Cognitive impairment and quality of life in patients with myotonic dystrophy type 1

Abstract

Introduction: This study sought to clarify whether specific cognitive abilities are impaired in patients with myotonic dystrophy type 1 (DM1) as well as to investigate the relationships among quality of life (QoL), cognitive function, and psychological factors.

Methods: Sixty patients with DM1 were evaluated on cognitive functioning (abstract reasoning, attention/working memory, executive function, processing speed, and visuoconstructive ability), apathy, depression, excessive daytime sleepiness, fatigue, and QoL. QoL was assessed by 2 domains of the Muscular Dystrophy Quality of Life Scale (Psychosocial Relationships and Physical Functioning and Health).

Results: More than half of the patients exhibited cognitive impairment in attention/working memory, executive function, processing speed, and visuoconstructive ability. The Psychosocial Relationships factor was associated with processing speed, attention/working memory, and apathy, whereas depression and fatigue were associated with 2 QoL domains.

Discussion: Our study identified specific cognitive impairments in DM1. Specific cognitive functions and psychological factors may be potential contributors to QoL. Muscle Nerve 57: 742-748, 2018.

Keywords: central nervous system; cognitive deficit; depression; fatigue; myotonic dystrophy type 1; quality of life.