Natural History, Trial Readiness and Gene Discovery: Advances in Patient Registries for Neuromuscular Disease

Adv Exp Med Biol. 2017:1031:97-124. doi: 10.1007/978-3-319-67144-4_5.

Abstract

Inherited neuromuscular diseases (NMDs) are genetic disorders that affect the skeletal muscles or the nerves controlling muscle function. With a new generation of diagnostic options and recent advances in translational research improving the opportunities for therapy development for these rare conditions, capturing patient information in databases collecting a range of clinical and genetic data together with contact details has assumed an increasingly important role in trial planning and recruitment as well as natural history data collection. Here we provide an overview of a decade of patient registration activities in the NMD field, with a particular focus on patient registries set up with trial readiness in mind. A summary is provided of databases collecting precise genetic information focused on confirming the causative mutation and their evolution into registries that combine genetic data with additional clinical information useful for trial feasibility and recruitment. Use of these systems for a range of purposes beyond trial recruitment, including natural history assessment, care standards monitoring, genotype-phenotype correlation and disease burden evaluation is also described within the context of research networks (TREAT-NMD) and European Reference Networks (ERN-EURO-NMD). New initiatives including registries using controlled vocabularies for computational accessibility that focus on phenotypic data capture for gene discovery are analysed, and examples of the lessons learned at every stage are provided in order to allow new patient registration initiatives to benefit from the extensive experience gained.

Keywords: Data sharing; Genetic databases; Interoperability; NMD; Natural history; Neuromuscular disease; Next-generation sequencing; Patient registries; Phenotype ontologies; Trial readiness; Trial recruitment.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Clinical Trials as Topic / methods*
  • Databases, Factual
  • Genetic Predisposition to Disease
  • Genomics / methods*
  • Humans
  • Neuromuscular Diseases* / diagnosis
  • Neuromuscular Diseases* / epidemiology
  • Neuromuscular Diseases* / genetics
  • Neuromuscular Diseases* / therapy
  • Phenotype
  • Prognosis
  • Rare Diseases* / diagnosis
  • Rare Diseases* / epidemiology
  • Rare Diseases* / genetics
  • Rare Diseases* / therapy
  • Registries*
  • Research Design*