Psychological characteristics of patients with myotonic dystrophy type 1
| Autor: | J. F. Gagnon, Louis Richer, Jean Mathieu, Stéphane Jean, J. A. Bertrand, Luc Laberge, Cynthia Gagnon |
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| Rok vydání: | 2014 |
| Předmět: |
Adult
Male medicine.medical_specialty media_common.quotation_subject Neuropsychological Tests Myotonic dystrophy Disease severity Surveys and Questionnaires medicine Humans Myotonic Dystrophy Personality Cognitive skill Big Five personality traits Muscular dystrophy Psychiatry Suicidal ideation media_common Mental Disorders Cognition General Medicine Middle Aged medicine.disease 3. Good health Phenotype Neurology Female Neurology (clinical) medicine.symptom Psychology |
| Zdroj: | Acta Neurologica Scandinavica. 132:49-58 |
| ISSN: | 0001-6314 |
| DOI: | 10.1111/ane.12356 |
| Popis: | Bertrand JA, Jean S, Laberge L, Gagnon C, Mathieu J, Gagnon JF,Richer L. Psychological characteristics of patients with myotonicdystrophy type 1.Acta Neurol Scand: DOI 10.1111/ane.12356.© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.Objectives – Myotonic dystrophy type 1 (DM1) is the most commonadult-onset muscular dystrophy. It is associated with motor symptomsbut patients also display non-motor symptoms such as particularpersonality traits. Studies have reported mixed results aboutpersonality characteristics which may be attributable to small samplesizes, different disease severity of groups studied, and use of differentquestionnaires or method. This study aimed to describe thepsychological characteristics of a large cohort of patients with DM1,to characterize those at risk of developing a psychiatric disorder, andto compare characteristics between two DM1 phenotypes, a mild andmore severe adult-onset phenotype. Methods – Two hundred patientswith DM1 (152 adult-onset; 48 mild) were asked to completequestionnaires assessing personality traits, psychological symptoms,self-esteem, and suicidal risk. Neurological and neuropsychologicalassessments were performed to compare personality characteristics toclinical and cognitive measures. Results – Patients with DM1 globallyshowed personality traits and psychological symptoms in the averagerange compared to normative data, with normal levels of self-esteemand suicidal ideation. However, 27% of patients were found to be athigh risk of developing a psychiatric disorder. Moreover,psychological traits differed across phenotypes, with the most severephenotype tending to show more severe psychological symptoms. Thepresence of higher phobic anxiety and lower self-esteem wasassociated with lower education, a higher number of CTG repeats,more severe muscular impairment, and lower cognitive functioning(P < 0.001). Conclusions – Different phenotypes should thus be takeninto account in clinical settings for individual management of patientsand optimizing therapeutic success. |
| Databáze: | OpenAIRE |
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