Anosognosia in amyotrophic lateral sclerosis: A cross-sectional study of 85 individuals and their relatives.
Ann Phys Rehabil Med
; 64(5): 101440, 2021 Sep.
Article
em En
| MEDLINE
| ID: mdl-33065300
ABSTRACT
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.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Doenças Neurodegenerativas
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Agnosia
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Apatia
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Esclerose Lateral Amiotrófica
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article