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West Syndrome

West Syndrome

It is an epileptic seizure disorder also called as epileptic or infantile spasms. It is characterized by infantile spasms due to abnormal brain wave called Hypsarrthymia and intellectual disability (mental retardation). The characteristic feature is violent ‘jack knife’ or ‘salaam’ movements in which whole body bend forwards in half or may occur as mild twitching of shoulder or eye changes. Spasm occurs in early months after birth. It can also occur in grown up children called as Epileptic spasms. It occurs more commonly in males than in females.


It can be caused by:

1) Hemi-megaloencephaly: increased brain size of one side

2) Neonatal infections

3) Down syndrome

4) X-linked epileptic disorder

5) Neurocutaneous disorders like Tuberous sclerosis complex

6) Sturge weber syndrome etc.


Signs and symptoms:- 

1) Age of onset is around 6 months 

2) Involuntary muscle spasms

3) Each spasm begins suddenly and lasts for few seconds and occurs in clusters that lasts over 10-20 minutes

4) It occurs upon awakening or after feeding 

5) Movements are sudden, involuntary contractions of hand, neck and trunk and extension of legs and arms.

6) EEG shows arrhythmia

7) Regression of skills or delay in acquiring skills.


Diagnosis:-

1) EEG- characteristically shows hypsarrythmia

2) CT scan of brain

3) MRI- brain to rule out brain malformation and any other lesions

4) Skin examination- to rule out any lesions as seen in Tuberous sclerosis disease

5) Molecular genetic testing


Treatment:-

It should be coordinated effort of pediatrician, neurologist, surgeon, therapist to treat child.


Use of anticonvulsant drugs

Use of ACTH hormone

Other drugs like pyridoxine, predinisolone etc.


The shorten time between diagnosis and treatment will have a less deleterious effect on development.

Approximately another third of children will continue to have epileptic spasms at an elder age. The last third to quarter of patients will have spasms that resolve with time.

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