Abstract：
BACKGROUND: The data on specific comorbidities in children with dyskinetic cerebral palsy (DCP) is limited. We evaluated the pattern of comorbidities and health related quality of life (HRQOL) in these children and compared them between etiological and motor impairment subgroups.
METHODS: This cross-sectional study was conducted over 18 months in children with DCP of both sex, and age between one and 14 years. Comorbidities were assessed using standardized scales such as gross motor functioning scale (GMFCS), developmental profile-3 (DP-3), developmental behaviour checklist, sleep behaviour questionnaire (SBQ), and caregiver questionnaire.
RESULTS: Sixty-five children with DCP were evaluated (hyperbilirubinemia n = 43, 66% and perinatal asphyxia n = 19, 29%). The majority of children were severely affected in gross motor functioning (level IV 29.2% and level V 53.8%). Epilepsy was seen in 21.5% of cases (19% in hyperbilirubinemia and 32% in asphyxia, p = 0.4). The mean age of onset of seizures was 15.4 + 20.6 months (range 2-72). Visual problems were seen in 54% of cases and included upgaze palsy, squint, refractive error, optic atrophy and cortical blindness. A significant proportion of children with hyperbilirubinemia had upgaze palsy as compared to those with perinatal asphyxia (70% vs. 32%, p 0.01). Rest of the visual problems were not significantly different between the two etiological subgroups. Drooling (87.6%), protein-energy malnutrition (66.6%), and reflux (57%) were the most common gastrointestinal problems in children with DCP. Children with DCP showed problems in social relating (33.8%), anxiety (26.2%), and self-absorbed behaviour (7.7%). However, there were no statistically significant differences between the etiological, motor impairment and age-based subgroups. Children with DCP had high scores on SBQ, suggesting sleep problems. Sleep scores were similar in the hyperbilirubinemia and perinatal asphyxia subgroups. Greater sleep problems were noted in children aged < 4y (70.6 + 10.1 vs. 56.5 + 11.3, p < 0.05 as compared to children above 4y of age) and severe motor impairments (68.2 + 11.3 vs. 57.2 + 13.1, p 0.008 as compared to mild-moderate motor impairment). Poor overall developmental scores were seen in 61.5% children and were significantly associated with GMFCS (p 0.04). The majority of children showed impairments in physical (58.5%), adaptive behaviour (58.5%), social-emotional (50.8%), cognitive (60%) and communication (52%) subscales of DP-3. Cognitive impairment was similar in the etiological (hyperbilirubinemia vs. perinatal asphyxia, p = 0.3), and motor impairment (mild-moderate vs. severe, p = 0.9) subgroups. HRQOL was significantly affected by motor impairment in positioning-transfer (p value 0.0001), and interaction-communication domains (p value 0.0001), however, there was no difference based on the etiology of hyperbilirubinemia and asphyxia.
CONCLUSIONS: Children with DCP demonstrate several comorbidities and impaired quality of life. These are similar in hyperbilirubinemia and perinatal asphyxia cohorts, expect for significant proportion of upgaze palsy in DCP secondary to hyperbilirubinemia. Younger children have more problematic behaviour and impaired sleep quality. Severe motor disability influences the developmental outcomes, cognition, sleep and HRQOL in children with DCP.
METHODS: This cross-sectional study was conducted over 18 months in children with DCP of both sex, and age between one and 14 years. Comorbidities were assessed using standardized scales such as gross motor functioning scale (GMFCS), developmental profile-3 (DP-3), developmental behaviour checklist, sleep behaviour questionnaire (SBQ), and caregiver questionnaire.
RESULTS: Sixty-five children with DCP were evaluated (hyperbilirubinemia n = 43, 66% and perinatal asphyxia n = 19, 29%). The majority of children were severely affected in gross motor functioning (level IV 29.2% and level V 53.8%). Epilepsy was seen in 21.5% of cases (19% in hyperbilirubinemia and 32% in asphyxia, p = 0.4). The mean age of onset of seizures was 15.4 + 20.6 months (range 2-72). Visual problems were seen in 54% of cases and included upgaze palsy, squint, refractive error, optic atrophy and cortical blindness. A significant proportion of children with hyperbilirubinemia had upgaze palsy as compared to those with perinatal asphyxia (70% vs. 32%, p 0.01). Rest of the visual problems were not significantly different between the two etiological subgroups. Drooling (87.6%), protein-energy malnutrition (66.6%), and reflux (57%) were the most common gastrointestinal problems in children with DCP. Children with DCP showed problems in social relating (33.8%), anxiety (26.2%), and self-absorbed behaviour (7.7%). However, there were no statistically significant differences between the etiological, motor impairment and age-based subgroups. Children with DCP had high scores on SBQ, suggesting sleep problems. Sleep scores were similar in the hyperbilirubinemia and perinatal asphyxia subgroups. Greater sleep problems were noted in children aged < 4y (70.6 + 10.1 vs. 56.5 + 11.3, p < 0.05 as compared to children above 4y of age) and severe motor impairments (68.2 + 11.3 vs. 57.2 + 13.1, p 0.008 as compared to mild-moderate motor impairment). Poor overall developmental scores were seen in 61.5% children and were significantly associated with GMFCS (p 0.04). The majority of children showed impairments in physical (58.5%), adaptive behaviour (58.5%), social-emotional (50.8%), cognitive (60%) and communication (52%) subscales of DP-3. Cognitive impairment was similar in the etiological (hyperbilirubinemia vs. perinatal asphyxia, p = 0.3), and motor impairment (mild-moderate vs. severe, p = 0.9) subgroups. HRQOL was significantly affected by motor impairment in positioning-transfer (p value 0.0001), and interaction-communication domains (p value 0.0001), however, there was no difference based on the etiology of hyperbilirubinemia and asphyxia.
CONCLUSIONS: Children with DCP demonstrate several comorbidities and impaired quality of life. These are similar in hyperbilirubinemia and perinatal asphyxia cohorts, expect for significant proportion of upgaze palsy in DCP secondary to hyperbilirubinemia. Younger children have more problematic behaviour and impaired sleep quality. Severe motor disability influences the developmental outcomes, cognition, sleep and HRQOL in children with DCP.
摘要：
背景：关于运动障碍性脑瘫（DCP）儿童的特定合并症的数据有限。我们评估了这些儿童的合并症和健康相关生活质量（HRQOL）的模式，并在病因和运动障碍亚组之间进行了比较。
方法：这项横断面研究是在18个月内对两种性别的DCP儿童进行的，年龄在1到14岁之间。合并症使用标准化量表进行评估，例如粗大运动功能量表（GMFCS），发育剖面-3(DP-3)，发展行为检查表，睡眠行为问卷（SBQ），和照顾者问卷。
结果：对65例DCP患儿进行了评估（高胆红素血症n=43，66％，围产期窒息n=19，29％）。大多数儿童的粗大运动功能受到严重影响（IV级29.2％和V级53.8％）。癫痫在21.5％的病例中可见（高胆红素血症为19％，窒息为32％，p=0.4)。癫痫发作的平均年龄为15.4+20.6个月(范围2-72)。54％的病例出现视觉问题，包括上凝视麻痹，斜视,屈光不正,视神经萎缩和皮质盲.与围产期窒息的儿童相比，高胆红素血症的儿童中有很大一部分患有上凝视麻痹（70％vs.32%,P0.01)。其余的视觉问题在两个病因亚组之间没有显着差异。流口水（87.6%），蛋白质-能量营养不良（66.6%），反流（57％）是DCP患儿最常见的胃肠道问题。患有DCP的儿童在社交方面表现出问题（33.8％），焦虑（26.2%），和自我吸收行为(7.7%)。然而,病因之间没有统计学上的显著差异，运动障碍和基于年龄的亚组。患有DCP的儿童在SBQ上得分很高，暗示睡眠问题。高胆红素血症和围产期窒息亚组的睡眠评分相似。4岁以下儿童的睡眠问题更大（70.6+10.1vs.56.5+11.3，与4岁以上的儿童相比，p<0.05）和严重的运动障碍（68.2+11.3vs.57.2+13.1，p为0.008，与轻度-中度运动障碍相比）。在61.5％的儿童中，总体发育评分较差，并且与GMFCS显着相关（p0.04）。大多数儿童表现出身体受损(58.5%)，适应性行为（58.5%），社会情感（50.8%），DP-3的认知(60%)和沟通(52%)分量表。认知障碍在病因上相似（高胆红素血症与围产期窒息,p=0.3),和运动障碍(轻度-中度vs.严重,p=0.9)亚组。HRQOL在定位转移中受到运动损伤的显着影响（p值0.0001），和交互通信域(p值0.0001)，然而,根据高胆红素血症和窒息的病因没有差异。
结论：DCP患儿表现出多种合并症和生活质量受损。这些在高胆红素血症和围产期窒息队列中相似，预计在继发于高胆红素血症的DCP中，上凝视麻痹的比例很大。年幼的孩子有更多的问题行为和睡眠质量受损。严重的运动障碍会影响发育结果，认知，DCP儿童的睡眠和HRQOL。
方法：这项横断面研究是在18个月内对两种性别的DCP儿童进行的，年龄在1到14岁之间。合并症使用标准化量表进行评估，例如粗大运动功能量表（GMFCS），发育剖面-3(DP-3)，发展行为检查表，睡眠行为问卷（SBQ），和照顾者问卷。
结果：对65例DCP患儿进行了评估（高胆红素血症n=43，66％，围产期窒息n=19，29％）。大多数儿童的粗大运动功能受到严重影响（IV级29.2％和V级53.8％）。癫痫在21.5％的病例中可见（高胆红素血症为19％，窒息为32％，p=0.4)。癫痫发作的平均年龄为15.4+20.6个月(范围2-72)。54％的病例出现视觉问题，包括上凝视麻痹，斜视,屈光不正,视神经萎缩和皮质盲.与围产期窒息的儿童相比，高胆红素血症的儿童中有很大一部分患有上凝视麻痹（70％vs.32%,P0.01)。其余的视觉问题在两个病因亚组之间没有显着差异。流口水（87.6%），蛋白质-能量营养不良（66.6%），反流（57％）是DCP患儿最常见的胃肠道问题。患有DCP的儿童在社交方面表现出问题（33.8％），焦虑（26.2%），和自我吸收行为(7.7%)。然而,病因之间没有统计学上的显著差异，运动障碍和基于年龄的亚组。患有DCP的儿童在SBQ上得分很高，暗示睡眠问题。高胆红素血症和围产期窒息亚组的睡眠评分相似。4岁以下儿童的睡眠问题更大（70.6+10.1vs.56.5+11.3，与4岁以上的儿童相比，p<0.05）和严重的运动障碍（68.2+11.3vs.57.2+13.1，p为0.008，与轻度-中度运动障碍相比）。在61.5％的儿童中，总体发育评分较差，并且与GMFCS显着相关（p0.04）。大多数儿童表现出身体受损(58.5%)，适应性行为（58.5%），社会情感（50.8%），DP-3的认知(60%)和沟通(52%)分量表。认知障碍在病因上相似（高胆红素血症与围产期窒息,p=0.3),和运动障碍(轻度-中度vs.严重,p=0.9)亚组。HRQOL在定位转移中受到运动损伤的显着影响（p值0.0001），和交互通信域(p值0.0001)，然而,根据高胆红素血症和窒息的病因没有差异。
结论：DCP患儿表现出多种合并症和生活质量受损。这些在高胆红素血症和围产期窒息队列中相似，预计在继发于高胆红素血症的DCP中，上凝视麻痹的比例很大。年幼的孩子有更多的问题行为和睡眠质量受损。严重的运动障碍会影响发育结果，认知，DCP儿童的睡眠和HRQOL。
