West’s Syndrome

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What is West’s Syndrome?

The average onset generally begins at an average age of 6 months. Symptoms may include a pattern of an infant bending forward with a stiffening of the body, including the arms and the legs. Each episode generally last for a few seconds. These episodes typically occurs I clusters that can last up to 20 minutes.

Causes
  • Trauma
  • Brain malfunctions
  • Infections
  • Down syndrome
  • Tuberous sclerosis complex
Statistics and Facts

West syndrome occurs 1 in every 2,000 to 6,000 live births, which peak between the ages of 4 and 7 months old. 90 percent of incidences occur before the age of 1 year. West syndrome accounts for approximately 30 percent of incidences involving infants. Boys are more likely to be affected than girls.

Other Know Names
  • Generalized Flexion Epilepsy
  • Infantile Epileptic Encephalopathy
  • Infantile Myoclonic Encephalopathy
  • Jackknife Convulsions
  • Massive Myoclonia
  • Infantile Spasms
  • Salaam Spasms
History

Initially discovered in the 1840’s by Dr. William James West when he noticed his own son, James E. West showed the characteristics of “bobbings” that caused a complete heaving of the head towards his knees and then immediately relaxing to the upright position. Dr. West originally coined the phenomena as “Salaam Tics”.

Diagnosis and Testing
  • Electroencephalography (EEG)
  • A physical and neurological exam
  • Computer Tomography (CT)
  • Magnetic resonance Imaging (MRI)
  • Blood Test
  • Urine Test
  • Wood Lamp (for the purpose determining if tuberous sclerosis is a possible diagnosis)
Treatment
  • Steroid therapy by injection into a muscle or prednisone by mouth
  • A seizure medication called Sabril
  • Ketogenic diet

 

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions.

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