West Syndrome

(Infantile Spasm/Generalized flexion Epilepsy/ Infantile Epileptic Encephalopathy/ infantile Myoclonic Encephalopathy / Jerk knife Convulsion / Massive Myoclonic / Salaam spasm)

West Syndrome , a neurological disorder has three distinct features namely: Infantile spasm, Hypsarrhythmia, and Mental retardation.

Incidence
1 in 4000-6000 live births, Boys are more affected than Girls in the ration of 3:2

Symptoms / Clinical Signs
The epileptic seizure which is observed in Infantile Spasm can be described as

  • Lightning Attacks
  • Nodding Attacks
  • Salaam or Jerk Knife Attacks

Causes with Types
Infantile Spasm can be classified into three classes based on its etiology (cause)

  •  Symptomatic- Identified cause-75% of total cases
  • Cryptogenic- Suspected cause-12% of total cases
  • Idiopathic- no known cause-13% of total cases

     
Diagnosis Making (Tests and Investigations)

  • Neurological examination of these children shows abnormal cognitive function and motor developmental delay.
  • MRI / CT scan of the brain, EEG and a few lab tests are done to clinically confirm

Management / Treatment of Infantile Spasm (West Syndrome)

  •  Medical Care

When a child is diagnosed with Infantile spasm, the first thing to start is medication. The goal of the treatment is to make the child seizure free and getting minimum side effects of the medications. A number of medicines are available for this condition. There is no hard and fast protocol available to treat this disorder. It has been found that treating the Infantile Spasm is more difficult than common epilepsy.

  • Habilitation Therapies

As the child gets developmental delay both in physical and mental ability; the child should be provided Developmental therapy (Combination of Physiotherapy and Occupational therapy), Special education and Speech therapy regularly. Postural, Orthotic and Mobility aids are also prescribed based on age and needs. Once the child is seizure free, the child gets quick motor development but cognition remains a concern.

  • Surgical Treatment

Advance and specialized neurosurgical options are available. Focal Cortical Resection is a common procedure. 

  • Ketogenic Diets

Nowadays ketogenic diet has been one of the options to treat a variety of Infantile Spasm successfully.

  • Prognosis

With advancement of Science, the prognosis of children with Infantile Spasm has improved a lot during the last decades. Prognosis depends on the type of the Infantile Spasm. In general the cognitive prognosis is the prime concern. Children with Idiopathic Infantile Spasm do better than other variety of Infantile Spasm.

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