Abstract

Vital capacity (VC) and maximum mouth pressures are often used to monitor respiratory function in motor neuron disease (MND), but require the use of a mouthpiece. Sniff nasal inspiratory pressure (SNIP) is a simple and reliable means of measuring inspiratory muscle strength; it does not involve the use of a mouthpiece and might therefore be better than VC or mouth pressures for assessing patients with bulbar disease. SNIP, maximum inspiratory (MIP) and expiratory mouth pressure (MEP), VC and arterial carbon dioxide tension (Pa,CO2) were measured in 59 consecutive patients attending a specialist MND clinic. Thirty-one had bulbar involvement on clinical grounds. Both SNIP and VC were inversely related to Pa,CO2 in nonbulbar patients only. Neither MIP nor MEP were related to Pa,CO2. The 10 patients with an elevated Pa,CO2 (>6 kPa) had significantly lower SNIP and VC than normocapnic patients. Sniff nasal inspiratory pressure can be used to monitor respiratory function in motor neuron disease. It is quick and easy for patients to perform, but otherwise appears to offer little advantage over vital capacity measurement. Patients with bulbar disease are often poor at performing sniff nasal inspiratory pressure manoeuvres, possibly because of upper airway collapse or inability to close the mouth completely during the manoeuvre.

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