Respiratory function assessment and intervention in neuromuscular disorders

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Curr Opin Neurol. 2005 Oct;18(5):543-547.

Respiratory function assessment and intervention in neuromuscular

disorders.

Mellies U, Dohna-Schwake C, Voit T.

University of Essen, Children's Hospital, Department of General

Pediatrics and Neuropediatrics, Essen, Germany.

PURPOSE OF REVIEW: Published research on neuromuscular respiratory

failure has increased rapidly over the past decade. Progress in the

management of respiratory complications and preventive care have

improved outcomes and quality of life for many. In particular,

noninvasive positive pressure ventilation (NIPPV) is effective in

resolving respiratory failure and has dramatically changed the

management of these patients. In this review we discuss recent

studies assessing the course of respiratory failure, with a focus on

non-Duchenne muscular dystrophy; investigating the impact NIPPV has

when it is introduced before diurnal respiratory failure develops;

and evaluating a device to treat insufficient cough - the mechanical

insufflator-exsufflator (MI-E).

RECENT FINDINGS: Studies in children with congenital muscular

dystrophies and spinal muscular atrophies detected a high prevalence

of respiratory complications and confirmed that respiratory failure

can be predicted by measurement of vital capacity. NIPPV is an

established treatment that is effective in the long-term management

of end-stage diurnal respiratory failure. Additionally, recent

studies showed improvement in symptoms, quality of life and

prevention of respiratory complications when it is introduced for

nocturnal hypoventilation before the development of diurnal

respiratory failure. However, data on the efficiency of the MI-E are

limited, and studies comparing it with other techniques of assisted

coughing are needed.

SUMMARY: Recent findings stress the importance of regular respiratory

assessment in neuromuscular disease, including sleep studies;

strongly support the introduction of NIPPV for management of

symptomatic nocturnal hypoventilation; and suggest that prospective

randomized trials are required to verify the usefulness of the MI-E.