Neurologist | Respiratory physician | |
History | Breathlessness Orthopnoea Recurrent chest infections Sleep quality Morning headaches Daytime sleepiness | Limb muscle weakness Swallowing problems Aspiration |
Examination | Use of accessory muscles of respiration Reduced chest expansion on maximal inspiration Abdominal paradox (inward movement of the abdomen during inspiration when supine) Ability to generate an adequate cough | Full neurological examination Muscle fasciculation (abnormal muscle movements may occur with hypercapnia) |
Investigations | Spirometry, mouth and sniff pressures, cough peak flow and oxygen saturation should be performed at baseline and, thereafter, at three monthly intervals, unless it is shown that the rate of decline allows a longer follow-up interval [3] If the oxygen saturation is <94% or <92% in those with chronic lung disease, or if they have sleep-related symptoms, arterial blood gas analysis and at a minimum overnight oximetry with or without the addition of transcutaneous CO2 or a respiratory variable sleep study Full polysomnography is not necessary A raised PaCO2 should lead to urgent specialist respiratory review (the patient should be seen within no more than 1 week) [3]. | |
Respiratory physiotherapy | If the patients has weak cough, with a cough peak flow <160 L·min−1 [4], or poor glottic coordination because of bulbar involvement, there should be early review by a respiratory physiotherapist with particular expertise in secretion clearance techniques |
PaCO2: arterial carbon dioxide tension.