Introduction: West syndrome is an infantile epileptic encephalopathy with  heterogeneous etiology and variable course.¹ This study describes the clinical, therapy and outcome related characteristics of children with West syndrome in a tertiary care hospital in north India.

Materials and Methods: In this retrospective analysis, 310 children with West syndrome enrolled from January 2009 to March 2013 were reviewed.

Results: The median age at onset of spasms was 5 months and age at presentation was 18 months.  The predominant spasm semiology was flexor [78.4%], perinatal cerebral insult [55.5%] being the commonest etiology. 91.6% (284/310) children received ACTH (140) or oral steroid (144) as first line therapy. Difference in proportion of patients showing complete resolution of spasm between ACTH (40%, 56/140) and oral steroids group(46.5%, 67/144) was not significant ( p=0.27). Nineteen patients received ketogenic diet with 15 (79%) showing greater than 50 % seizure reduction. In 46.5% patients spasms resolved, 9% evolved to Lennox-Gastaut syndrome, 18.1% developed other seizure types and 26.5% had persistent spasms at last follow up (median follow up of 10 months). The median age at initiation of treatment of choice was significantly less (p<0.001) in patients who showed complete cessation of spasm (15 months) compared to those with partial or no response ( 20 months).

Conclusions: ACTH is the first line treatment in west syndrome, however oral steroids may be considered in resource-limited settings. Dietary therapy should be considered in pharmacoresistant cases. Early identification, appropriate management and good supportive care are critical for optimal neuro-developmental outcome.²