age. West himself referred to the seizures as “salaam ticks” because of their similarity to the Muslim ceremonial greeting.

West syndrome is sometimes referred to as infantile spasms syndrome, but infantile spasms (epileptic seizures in infants) are just one of its three main symptoms, the other two being developmental regression and EEG with a characteristic layout of hypsarrhythmia (chaotic and disorganized brain electrical activity with no visible pattern). For diagnosis, at least two of the three symptoms must be present.

Infantile spasms usually start between the ages of four and eight months, although in some cases the onset may be as early as two months of age or as late as between two to four years of age. They can be caused by a number of conditions such as brain injury, developmental abnormalities, metabolic disorder, or genetic mutations. In some cases, no clear cause of infantile spasms can be identified (idiopathic epilepsy).

The main goal of treatment for West syndrome is to eliminate the seizures and resolve the pathognomonic EEG pattern. Treatment depends on the underlying cause and may include medications, surgery (if the spasms are caused by a brain lesion), ketogenic diet, and speech and/or occupational therapy if regression or developmental delays are present. Medical professionals typically develop a personalized seizure management plan, working closely with their patients’ families.

The long-term prognosis for children affected by West syndrome depends on the underlying cause of the condition and its form, the extent of regression/developmental delays, and timeliness of diagnosis. Some children may do well once they start receiving treatment, but most experience severe physical and cognitive impairments. About 5% of children with West syndrome do not survive beyond five years of age.

The origin of World West Syndrome Day is unclear but the observance seems to have originated in Spain or another Spanish-language country. The best ways to get involved include learning more about West syndrome, donating to an organization that helps patients affected by the syndrome and their families or funds research, and spreading the world on social media with the hashtags #WorldWestSyndromeDay, #WestSyndromeDay and #DíaMundialDelSíndromeDeWest.