多角度分析儿童言语失用症与其合并神经发育障碍的异同

Analysis of similarities and differences between childhood apraxia of speech and neurodevelopmental disorders in children

目的我们旨在多角度比较仅伴随语言障碍的儿童言语失用症(CAS)儿童与同时伴随其他神经发育障碍儿童的临床情况。方法连续选择了黄河三门峡医院从2012年—2021年期间被诊断为CAS的16例病例,CAS患者的诊断基于全面评估。当儿童在一项或多项标准化语言测试中得分低于同龄平均水平1.5个标准差时,就被诊断为语言障碍(LI)。根据仅存在LI或同时存在其他神经发育障碍(NDD)的情况,将患儿分为CL组和CLC组。收集患者的临床资料,包括性别、评估时年龄、非语言智力、初语年龄、早期大肌肉运动发育情况等。对两组患者进行言语-语言评估、基因和遗传学评估以及颅脑影像学评估。结果16例患儿评估时平均年龄(6.9±3.7)岁,包括10例男孩和6例女孩。CL组共5例,CLC组共11例,性别在两组间差异无统计学(P=0.801)。平均初语年龄为(24.7±11.4)个月,两组间差异无统计学(P=0.094)。平均非语言智力得分为(91.0±19.9),CL组儿童的非语言智力明显高于CLC组(P<0.001)。37.5%的患者出现早期大肌肉运动发育延迟,且CLC组的发生率高于CL组(P=0.014)。共11例患儿接受了基因检测,其中5(44.4%)例患儿出现了异常,且在CLC组儿童中的发生率较高(P<0.001)。另外50.0%的病例存在口语/书面语言障碍家族病史,CL组患儿阳性家族病史比例(60.0%)显著高于CLC组患儿(45.5%)(P=0.046)。两组患儿在言语方面的特征没有显著差异(P>0.05)。37.5%儿童出现感受性语言障碍,且CLC组儿童的感受性语言障碍的比例高于CL组儿童,且差异有统计学意义(P=0.001)。两组间脑MR结构/信号异常分布差异无统计学意义(P=0.523)。结论多角度分析CAS与其合并神经发育障碍可能在非语言智力、早期大肌肉运动发育、基因遗传等方面存在差异,应给予相应关注,以便对患儿进行个性化管理。

Objective We aimed to compare the clinical outcomes of children with childhood apraxia of speech(CAS)and other neurodevelopmental disorders from multiple perspectives.Methods Sixteen cases diagnosed with CAS in our hospital from 2012 to 2021 were selected consecutively,and the diagnosis of CAS was based on a comprehensive assessment.Children are diagnosed with language impairment(LI)when they score 1.5 standard deviations below the average for their age on one or more standardized language tests.The children were divided into CL group and CLC group according to the presence of LI alone or co-existence of complex neurodevelopmental comorbidities(NDD).Clinical data were collected,including gender,age at assessment,age at first language,non-verbal intelligence,and early gross motor development,etc.Speech-language assessment,genetic and genetic assessment,and craniocerebral imaging assessment were performed in both groups.Results The mean age of the 16 children was(6.9±3.7)years at the time of assessment,including 10 boys and 6 girls.There were 5 cases in the CL group and 11 cases in the CLC group,and there was no statistical difference in gender between the two groups(P=0.801).The mean age was(24.7±11.4)months,and there was no significant difference between the two groups(P=0.094).The average score of non-verbal intelligence was(91.0±19.9),and the non-verbal intelligence of the CL group was significantly higher than that of the CLC group(P<0.001).37.5%of patients had early delay of great muscle motor development,and the incidence of CLC group was higher than CL group(P=0.014).Eleven children underwent genetic testing,of which 5(44.4%)had abnormalities,with a high incidence in the CLC group(P<0.001).In addition,50.0%of the patients had a family history of oral/written language impairment,and the percentage of positive family history in the CL group(60.0%)was significantly higher than that in the CLC group(45.5%)(P=0.046).There was no significant difference in speech between the two groups(P>0.05).37.5%of the children had receptive language impairment,and the proportion of the CLC group was higher than the CL group,and the difference was statistically significant(P=0.001).There was no significant difference in the abnormal distribution of MR structure/signal between the two groups(P=0.523).Conclusion From multiple perspectives,CAS and its combination with neurodevelopmental disorders may have differences in nonverbal intelligence,early large muscle motor development,genetic inheritance,and so on.Corresponding attention should be paid to personalized management of children.