View in EDS PDF Full Text

Language Skills in Patients With Alexander Disease.

Saved in:
Bibliographic Details
Title: Language Skills in Patients With Alexander Disease.
Authors: Levin, Debra1 levind@chop.edu, Levin, Jordan2, Joung, Joshua2, Liu, Geraldine W.2, Donaher, Joseph1, Faig, Walter3, Waldman, Amy T.2,4
Source: American Journal of Speech-Language Pathology. Jan2026, Vol. 35 Issue 1, p211-225. 15p.
Subject Terms: *Communicative competence, *Statistical correlation, *Data analysis, *Phonological awareness, *Brain, *Speech evaluation, *Vocabulary, *Language acquisition, Age of onset, Secondary analysis, Research funding, Kruskal-Wallis Test, Neurodegeneration, Age distribution, X-linked bulbo-spinal atrophy, Functional status, Descriptive statistics, Linguistics, Genetic disorders, Statistics, Data analysis software, Phenotypes, Nonparametric statistics, Biomarkers, Symptoms
Geographic Terms: Pennsylvania
Abstract: Purpose: This study aimed to investigate the language skills of children with Alexander disease (AxD) and determine whether disease subtypes and/or age contributes to variability in language performance. Method: Eighty-two participants with AxD, confirmed by GFAP testing, were enrolled, and the disease phenotype was determined using clinical and imaging features (56 cerebral, nine intermediate, and 17 bulbospinal). The participants’ language abilities were assessed using the Preschool Language Scale–Fifth Edition (PLS-5), the Clinical Evaluation of Language Fundamentals–Fifth Edition (CELF-5), and the Peabody Picture Vocabulary Test–Fourth Edition (PPVT-4), based on the patient’s age and linguistic level. The Communication Function Classification System was also used to determine functional communication abilities. Wilcoxon signed-ranks test or Kruskal–Wallis test was used to determine differences across groups. Results: The mean PLS-5 Total Language Score (TLS) was 70.10 (SD = 17.62). Performance on the CELF-5 varied by phenotype (Core Language Score [CLS]: cerebral, M = 57.22, SD = 19.89; intermediate, M = 92.50, SD = 20.54; bulbospinal, M = 91.56, SD = 19.51). PLS-5 performance was negatively correlated with age at the first research study visit for cerebral participants (TLS: rho = −.367, p = .017). CELF-5 and PPVT-4 scores, on the other hand, were not correlated with age at first visit but with age at first neurological symptom (CLS: rho = .576, p = .003; PPVT-4: rho = .336, p = .011). Conclusions: Cerebral patients demonstrated significant language deficits, whereas intermediate and bulbospinal subjects displayed stronger abilities, with deficits in some patients. These implications can be used to determine the functional impact of communication deficits and to provide accommodations and interventions in the treatment plans of patients with AxD. [ABSTRACT FROM AUTHOR]
Copyright of American Journal of Speech-Language Pathology is the property of American Speech-Language-Hearing Association and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Education Research Complete
FullText Links:
  – Type: pdflink
Text:
  Availability: 0
Header DbId: ehh
DbLabel: Education Research Complete
An: 190920660
AccessLevel: 6
PubType: Academic Journal
PubTypeId: academicJournal
PreciseRelevancyScore: 0
IllustrationInfo
Items – Name: Title
  Label: Title
  Group: Ti
  Data: Language Skills in Patients With Alexander Disease.
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22Levin%2C+Debra%22">Levin, Debra</searchLink><relatesTo>1</relatesTo><i> levind@chop.edu</i><br /><searchLink fieldCode="AR" term="%22Levin%2C+Jordan%22">Levin, Jordan</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Joung%2C+Joshua%22">Joung, Joshua</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Liu%2C+Geraldine+W%2E%22">Liu, Geraldine W.</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Donaher%2C+Joseph%22">Donaher, Joseph</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Faig%2C+Walter%22">Faig, Walter</searchLink><relatesTo>3</relatesTo><br /><searchLink fieldCode="AR" term="%22Waldman%2C+Amy+T%2E%22">Waldman, Amy T.</searchLink><relatesTo>2,4</relatesTo>
– Name: TitleSource
  Label: Source
  Group: Src
  Data: <searchLink fieldCode="JN" term="%22American+Journal+of+Speech-Language+Pathology%22">American Journal of Speech-Language Pathology</searchLink>. Jan2026, Vol. 35 Issue 1, p211-225. 15p.
– Name: Subject
  Label: Subject Terms
  Group: Su
  Data: *<searchLink fieldCode="DE" term="%22Communicative+competence%22">Communicative competence</searchLink><br />*<searchLink fieldCode="DE" term="%22Statistical+correlation%22">Statistical correlation</searchLink><br />*<searchLink fieldCode="DE" term="%22Data+analysis%22">Data analysis</searchLink><br />*<searchLink fieldCode="DE" term="%22Phonological+awareness%22">Phonological awareness</searchLink><br />*<searchLink fieldCode="DE" term="%22Brain%22">Brain</searchLink><br />*<searchLink fieldCode="DE" term="%22Speech+evaluation%22">Speech evaluation</searchLink><br />*<searchLink fieldCode="DE" term="%22Vocabulary%22">Vocabulary</searchLink><br />*<searchLink fieldCode="DE" term="%22Language+acquisition%22">Language acquisition</searchLink><br /><searchLink fieldCode="DE" term="%22Age+of+onset%22">Age of onset</searchLink><br /><searchLink fieldCode="DE" term="%22Secondary+analysis%22">Secondary analysis</searchLink><br /><searchLink fieldCode="DE" term="%22Research+funding%22">Research funding</searchLink><br /><searchLink fieldCode="DE" term="%22Kruskal-Wallis+Test%22">Kruskal-Wallis Test</searchLink><br /><searchLink fieldCode="DE" term="%22Neurodegeneration%22">Neurodegeneration</searchLink><br /><searchLink fieldCode="DE" term="%22Age+distribution%22">Age distribution</searchLink><br /><searchLink fieldCode="DE" term="%22X-linked+bulbo-spinal+atrophy%22">X-linked bulbo-spinal atrophy</searchLink><br /><searchLink fieldCode="DE" term="%22Functional+status%22">Functional status</searchLink><br /><searchLink fieldCode="DE" term="%22Descriptive+statistics%22">Descriptive statistics</searchLink><br /><searchLink fieldCode="DE" term="%22Linguistics%22">Linguistics</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+disorders%22">Genetic disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Statistics%22">Statistics</searchLink><br /><searchLink fieldCode="DE" term="%22Data+analysis+software%22">Data analysis software</searchLink><br /><searchLink fieldCode="DE" term="%22Phenotypes%22">Phenotypes</searchLink><br /><searchLink fieldCode="DE" term="%22Nonparametric+statistics%22">Nonparametric statistics</searchLink><br /><searchLink fieldCode="DE" term="%22Biomarkers%22">Biomarkers</searchLink><br /><searchLink fieldCode="DE" term="%22Symptoms%22">Symptoms</searchLink>
– Name: SubjectGeographic
  Label: Geographic Terms
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22Pennsylvania%22">Pennsylvania</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Purpose: This study aimed to investigate the language skills of children with Alexander disease (AxD) and determine whether disease subtypes and/or age contributes to variability in language performance. Method: Eighty-two participants with AxD, confirmed by GFAP testing, were enrolled, and the disease phenotype was determined using clinical and imaging features (56 cerebral, nine intermediate, and 17 bulbospinal). The participants’ language abilities were assessed using the Preschool Language Scale–Fifth Edition (PLS-5), the Clinical Evaluation of Language Fundamentals–Fifth Edition (CELF-5), and the Peabody Picture Vocabulary Test–Fourth Edition (PPVT-4), based on the patient’s age and linguistic level. The Communication Function Classification System was also used to determine functional communication abilities. Wilcoxon signed-ranks test or Kruskal–Wallis test was used to determine differences across groups. Results: The mean PLS-5 Total Language Score (TLS) was 70.10 (SD = 17.62). Performance on the CELF-5 varied by phenotype (Core Language Score [CLS]: cerebral, M = 57.22, SD = 19.89; intermediate, M = 92.50, SD = 20.54; bulbospinal, M = 91.56, SD = 19.51). PLS-5 performance was negatively correlated with age at the first research study visit for cerebral participants (TLS: rho = −.367, p = .017). CELF-5 and PPVT-4 scores, on the other hand, were not correlated with age at first visit but with age at first neurological symptom (CLS: rho = .576, p = .003; PPVT-4: rho = .336, p = .011). Conclusions: Cerebral patients demonstrated significant language deficits, whereas intermediate and bulbospinal subjects displayed stronger abilities, with deficits in some patients. These implications can be used to determine the functional impact of communication deficits and to provide accommodations and interventions in the treatment plans of patients with AxD. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of American Journal of Speech-Language Pathology is the property of American Speech-Language-Hearing Association and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
PLink https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=ehh&AN=190920660
RecordInfo BibRecord:
  BibEntity:
    Identifiers:
      – Type: doi
        Value: 10.1044/2025_AJSLP-25-00185
    Languages:
      – Code: eng
        Text: English
    PhysicalDescription:
      Pagination:
        PageCount: 15
        StartPage: 211
    Subjects:
      – SubjectFull: Communicative competence
        Type: general
      – SubjectFull: Statistical correlation
        Type: general
      – SubjectFull: Data analysis
        Type: general
      – SubjectFull: Phonological awareness
        Type: general
      – SubjectFull: Brain
        Type: general
      – SubjectFull: Speech evaluation
        Type: general
      – SubjectFull: Vocabulary
        Type: general
      – SubjectFull: Language acquisition
        Type: general
      – SubjectFull: Age of onset
        Type: general
      – SubjectFull: Secondary analysis
        Type: general
      – SubjectFull: Research funding
        Type: general
      – SubjectFull: Kruskal-Wallis Test
        Type: general
      – SubjectFull: Neurodegeneration
        Type: general
      – SubjectFull: Age distribution
        Type: general
      – SubjectFull: X-linked bulbo-spinal atrophy
        Type: general
      – SubjectFull: Functional status
        Type: general
      – SubjectFull: Descriptive statistics
        Type: general
      – SubjectFull: Linguistics
        Type: general
      – SubjectFull: Genetic disorders
        Type: general
      – SubjectFull: Statistics
        Type: general
      – SubjectFull: Data analysis software
        Type: general
      – SubjectFull: Phenotypes
        Type: general
      – SubjectFull: Nonparametric statistics
        Type: general
      – SubjectFull: Biomarkers
        Type: general
      – SubjectFull: Symptoms
        Type: general
      – SubjectFull: Pennsylvania
        Type: general
    Titles:
      – TitleFull: Language Skills in Patients With Alexander Disease.
        Type: main
  BibRelationships:
    HasContributorRelationships:
      – PersonEntity:
          Name:
            NameFull: Levin, Debra
      – PersonEntity:
          Name:
            NameFull: Levin, Jordan
      – PersonEntity:
          Name:
            NameFull: Joung, Joshua
      – PersonEntity:
          Name:
            NameFull: Liu, Geraldine W.
      – PersonEntity:
          Name:
            NameFull: Donaher, Joseph
      – PersonEntity:
          Name:
            NameFull: Faig, Walter
      – PersonEntity:
          Name:
            NameFull: Waldman, Amy T.
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 01
              M: 01
              Text: Jan2026
              Type: published
              Y: 2026
          Identifiers:
            – Type: issn-print
              Value: 10580360
          Numbering:
            – Type: volume
              Value: 35
            – Type: issue
              Value: 1
          Titles:
            – TitleFull: American Journal of Speech-Language Pathology
              Type: main
ResultId 1