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.
