WEST Syndrome:

When does a medical student considers herself lucky? 

When she gets to see a patient with a disease so rare, that it's a once in a lifetime case. 

I was the lucky one.

Patient History-

We have a boy around 2 years of age, coming from lower socio economic class. He was a full term baby with normal birth weight. However, there is a history of HIE i.e Hypoxic ischemic encephalopathy during the time of birth.

Since then, patient has complaints of seizures; sometimes so severe that hospitalization was necessary. He has been hospitalised on and off every 1 month. 

Patient turned 2 years and still isn't able to crawl/sit/stand/walk and only speaks monosyllable words. This is called developmental delay.

EEG was found to be hypsarrythmic. 

Thus, Diagnosis of West syndrome was made.

What is West Syndrome?

It is a seizure syndrome and it has characteristic triad of:-

1. Infantile spasms- salaam spells

2. Developmental delay

3. Hypsarrythmia- high amplitude spikes in a         chaotic background.

Patient presentation?

Patients presents with history of salaam spells that is sudden dropping of head and flexion of arms occurring mostly during time of waking. Mother complaints of baby unable to sit or crawl.

Causes;

1. Hypoxic ischemic encephalopathy 

2. Neurocutaneous syndrome like tuberous sclerosis

3. Perinatal infections

4. Hemorrhage 

5. Idiopathic

EEG findings- high amplitude spikes in a chaotic background. 

Credits- epilepsydiagnosis.com

Treatment- no definitive treatment. Only symptomatic management.

Vigabatrin is the drug of choice; especially in Tuberous sclerosis.

ACTC and prednisone for 2-12 weeks, according to response.

Prognosis - poor if developmental retardation is found.

Thank you.

- Thirak Vaishnav