Lottie D Morison, Ruth Braden, David J Amor, Angela T Morgan
{"title":"与FOXP2相关的7q31缺失相关的言语和语言障碍","authors":"Lottie D Morison, Ruth Braden, David J Amor, Angela T Morgan","doi":"10.1002/ajmg.a.64190","DOIUrl":null,"url":null,"abstract":"<p><p>Some 7q31 deletions encompass FOXP2, a gene long associated with speech and language disorders. Intragenic pathogenic FOXP2 variants cause FOXP2-related speech and language disorder, which has been well characterized in the literature. Conversely, the phenotype associated with 7q31 deletions is neglected. Here we characterize the phenotype of eight individuals (4 males) with 7q31 deletions (median age 4 years, 3 months, range 1-32 years). Deletion size ranged from 6.8 to 15.2 Mb. All had protracted speech and language milestones, and those with larger deletions had little to no speech. All verbal individuals had childhood apraxia of speech (5/5, 100%). Participants used augmentative and alternative communication (AAC) including key word sign (5/8, 63%), and low-tech (6/8, 75%) and high-tech (4/8, 50%) systems. Oral and written language impairment was universal. The larger the deletion size, the poorer an individual's language skills (p = 0.03, p < 0.05). Daily living, socialization, and motor skills were also impaired. Cognition ranged from average to severely impaired. Childhood feeding impairment (50%), sleep disturbance (38%), structural brain abnormalities (38%), and autism (25%) were noted. All individuals received one or more allied health therapies. Speech and language impairments emphasize the need for early, tailored speech therapy, including literacy and AAC interventions, for individuals with 7q31 deletions.</p>","PeriodicalId":7507,"journal":{"name":"American Journal of Medical Genetics Part A","volume":" ","pages":"e64190"},"PeriodicalIF":1.7000,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Speech and Language Disorders Associated With 7q31 Deletions Implicating FOXP2.\",\"authors\":\"Lottie D Morison, Ruth Braden, David J Amor, Angela T Morgan\",\"doi\":\"10.1002/ajmg.a.64190\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Some 7q31 deletions encompass FOXP2, a gene long associated with speech and language disorders. Intragenic pathogenic FOXP2 variants cause FOXP2-related speech and language disorder, which has been well characterized in the literature. Conversely, the phenotype associated with 7q31 deletions is neglected. Here we characterize the phenotype of eight individuals (4 males) with 7q31 deletions (median age 4 years, 3 months, range 1-32 years). Deletion size ranged from 6.8 to 15.2 Mb. All had protracted speech and language milestones, and those with larger deletions had little to no speech. All verbal individuals had childhood apraxia of speech (5/5, 100%). Participants used augmentative and alternative communication (AAC) including key word sign (5/8, 63%), and low-tech (6/8, 75%) and high-tech (4/8, 50%) systems. Oral and written language impairment was universal. The larger the deletion size, the poorer an individual's language skills (p = 0.03, p < 0.05). Daily living, socialization, and motor skills were also impaired. Cognition ranged from average to severely impaired. Childhood feeding impairment (50%), sleep disturbance (38%), structural brain abnormalities (38%), and autism (25%) were noted. All individuals received one or more allied health therapies. Speech and language impairments emphasize the need for early, tailored speech therapy, including literacy and AAC interventions, for individuals with 7q31 deletions.</p>\",\"PeriodicalId\":7507,\"journal\":{\"name\":\"American Journal of Medical Genetics Part A\",\"volume\":\" \",\"pages\":\"e64190\"},\"PeriodicalIF\":1.7000,\"publicationDate\":\"2025-07-26\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American Journal of Medical Genetics Part A\",\"FirstCategoryId\":\"99\",\"ListUrlMain\":\"https://doi.org/10.1002/ajmg.a.64190\",\"RegionNum\":4,\"RegionCategory\":\"生物学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"GENETICS & HEREDITY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Medical Genetics Part A","FirstCategoryId":"99","ListUrlMain":"https://doi.org/10.1002/ajmg.a.64190","RegionNum":4,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"GENETICS & HEREDITY","Score":null,"Total":0}
Speech and Language Disorders Associated With 7q31 Deletions Implicating FOXP2.
Some 7q31 deletions encompass FOXP2, a gene long associated with speech and language disorders. Intragenic pathogenic FOXP2 variants cause FOXP2-related speech and language disorder, which has been well characterized in the literature. Conversely, the phenotype associated with 7q31 deletions is neglected. Here we characterize the phenotype of eight individuals (4 males) with 7q31 deletions (median age 4 years, 3 months, range 1-32 years). Deletion size ranged from 6.8 to 15.2 Mb. All had protracted speech and language milestones, and those with larger deletions had little to no speech. All verbal individuals had childhood apraxia of speech (5/5, 100%). Participants used augmentative and alternative communication (AAC) including key word sign (5/8, 63%), and low-tech (6/8, 75%) and high-tech (4/8, 50%) systems. Oral and written language impairment was universal. The larger the deletion size, the poorer an individual's language skills (p = 0.03, p < 0.05). Daily living, socialization, and motor skills were also impaired. Cognition ranged from average to severely impaired. Childhood feeding impairment (50%), sleep disturbance (38%), structural brain abnormalities (38%), and autism (25%) were noted. All individuals received one or more allied health therapies. Speech and language impairments emphasize the need for early, tailored speech therapy, including literacy and AAC interventions, for individuals with 7q31 deletions.
期刊介绍:
The American Journal of Medical Genetics - Part A (AJMG) gives you continuous coverage of all biological and medical aspects of genetic disorders and birth defects, as well as in-depth documentation of phenotype analysis within the current context of genotype/phenotype correlations. In addition to Part A , AJMG also publishes two other parts:
Part B: Neuropsychiatric Genetics , covering experimental and clinical investigations of the genetic mechanisms underlying neurologic and psychiatric disorders.
Part C: Seminars in Medical Genetics , guest-edited collections of thematic reviews of topical interest to the readership of AJMG .