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Background: Amyotrophic lateral sclerosis (ALS) has long been considered a pure motor neurodegenerative disease. However, now, extra-motor manifestations such as cognitive-behavioral disorders are considered not rare and are even a severity factor of the disease. Experiencing anosognosia (i.e., the inability to recognize neurological symptoms) might affect care and treatment compliance in ALS. Regardless, this pivotal feature has been little investigated., Objectives: By comparing patients' and caregivers' reports, we analysed whether patients with ALS would experience a lack of awareness about their executive disorders and their apathy symptoms., Methods: From the ALS reference center in Paris, we included 85 patients (47 men, mean [SD] age 60.5 [12] years and ALS-Functional Rating Scale-revised score 8 to 46) and their primary family caregivers who all completed the Dysexecutive Questionnaire (DEX) and the Apathy Evaluation Scale (AES). Overall scores and answers were compared by agreement/disagreement statistical methods., Results: Caregivers reported higher levels of cognitive-behavioral disorders than did patients, but reports matched when cognitive-behavioral disorders were absent or mild. With published DEX and AES cutoffs, 32% and 51% of patients had executive disorders and apathy, respectively. In these patients with significant impairment, Bland-Altman plots (i.e., visual display agreement that represents the difference between the patient's and caregiver's scores as a function of their average) showed a strong discrepancy between joint reports: patients underestimated their symptoms by a mean bias of -6.81 DEX points (95% confidence interval -11.88, -1.75) and -8.85 AES points (95% confidence interval -11.72, -5.98). We found no clear relationship between bulbar or spinal ALS subtypes and anosognosia., Conclusions: ALS patients with a cognitive-behavioral phenotype show anosognosia by a mismatch between self and proxy reports, which warrants further investigation in neuroimaging. Systematic longitudinal screening of anosognosia is needed to propose targeted psychoeducation in patient-caregiver dyads showing disagreement., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: Aphasia severity is known to affect quality of life (QoL) in stroke patients, as is mood disorders, functional limitations, limitations on activities of daily life, economic status and level of education. However, communication limitation or fatigue has not been explored in this specific population., Objective: We aimed to investigate whether these factors were associated with QoL in patients with aphasia after stroke., Methods: Patients with aphasia were included from April 2014 to November 2017 after a first stroke and were followed for 2 years post-stroke. QoL was assessed at follow-up by the French Sickness Impact Profile 65 (SIP-65). We explored predictors such as mood disorders, communication impairment, fatigue, limitations on activities of daily life, and aphasia severity in addition to socio-demographic factors., Results: We included 32 individuals (22 men; mean age 60.7 [SD 16.6] years) with aphasia after a first stroke. Poor QoL as assessed by the SIP-65 was significantly associated (Pearson correlations) with increased severity of aphasia initially (P=0.008) and at follow-up (P=0.01); increased communication activity limitations at follow-up (P<0.001); increased limitations on activities of daily life at baseline (P=0.008) and follow-up (P<0.001); increased fatigue at follow-up (P=0.001); and increased depression symptoms at follow-up (P=0.001). On multivariable analysis, QoL was associated with communication activity limitations, limitations on activities of daily life, fatigue and depression, explaining more than 75% of the variance (linear regression R 2 =0.756, P<0.001). The relative importance in predicting the variance was 32% for limitations on activities of daily life, 21% fatigue, 23% depression and 24% communication activity limitations., Conclusion: Aphasia severity, mood disorders and functional limitations may have a negative effect on QoL in patients with aphasia. Also, for the first time, we show that fatigue has an important impact on QoL in this population. Specific management of this symptom might be beneficial and should be explored in future studies., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)