NEUROPSYCHOLOGICAL ABNORMALITIES IN CHILDREN WITH CONGENITAL CENTRAL HYPOVENTILATION SYNDROME
NEUROPSYCHOLOGICAL ABNORMALITIES IN CHILDREN WITH CONGENITAL CENTRAL HYPOVENTILATION SYNDROME
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DOI：
10.1016/s0022-3476(05)80902-3
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发表时间：
1992-03-01
影响因子：
5.1
通讯作者：
NELSON, MN
中科院分区：
文献类型：
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作者：
SILVESTRI, JM;WEESEMAYER, DE;NELSON, MN
我们假设智力、神经发育和视觉运动测试能够表征先天性中枢性通气不足综合征患儿中枢神经系统受累的范围和性质。对17名先天性中枢性低通气综合征患儿（年龄0.1 ~ 14.3岁）进行了适合儿童的智力（Brazelton新生儿行为评估、Bayley婴儿发育量表、韦氏学龄前和初级智力量表修订版和韦氏儿童智力量表修订版）、神经发育（Halsted-Reitan神经心理成套测验）和视觉-运动（Beery视觉-运动整合测验）测试。我们发现智商和发育商的范围很广，从大于第85百分位数到小于第5百分位数，语言和表现指标之间存在差异。多个不对称和缺陷的运动性能，不一贯相关的利手，也被发现。显着的眼手协调缺陷是罕见的，但一般性能远低于平均水平。通过卡方分析比较了智商大于或等于70和小于70的儿童的全面智商、语言智商和操作智商以及发育智商与其他相关条件的关系，这些相关条件包括：辅助支持时间、首次住院时间、生长、肺动脉高压、癫痫发作、脑萎缩、中枢和外周听力缺陷以及眼科异常;这些结果支持先天性中枢性肺通气不足综合征是一种弥漫性中枢神经系统疾病的假设。然而，暂时性缺氧和相关条件对神经发育测试结果的影响不能肯定地排除。
We hypothesized that intellectual, neurodevelopmental, and visual-motor tests would be able to characterize the scope and nature of central nervous system involvement in children with congenital central hypoventilation syndrome. Age-appropriate intellectual (Brazelton Neonatal Behavioral Assessment, Bayley Scales of Infant Development, Wechsler Preschool and Primary Scale of Intelligence-Revised and Wechsler Intelligence Scale for Children-Revised), neurodevelopmental (Halsted-Reitan neuropsychologic battery), and visual-motor (Beery visual-motor integration test) tests were given to 17 children with congenital central hypoventilation syndrome (age 0.1 to 14.3 years). We found a broad range in IQ and developmental quotient, from greater than the 85th percentile to less than the 5th percentile, with discrepancies noted between verbal and performance measures. Multiple asymmetries and deficiencies of motor performance, not consistently related to handedness, were also found. Significant eye-hand coordination deficits were uncommon, but general performance was well below average. Full, verbal, and performance IQs and developmental quotients greater-than-or-equal-to 70 and < 70 were compared by chi-square analysis with other associated conditions, including hours of ventilatory support, duration of initial hospitalization, growth, pulmonary hypertension, seizures, brain atrophy, central and peripheral hearing deficits, and ophthalmologic abnormalities; no statistically significant associations were found. These results lend support to the hypothesis that congenital central hypoventilation syndrome is a diffuse central nervous system process. However, the effects of transient hypoxic and associated conditions on neurodevelopmental test results cannot be excluded with certainty.