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Lexical Decision Task for Studying Written Word Recognition in Adults with and without Dementia or Mild Cognitive Impairment
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Language Skills in Patients With Alexander Disease.

Debra Levin1, Jordan Levin2, Joshua Joung2

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|November 18, 2025
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This summary is machine-generated.

Children with Alexander disease (AxD) show varying language skills. Cerebral AxD patients have significant deficits, while intermediate and bulbospinal subtypes perform better, indicating disease subtype impacts communication abilities.

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Area of Science:

  • Neuroscience
  • Genetics
  • Pediatric Neurology

Background:

  • Alexander disease (AxD) is a rare genetic leukoencephalopathy characterized by mutations in the glial fibrillary acidic protein (GFAP) gene.
  • Language development is often affected in neurodegenerative disorders, but specific patterns in AxD subtypes are not well-defined.

Purpose of the Study:

  • To investigate the language skills of children diagnosed with Alexander disease (AxD).
  • To determine if specific AxD disease subtypes (cerebral, intermediate, bulbospinal) or patient age influence language performance.
  • To correlate language assessment scores with disease progression markers.

Main Methods:

  • Eighty-two participants with confirmed AxD (GFAP testing) were categorized into cerebral (n=56), intermediate (n=9), and bulbospinal (n=17) phenotypes.
  • Language abilities were assessed using the Preschool Language Scale-Fifth Edition (PLS-5), Clinical Evaluation of Language Fundamentals-Fifth Edition (CELF-5), and Peabody Picture Vocabulary Test-Fourth Edition (PPVT-4).
  • The Communication Function Classification System (CFCS) evaluated functional communication; statistical analyses included Wilcoxon signed-ranks and Kruskal-Wallis tests.

Main Results:

  • Mean PLS-5 Total Language Score (TLS) was 70.10 (SD=17.62).
  • CELF-5 Core Language Scores (CLS) differed significantly by phenotype: cerebral (M=57.22), intermediate (M=92.50), and bulbospinal (M=91.56).
  • Cerebral AxD patients showed a negative correlation between PLS-5 TLS and age at first visit (rho=-.367, p=.017). CELF-5 CLS and PPVT-4 scores correlated with age at first neurological symptom (rho=.576, p=.003 and rho=.336, p=.011, respectively).

Conclusions:

  • Significant language deficits were observed in the cerebral AxD phenotype.
  • Intermediate and bulbospinal AxD subtypes generally demonstrated stronger language abilities, though some patients exhibited deficits.
  • Findings highlight the functional impact of communication deficits in AxD, informing tailored interventions and accommodations in patient treatment plans.