Developmental Profiles in Children and Young Adults with Alexander Disease.

The study aims to describe the developmental profile of children and young adults with Alexander disease [AxD] infantile form, analyzing their clinical features, adaptive behavior and neuropsychological skills. Participants were eight children or young adults (Mean age = 11 years; SD = 6.86; range =...

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Publicado en:Developmental Neurorehabilitation Vol. 26; no. 4; pp. 253 - 262
Autores principales: Zampini, Laura, Draghi, Lara, Zanchi, Paola
Formato: research tables/charts Journal Article
Publicado: Taylor & Francis Ltd May2023
Acceso en línea:Ver este registro en EBSCOhost
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      dt: May2023
      vid: 26
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      pub: Taylor & Francis Ltd
      place: Philadelphia, Pennsylvania
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        10.1080/17518423.2023.2209834
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        atl: Developmental Profiles in Children and Young Adults with Alexander Disease.
      aug:
        au:
          Zampini, Laura
          Draghi, Lara
          Zanchi, Paola
        affil: Department of Psychology, University of Milano-Bicocca, Milan, Italy
      sug:
        subj:
          Heredodegenerative Disorders, Nervous System
          Child Development
          Adolescent Development
          Adaptation, Psychological
          Neuropsychological Tests
          Parental Attitudes
          Parents Psychosocial Factors
          Human
          Male
          Female
          Child, Preschool
          Child
          Adolescence
          Young Adult
          Multimethod Studies
          Clinical Competence
          Semi-Structured Interview
          Questionnaires
          Descriptive Statistics
          Funding Source
          Child, Preschool: 2-5 years
          Child: 6-12 years
          Adolescent: 13-18 years
          Male
          Female
      ab: The study aims to describe the developmental profile of children and young adults with Alexander disease [AxD] infantile form, analyzing their clinical features, adaptive behavior and neuropsychological skills. Participants were eight children or young adults (Mean age = 11 years; SD = 6.86; range = 5–23) and their parents. A multi-method approach was adopted to assess participant competencies: (1) an online parent survey, (2) a semi-structured interview with parents, and (3) a direct assessment of the participant's neuropsychological skills. Only four parents and their children completed all measures, and a common developmental profile could not be identified. The participants experienced substantial impairment in gross-motor skills, memory and narrative macrostructure. Most parents reported a regressive trend in at least one area. The high individual variability and the regressive trend highlight the need for an accurate and periodic assessment of each individual's developmental profile.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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