West Syndrome

West syndrome is generally known as Infantile Spasm. The other names of this condition are “generalized flexion Epilepsy”, “Infantile epileptic encephalopathy”, “Infantile myoclonic encephalopathy”, “Jerk knife convulsion”, “Massive myoclonic”, and “Salaam Spasm”. West syndrome has three distinct features namely: Infantile spasm, Hypsarrhythmia, and Mental retardation. It is an age dependent disorder.

Incidence
The incidence is 1 in 4000-6000 live births. Boys are more likely to be affected than girls. The ratio is approximately 3:2.

Symptoms / Clinical Signs

  • The epileptic seizure which is observed in Infantile Spasm can
  • Lightning Attacks
  • Nodding Attacks
  • Salaam or Jerk Knife Attacks

Types
Infantile Spasm can be classified into three classes based on its etiology
-Symptomatic
- Cryptogenic
- Idiopathic

Symptomatic
When the symptom is present due to identified factor, it is called symptomatic. A number disorders like encephalitis, hydrocephaly, Microcephaly hydrocephalus, schizencephaly, polymicrogyria, tuberous sclerosis, HIE, meningitis, trauma, intracranial hemorrhage, phenylketonuria, biotinidase deficiency, pyrodoxin dependency.
Of patients with Infantile Spasm, 70-75% cases have Symptomatic Infantile Spasm

Cryptogenic
When no specific cause is identified but there is a suspicion on a cause. It is called Cryptogenic Infantile Spasm. Generally 8-12 % of cases with Infantile Spasm have Cryptogenic Infantile Spasm

Idiopathic
When there is no psychomotor developmental delay is observed and the child has Infantile Spasm, it is considered as Idiopathic Infantile Spasm. 9-14% of cases with Infantile Spasm belong to this group.

 
Causes
The exact of Infantile spasm is unknown in most of the cases. A few hypotheses are described to make it clear.
A group of researchers feel it is a malfunction of the neurotransmitter function in the regulation of GABA transmission process.
Other group of researchers feels it is a hyper production of corticotrophin releasing hormone.

      
Diagnosis making / Tests and Investigations
Generally males are affected than females. 90% of children who have Infantile Spasm gets first episode during the first year of life. The peak onset is 4-6 months of age. On neurological examination these children show abnormal cognitive function and motor developmental delay. To confirm the diagnosis, MRI / CT Scan of the brain, EEG and a few lab tests are done.

Management / Treatment
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 Therapy
As the child gets developmental delay both in physical and mental ability, the chil should be given Physiotherapy, Occupational therapy and Special education regularly. Postural Aids and Orthotic Aids are generally required in the management of the child. Once the child is seizure free, the child gets very fast motor development but cognition remain a concern.

Surgical Treatment
A few neurosurgical options are available. Focal Cortical Resection is advised by some neurologist. 

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 not so good. Children with Idiopathic Infantile Spasm do better than other variety of Infantile Spasm.