Department of Disability Studies, Faculty of Medicine, University of Kelaniya, Ragama, Sri Lanka.
Department of Paediatrics, Faculty of Medicine, University of Colombo, Colombo, Sri Lanka.
Dev Med Child Neurol. 2019 Nov;61(11):1295-1301. doi: 10.1111/dmcn.14205. Epub 2019 Mar 10.
To describe the baseline developmental profile and influence of clinical and demographic factors on the developmental skills of infants diagnosed with infantile spasms.
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.
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.
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.
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.
描述婴儿痉挛症患儿的发育基线特征，并探讨临床和人口统计学因素对其发育技能的影响。
本研究为一项横断面、纵向研究，共纳入 95 例新诊断为婴儿痉挛症的婴儿。所有婴儿均接受贝利婴幼儿发育量表评估，评估领域包括认知、接受性沟通、表达性沟通、精细运动和粗大运动发育；还进行了视觉、听觉和社会行为评估。将婴儿分为“早发型”（<6 个月）和“晚发型”（≥6 个月）；如果在 28 天内出现，则视为“早发型”，否则视为“迟发型”。确定了产前、围产期和产后的危险因素。
超过 90%的婴儿在所有领域均存在发育障碍，多数为严重延迟；99%的婴儿存在认知障碍。迟发型与接受性沟通延迟显著相关（优势比[OR]=5.35；95%置信区间[CI]=1.05-27.32）。6 个月或以下发病与听觉（OR=2.8；95%CI=1.16-6.8）和视觉（OR=3.03；95%CI=1.22-7.57）行为异常有关。新生儿感染均影响接受性（OR=1.12；95%CI=1.04-1.2）和表达性沟通（OR=1.08；95%CI=1.02-1.14）延迟。新生儿癫痫显著影响视觉、听觉和社会功能障碍。表达性沟通和粗大运动发育有共同的围产期危险因素。
发病时的不良发育状态与迟发型和新生儿危险因素有关，应提醒临床医生对高危婴儿进行监测，并寻求及时的干预措施。
90%的婴儿在发病时存在认知、沟通和运动技能障碍。视觉、听觉和社会行为障碍与围产期风险显著相关。视觉、听觉和社会行为障碍与新生儿癫痫显著相关。
