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Respiratory involvement in inherited primary muscle conditions

¹ Department of Neurology, Queen’s Medical Centre, Nottingham, UK
² Department of Respiratory Medicine, Queen’s Medical Centre

Correspondence to:
Correspondence to:
A Wills
Department of Neurology, Queen’s Medical Centre, Nottingham NG7 2UH, UK; adewills61{at}hotmail.com

Patients with inherited muscle disorders can develop respiratory muscle weakness leading to ventilatory failure. Predicting the extent of respiratory involvement in the different types of inherited muscle disorders is important, as it allows clinicians to impart prognostic information and offers an opportunity for early interventional management strategies. The approach to respiratory assessment in patients with muscle disorders, the current knowledge of respiratory impairment in different muscle disorders and advice on the management of respiratory complications are summarised.

Abbreviations: AMD, acid maltase deficiency; DM1, myotonic dystrophy; DM2, proximal myotonic myopathy; DMD, dystrophinopathy; EDMD, Emery–Dreifuss muscular dystrophy; EDS, excessive daytime sleepiness; FRC, functional residual capacity; FSH, facioscapulohumeral dystrophy; FVC, forced vital capacity; LGMD, limb-girdle muscular dystrophy; MDC, congenital muscular dystrophy; MIP, maximal inspiratory mouth pressure; NIV, non-invasive invasive ventilation; PaCO₂, arterial carbon dioxide tension; SNIP, sniff nasal inspiratory pressure; SpO₂, single oxygen saturation