The myotonic dystrophy experience: a North American cross-sectional study

Muscle Nerve. 2019 Apr;59(4):457-464. doi: 10.1002/mus.26420. Epub 2019 Feb 5.

Abstract

Introduction: Myotonic dystrophy (DM) is a chronic, multisystemic, neurological condition. Patients and caregivers are uniquely suited to identify what symptoms are most important and highlight the unmet needs that are most relevant to DM.

Methods: We conducted a North American, cross-sectional study of people with DM type-1, congenital DM, and DM type-2 and their family members. We sent patients and caregivers separate surveys to identify and quantitate the issues of greatest importance, examine the differences between groups, and identify the most important challenges experienced by this population.

Results: 1,180 people with DM and 402 family members/caregivers responded to the surveys. They reported considerable physical and cognitive symptoms, extensive diagnostic delays, and varying clinical phenotypes on the basis of DM type.

Discussion: Marked disease burden and numerous unmet needs exist in DM. These needs vary based on DM type and highlight the complex clinical phenotypes of these neurological disorders. Muscle Nerve 59:457-464, 2019.

Keywords: caregiver; myotonic dystrophy; neuromuscular disease; patient report; unmet needs.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Activities of Daily Living
  • Adolescent
  • Adult
  • Caregivers
  • Cognition Disorders / etiology
  • Cognition Disorders / psychology
  • Cost of Illness
  • Cross-Sectional Studies
  • Delayed Diagnosis
  • Employment
  • Family
  • Female
  • Humans
  • Income
  • Male
  • Middle Aged
  • Myotonic Dystrophy / epidemiology
  • Myotonic Dystrophy / psychology*
  • Myotonic Dystrophy / therapy*
  • North America / epidemiology
  • Prevalence
  • Socioeconomic Factors
  • Surveys and Questionnaires
  • Young Adult