OBJECTIVE To describe the neurological and neurodevelopmental outcomes of children with Congenital Zika Syndrome (CZS) associated microcephaly beyond 2 years of age. METHOD We followed children with CZS-associated microcephaly in an outpatient clinic in Salvador, Brazil. Neurological and neurodevelopmental assessments were performed using the Hammersmith Infant Neurological Examination (HINE) and Bayley Scales of Infant and Toddler Neurodevelopment (Bayley-III) respectively. RESULTS Of the 42 children included, 19 were male (45.2%); median (interquartile range) age at neurological evaluation was 28 (25-32) months, and 36 (85.7%) had severe microcephaly. HINE and Bayley-III results were completed for 35/42 (83.3%) and 33/42 (78.5%) children respectively. Bayley-III identified a severe developmental delay in 32/33 (97.0%) children while 1/33 (3.0%) had only a mild delay. In the multivariable analysis, we found that Bayley-III and HINE scores were correlated. Better HINE scores were associated with higher Bayley-III cognitive raw scores (β = 0.29; CI 95% = 0.02-0.57) and motor raw scores (β = 0.43; CI 95% = 0.04-0.82) after adjusting for head circumference, prematurity, and age at neurodevelopmental evaluation. Furthermore, we found that greater head circumference at follow up was associated with higher cognitive (β = 1.27; CI 95% = 0.01-2.53) and motor raw scores (β = 2.03; CI 95% = 0.25-3.81). CONCLUSION Children with CZS-associated microcephaly demonstrate severe neurodevelopmental delays and slower growth rates than their peers over time. Still, they have remarkably heterogeneous neurodevelopmental profiles according to neurological exam scores which correlate with their long-term outcomes. We found that HINE scores effectively captured the heterogeneity of neurological capabilities among these children and could be predictive of cognitive and motor development progress.

中文翻译：

---

先天性寨卡综合征相关小头畸形儿童的异质性发育。

目的 描述 2 岁以上先天性寨卡综合征 (CZS) 相关小头畸形儿童的神经和神经发育结果。方法 我们在巴西萨尔瓦多的一家门诊随访了患有 CZS 相关小头畸形的儿童。神经学和神经发育评估分别使用 Hammersmith 婴儿神经学检查 (HINE) 和贝利婴儿和幼儿神经发育量表 (Bayley-III) 进行。结果 纳入的 42 名儿童中，男性 19 名（45.2%）；神经学评估时的中位（四分位距）年龄为 28 (25-32) 个月，36 (85.7%) 个月有严重的小头畸形。分别为 35/42 (83.3%) 和 33/42 (78.5%) 儿童完成了 HINE 和 Bayley-III 结果。Bayley-III 发现 32/33 (97.0%) 儿童存在严重的发育迟缓，而 1/33 (3. 0%) 只有轻微的延迟。在多变量分析中，我们发现 Bayley-III 和 HINE 分数是相关的。在调整头围、早产后，更好的 HINE 评分与更高的 Bayley-III 认知原始评分（β = 0.29；CI 95% = 0.02-0.57）和运动原始评分（β = 0.43；CI 95% = 0.04-0.82）相关，以及神经发育评估的年龄。此外，我们发现随访时较大的头围与较高的认知（β = 1.27；CI 95% = 0.01-2.53）和运动原始评分（β = 2.03；CI 95% = 0.25-3.81）相关。结论 随着时间的推移，患有 CZS 相关小头畸形的儿童表现出严重的神经发育延迟和较慢的生长速度。仍然，根据与他们的长期结果相关的神经学检查分数，他们具有显着异质的神经发育特征。我们发现 HINE 分数有效地捕捉了这些儿童神经功能的异质性，并且可以预测认知和运动发育进展。