Language impairment in sporadic and familial (type 8) amyotrophic lateral sclerosis: A comparative study

Caroline M. de Araújo PhD, Cássia de Alcântara PhD, Mariana A. Alencar PhD, Natália A. S. da Gama MSc, Marcelo M. Cruzeiro MD, PhD, Marcondes C. França Jr MD, PhD, Antônio Jaeger PhD, Sarah T. Camargos MD, PhD, Thais H. Machado PhD, Leonardo Cruz de Souza MD, PhD

Introduction/Aims Language is frequently affected in patients with sporadic amyotrophic lateral sclerosis (sALS), with reduced performance in naming, syntactic comprehension, grammatical expression, and orthographic processing. However, the language profile of patients with familial type 8 ALS (ALS8), linked to p.P56S VAPB mutation, remains unclear. We investigated language in patients with ALS8 by examining their auditory comprehension and verbal production.

Methods We included three groups of participants: (1) patients with sALS (n=20), (2) patients with familial ALS8 (n=22), and (3) healthy controls (n=21). The groups were matched for age, sex, and education level. All participants underwent a comprehensive language battery, including the Boston Diagnostic Aphasia Examination, the reduced Token test, letter fluency, categorical fluency (animals), word definition from the Cambridge Semantic Memory Research Battery, and a narrative discourse analysis. Participants also were evaluated using Addenbrooke's Cognitive Exam-Revised Version, the Hospital Anxiety and Depression Scale, and the ALS Functional Rating Scale-Revised.

Results Compared to controls, sALS and ALS8 patients had impaired performance on oral (syntactic and phonological processing) comprehension and inappropriate discourse cohesion. sALS and ALS8 did not differ in any language measure. There was no correlation between language scores and functional and psychiatric scales.

Discussion ALS8 patients exhibit language deficits that are independent of motor features. These findings are consistent with the current evidence suggesting that ALS8 has prominent non-motor features.

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