West syndrome in Infancy

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An infantile spasm is rare and complex form of epilepsy. It was first described by Dr W J West in the 1840s so the name is given West Syndrome. It is age related and occurs in first year of life mostly between 3-8 months babies. Electroencephalography (EEG) testing shows a specific pattern which is called as hypsarrhythmia. Spams may occur in clusters per day and it lasts for few second. After age of 5 infantile spasms generally vanishes but other types seizure may occur. It generally occurs after feeding or awakening and rare during sleep.

Alternatives names for West Syndrome

Infantile Epileptic Encephalopathy, Jackknife convulsions, Salaam spasms, Generalized Flexion Epilepsy, Infantile Myoclonic Encephalopathy, Massive Myoclonia and Infantile spasm are other names of west syndrome.

Causes of West syndrome

• Infantile spasms can be classified as symptomatic, cryptogenic or idiopathic.
  
• If an identifiable factor is responsible then it is called as symptomatic west syndrome
  
• When a direct cause not identified but the child having other neurological disorder is referred as cryptogenic west syndrome.
  
• When no cause is determined then it is called as idiopathic west syndrome.
  
• In about 25% cases it does not show any cause.
  
• Tuberous sclerosis and perinatal asphyxia are common causes of West syndrome.
  
• Birth injury, brain malformations, metabolic disorders and chromosomal abnormalities may responsible for development of spasms.
  
• Excessive production of Corticotropin-releasing hormone (CRH).

Symptoms of West Syndrome

• Body may bends forward and stiffening of the arms and legs.
  
• Arching of backs
  
• Drawing up of knees when lying down
  
• Crossing of arms across body
  
• Crying or may show irritability.
  
• Flexor muscles of the neck, trunks and limbs shows contraction.
  
• Developmental delay and long-term learning problems.
  
• Lack of responsiveness or loss of muscle tone.

How West Syndrome is diagnosed?

When electroencephalogram (EEG) shows a characteristic pattern called hypsarrhythmia then it is confirmed.
Blood tests, urine examination and CSF analysis is done.
CT and MRI scan are done to identify brain abnormalities.

Is there any treatment for West syndrome?

• Steroid therapy is mainly used for West syndrome. Prednisone is a steroid drug which helps to control spasms.
  
• Sabril is mostly used because of its effectiveness but it should be given with proper care.
  
• To reduce seizure benzodiazepines, valproate, lamotrigine or topamax medicines are recommended by doctor.
  
• Adrenocorticotropic hormone (ACTH) is used to minimize the production of corticotrophin-releasing hormone (CRH) with side effect.
  
• Brain surgery is another option but it totally depends on underlying cause such as brain lesion or structural abnormality.