Respiratory involvement in inherited primary muscle conditions

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J Neurol Neurosurg Psychiatry. 2006 Oct;77(10):1108-15.

Respiratory involvement in inherited primary muscle conditions.

Shahrizaila N, Kinnear WJ, Wills AJ.

Department of Neurology, Queen's Medical Centre, Nottingham NG7 2UH, UK.

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.