Back to Search Start Over

Genetic neuromuscular disorders: what is the best that we can do?

Authors :
Gianina Ravenscroft
Royston Ong
Nigel G. Laing
Source :
Neuromuscular Disorders. 31:1081-1089
Publication Year :
2021
Publisher :
Elsevier BV, 2021.

Abstract

The major advances in genetic neuromuscular disorders in the last 30 years have been: (a) identification of the genetic basis for hundreds of these disorders, (b) through knowing the genes, understanding their pathobiology and (c) subsequent implementation of evidence-based treatments for some of the disorders. New genomic technologies are providing precision diagnosis, mode of inheritance and likely prognosis for more patients than ever before. Parents of children with a genetic diagnosis can then use preimplantation or prenatal diagnosis to avoid having further affected children if they wish. But is this the best we can do for genetic neuromuscular disorders? Since the 1980s, it has been argued it would be better to identify Duchenne muscular dystrophy carrier mothers, rather than diagnose their affected sons. Carrier screening for recessive disorders can identify couples with a high chance of having affected children. It allows couples reproductive choice and can prevent infant morbidity and mortality and significant distress for families. Professional bodies in many countries now recommend prospective parents should be informed about carrier screening. Implementing and funding expensive therapies increases the cost-effectiveness of carrier screening, increasing its attractiveness to governments. Best practice for genetic neuromuscular disorders should include equitable access to carrier screening.

Details

ISSN :
09608966
Volume :
31
Database :
OpenAIRE
Journal :
Neuromuscular Disorders
Accession number :
edsair.doi.dedup.....3a5986a2a3ae45b89e30a99a821e0b66
Full Text :
https://doi.org/10.1016/j.nmd.2021.07.007