Developmental profile at initial presentation in children with infantile spasms

Abstract

Aim To describe the baseline developmental profile and influence of clinical and demographic factors on the developmental skills of infants diagnosed with infantile spasms. Method Ninety‐five infants (55 males, 40 females) newly diagnosed with infantile spasms were recruited for a cross‐sectional, longitudinal study. All infants underwent Bayley Scales of Infant and Toddler Development assessments in the cognitive, receptive communication, expressive communication, and fine and gross motor developmental domains; they also underwent visual, auditory, and social behaviour assessments. Infants were categorized as ‘early’ (<6mo) or ‘late’ (≥6mo) presenters; if presented within 28 days, this was considered as ‘early presentation’, whereas a delay greater than 28 days was considered as a ‘delay in presentation’. Antenatal, perinatal, and postnatal risk factors were identified. Results Over 90% of infants showed impairment in all domains, with the majority having severe delay; 99% showed cognitive impairment. Delayed presentation was significantly associated with receptive communication delay (odds ratio [OR]=5.35; 95% confidence interval [CI]=1.05–27.32). Onset at 6 months or less influenced auditory (OR=2.8; 95% CI=1.16–6.8) and visual (OR=3.03; 95% CI=1.22–7.57) behaviours. Neonatal infections impacted both receptive (OR=1.12; 95% CI=1.04–1.2) and expressive communication (OR=1.08; 95% CI=1.02–1.14) delay. Neonatal seizures significantly influenced visual, auditory, and social impairments. Expressive communication and gross motor development shared common perinatal risk factors. Interpretation Adverse developmental status at presentation, associated with delayed presentation and neonatal risk factors should alert clinicians to the surveillance of at‐risk infants and seek out timely interventions. What this paper adds Ninety per cent of infants showed impaired cognitive, communication, and motor skills at presentation. Visual, auditory, and social behaviour impairments were significantly associated with perinatal risks. Visual, auditory, and social behaviour impairments were significantly associated with neonatal seizures.

Management practices for West syndrome in South Asia: A survey study and meta‐analysis

“…Interestingly, some responders from Pakistan observe genetic causes more commonly, probably due to higher consanguinity. These responses are in accord with the results of meta‐analysis with a pooled mean LTTT of 2.4 months and a static structural insult to the tune of 80% 15–26,29,30 . The longer LTTT estimate (4.4 months) and a higher preponderance of static structural insult (82%) and acquired structural insult (71%) in India are bothersome.…”

“…Of 1012 studies screened, 18 studies fulfilled the eligibility criteria and were included for the meta‐analysis (Figure ) 7,15–31 . These studies were from three countries (India, Pakistan, and Sri Lanka), and there was no published study fulfilling eligibility criteria from Bangladesh, Bhutan, Nepal, and Myanmar.…”

Infantile Spasms: Opportunities to Improve Care

“…A recent study found 90% of infants with IS had developmental delay at the time of presentation. 23 More than half had impaired social responsiveness, such as absent social smile, as well as auditory impairments, including poor response to voice. In addition, 44% displayed visual inattention, which caregivers may notice as a decrease in eye contact or tracking.…”

Neurodevelopmental status in infantile spasms and West syndrome: the challenge of assessment

“…The study by Sumanasena et al . has made a significant contribution to the literature by systematically assessing developmental status, at the time of clinical presentation and before randomized treatment allocation as part of a clinical trial, in a cohort of 95 infants who had epileptic spasms and hypsarrhythmia.…”

“…The study by Sumanasena et al 2 has made a significant contribution to the literature by systematically assessing developmental status, at the time of clinical presentation and before randomized treatment allocation as part of a clinical trial, in a cohort of 95 infants who had epileptic spasms and hypsarrhythmia. More than 90% of the infants were found to have developmental delay in several domains at the time of presentation; only one of the infants had scores within the average range for all the developmental domains.…”