Primary progressive aphasia (PPA) is a form of early-onset dementia, characterised by a gradual decline in language abilities. PPA comprises three different subtypes with each subtype presenting...Show morePrimary progressive aphasia (PPA) is a form of early-onset dementia, characterised by a gradual decline in language abilities. PPA comprises three different subtypes with each subtype presenting unique linguistic impairments: nonfluent/agrammatic (nfvPPA), semantic (svPPA), and logopenic (lvPPA) PPA. However, diagnosing PPA in general, and specifically these variants, currently remains clinically challenging. To address this, the Mini Linguistic State Examination was adapted to the Dutch language (MLSE-NL). The MLSE-NL is a screening tool to evaluate language deficits related to PPA, measuring five different domains: motor speech, phonology, semantics, syntax, and working memory. This study aimed to validate and determine the effectiveness of MLSE-NL in distinguishing between PPA variants. A cross-sectional study was conducted with 99 participants, including 21 patients diagnosed with PPA and 78 cognitively healthy controls. A psychometric evaluation of the MLSE-NL was performed to evaluate the reliability and validity of the test. Furthermore, differences in performance on the MLSE-NL across PPA variants and the control group were analysed. Results showed that the MLSE-NL demonstrated robust psychometric properties, exhibiting high inter-rater reliability and strong convergent validity compared to other validated language tests. Significant differences were observed in the MLSE-NL total and subdomains scores between patients with PPA and control groups. Patients with PPA exhibited lower total scores and domain-specific scores on the MLSE-NL compared to cognitively healthy controls. Notably, within the motor speech and working memory domains, significant differences were observed within the PPA subtypes; nfvPPA, svPPA and lvPPA. No significant differences were found in the other domains. The findings of the current study are in line with outcomes from the original English version of the MLSE. Results showed that the MLSE is robust for use in another language and cultural context. When interpreting the results of the current study, the small sample size should be taken into account. In conclusion, the MLSE-NL emerges as a reliable and valid instrument for diagnosing and distinguishing PPA variants within the Dutch-speaking population. Future research with larger samples is necessary to validate these findings and expand the normative data.Show less
Background: The cognitive and neuropsychiatric deficits present in patients with the behavioral variant of Frontotemporal dementia (bvFTD) impacts their functionality in the activities of daily...Show moreBackground: The cognitive and neuropsychiatric deficits present in patients with the behavioral variant of Frontotemporal dementia (bvFTD) impacts their functionality in the activities of daily living (ADLs). These activities are divided into advanced, instrumental, and basic domains, which begin to be undermined throughout the dementia progression. The detection of the cognitive and behavioral factors involved in the functional impairment of each ADLs domain is crucial for the implementation of suitable treatments. Methods: A retrospective cross-sectional study was conducted with 27 patients with bvFTD in its early stage (< 2 years of evolution) and 32 healthy controls. A neuropsychological assessment was carried out wherein measures of cognitive function, neuropsychiatric symptoms, and functionality were obtained. The informant- report Technology-Activities of daily living questionnaire was used to assess the percentage of functional impairment on advanced, instrumental, and basic ADLs. Therefore, higher percentages imply worse performance on ADL. To identify the best determinants for each ADLs domain, three separate standard multiple regression analyses were performed, considering each functional impairment as the dependent variable and executive function, social cognition, disinhibition, and apathy as independent variables. Results: For the basic ADLs, a model that explains 28.2% of the variability was found, in which apathy (β = 0.33, p = .02) and disinhibition (β = 0.29, p = .04) were significant predictors of functional impairment. Concerning instrumental ADLs, the model produced accounted for 58.8% of the functional variability, with apathy (β = 0.70, p < .001) and executive function (β = -0.30, p = .04) as the main contributors. Finally, in terms of advanced ADLs, the model found explains 52.9% of variance, wherein only apathy was a significant predictor (β = 0.69, p < .001). Furthermore, social cognition deficits did not show an influence on functional performance Conclusion: The results of this study show the strong influence of neuropsychiatric features such as apathy and disinhibition with the functional impairment of patients with bvFTD in its early stage. Apathy is the primary determinant of functioning for all the ADLs domains. These results could guide the planning of tailored interventions and quality of life improvement.Show less
