AimsWest Syndrome is an age dependent epileptic encephalopathy with variable therapeutic response. Worldwide, there is scarce data on magnitude and effect of treatment lag on therapeutic response with adrenocorticotrophic hormone (ACTH) or steroid therapy in West Syndrome. There is conflicting evidence regarding impact of treatment lag in West Syndrome. The present study aimed to elucidate magnitude of diagnostic and treatment lag, potential determinants of treatment lag and its impact on short-term therapeutic response with ACTH or oral steroids therapy.MethodsWe conducted a prospective study in a tertiary care hospital in northern India between January-December 2014. A total of 82 consecutive children with West Syndrome were enrolled. Magnitude and determinants of treatment lag were determined in all the children. Parents were interviewed and medical records of the child were reviewed. The treatment lag was calculated as the time delay between onset of spasms and initiation of ACTH/oral steroids treatment. Short term therapeutic response was taken as cessation of spasms within 14 days of therapy and sustained for a period of 28 days from the last witnessed spasm. We analysed following potential predictors: age of onset of spasms, aetiology, treatment lag and gender for their association with short-term therapeutic response.ResultsThe median treatment lag duration was 90 days (95% CI: 110–198 days). The significant determinants of treatment lag in our study were: the pre-existing delay of children, educational status of the parents and qualification of the first practitioner visited. Our study showed shorter treatment lag (<1 month) was associated with a better spasms cessation rate (85% vs 15%; p=0.011).ConclusionWe observed a significant treatment lag in our children with West Syndrome. The lead time to treatment emerged as a potential modifiable risk factor for therapeutic response with ACTH/steroid therapy.

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AimsPhenytoin and Carbamazepine, the most common antiepileptic drugs as monotherapy, are associated with cutaneous adverse drug reactions (cADRs). These can range from mild maculopapular exanthema to life threatening conditions, including, Stevens–Johnson syndrome and toxic epidermal necrolysis. However, there is scarce data on incidence of these reactions. Our study aimed to prospectively determine the incidence and nature of cADRs in children on antiepileptic drugs monotherapy.MethodsA prospective observational study conducted at outpatient department of tertiary care referral hospital of Northern India from July 2015 to September 2016. All consecutive children (6 months–12 years old) recently initiated (<4 weeks) on AED monotherapy (phenytoin, carbamazepine or valproate) were enrolled and followed up at 1 and 3 months for cADRs. Occurrence of cADRs, if any, was evaluated. Causal relationship assessments of cADRs with antiepileptic drugs were performed by Naranjo’s algorithm and World Health Organisation–Uppsala Monitoring Centre scale. Severity assessment of cADRs was done with Hartwig’s severity scale.ResultsOf 295 children enrolled, 118 (40%) were on carbamazepine, 92 (31.2%) were on phenytoin and 85 (28.8%) were on valproate. A total of 11/295 (3.7%) children had cutaneous adverse drug reactions. Of 118 on carbamazepine, 4 (3.4%) had cADRs. Of 92 on phenytoin, 7 (7.6%) had cADRs. No children on valproate therapy had cADRs. The maculopapular rash was the most common 9/11 (81.8%). One child had urticaria and one had Drug Reaction with Eosinophilia and Systemic Symptoms. The mean duration interval from drug intake to rash onset was 14.5 (range 6–60) days. Discontinuation of antiepileptic drug was required in 8 (72.7%) cases. Using Naranjo algorithm, 81.8% cADRs were probable and 72.7% were probable on WHO- Uppsala Monitoring Centre scale. Most cADRs (63.6%) were in the level 3 (moderate) on Hartwigs severity scale.ConclusionsIncidence of cADRs with phenytoin and carbamazepine were 7.6% and 3.4% respectively. Maculopapular rash was the most common and preponderance of cADR had moderate severity. Awareness and knowledge of cADRs related information would be helpful in counselling while initiation of these antiepileptic drugs.