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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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Consensus-based care recommendations for adults with myotonic dystrophy type 2.

Benedikt Schoser1, Federica Montagnese1, Guillaume Bassez1

  • 1Ludwig-Maximilians- Universität (BS); Friedrich-Baur-Institut (FM), Munich, Germany; Institut de Myologie (GB), Paris, France; U.O. Neurologia (BF), IRCCS Policlinico San Donato, Milan, Italy; Vall d'Hebron University Hospital (JG), Barcelona, Spain; University of Rochester (CH, JH, RM, CT), Rochester, NY; University Hospital of Bonn (CK), Germany; Medical University of Warsaw (AK-P), Poland; University of Texas (RK) MD Anderson cancer center; Medical University of Warsaw (AL), Poland; Department of Biomedical Sciences for health (GM), University of Milan, Italy; Tampere University (BU), Finland; Myotonic Dystrophy Foundation (PF), San Francisco.

Neurology. Clinical Practice
|October 5, 2019
PubMed
Summary

Care recommendations for myotonic dystrophy type 2 (DM2), a rare muscle disease, have been developed by international experts. These guidelines aim to standardize and improve treatment for DM2 patients lacking causal therapy.

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Area of Science:

  • Neurology
  • Rare Diseases
  • Genetics

Background:

  • Myotonic dystrophy type 2 (DM2) is a rare, progressive multisystem disorder primarily impacting skeletal muscle.
  • Currently, no causal therapy exists for DM2, making symptomatic treatment crucial for managing complications.
  • Evidence-based guidelines for DM2 patient care are lacking.

Purpose of the Study:

  • To develop consensus-based care recommendations for myotonic dystrophy type 2 (DM2) patients.
  • To provide guidance for medical practitioners in managing this rare multisystem disease.
  • To address the absence of established evidence-based guidelines for DM2 care.

Main Methods:

  • The Myotonic Dystrophy Foundation (MDF) convened 15 international clinicians with extensive DM2 patient care experience.
  • A consensus-building process using the single text procedure was employed.
  • The methodology mirrored the successful development of recommendations for myotonic dystrophy type 1.

Main Results:

  • Consensus-based care recommendations for DM2 patients were generated.
  • A comprehensive 55-page document detailing these recommendations was produced.
  • A concise 4-page Quick Reference Guide was created for immediate clinical use.

Conclusions:

  • The developed recommendations will standardize and enhance the care of DM2 patients.
  • These guidelines will facilitate improved management, especially in centers lacking neuromuscular specialists.
  • The recommendations aim to optimize symptomatic treatment and limit disease-related complications.