Abstract
Despite recent interest in cognitive changes in patients with amyotrophic lateral sclerosis (ALS), investigations of language function looking at the level of word, sentence and discourse processing are relatively scarce. Data were obtained from 26 patients with sporadic ALS and 26 healthy controls matched for age, education, gender, anxiety, depression and executive function performance. Standardized language tasks included confrontation naming, semantic access, and syntactic comprehension. Quantitative production analysis (QPA) was used to analyse connected speech samples of the Cookie Theft picture description task. Results showed that the ALS patients were impaired on standardized measures of grammatical comprehension and action/verb semantics. At the level of discourse, ALS patients were impaired on measures of syntactic complexity and fluency; however, the latter could be better explained by disease related factors. Discriminant analysis revealed that syntactic measures differentiated ALS patients from controls. In conclusion, patients with ALS exhibit deficits in receptive and expressive language on tasks of comprehension and connected speech production, respectively. Our findings suggest that syntactic processing deficits seem to be the predominant feature of language impairment in ALS and that these deficits can be detected by relatively simple language tests.
Highlights
There is currently great interest in characterizing the cognitive profiles of patients with amyotrophic lateral sclerosis (ALS) who do not meet criteria for frontotemporal lobar degeneration (FTLD) [1]
The objective of this study was to clarify whether ALS patients without FTLD presented specific changes in connected speech production evident in a description of the Cookie Theft Picture, as well as other linguistic deficits as measured by a variety of other formal standardized language tests
The findings of the present study are consistent with literature indicating expressive and receptive language disturbances in ALS [6,8,9,10,11,12,13].We found that patients with ALS showed deficits in speech production fluency and in semantic and syntactic comprehension
Summary
There is currently great interest in characterizing the cognitive profiles of patients with amyotrophic lateral sclerosis (ALS) who do not meet criteria for frontotemporal lobar degeneration (FTLD) [1]. Considering that degeneration in ALS affects primarily posterior frontal regions, this line of research has mainly focused on the presence of executive functions In recent years research has shown that cognitive deficits go beyond an executive dysfunction syndrome and reveal that language is affected [5,6,7]. The specific nature of these deficits in ALS is not yet clear. This is critical because spontaneous connected speech is an essential part of everyday communication.
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