General management

• FVC should be monitored 2-4-hrly and intubation and mechanical ventilation should follow if FVC <1l. Other indices of respiratory function are less sensitive. In particular, arterial blood gases may be maintained up to the point of respiratory arrest.

• Weak respiratory muscles lead to progressive basal atelectasis and sputum retention. Chest infection is a significant risk; regular chest physiotherapy with intermittent positive pressure breathing are required for prevention where mechanical ventilation is not necessary.

• Patients who are immobile are at risk of venous stasis and deep venous thrombosis. Prophylaxis with subcutaneous heparin is reasonable. Immobile patients also require attention to posture to prevent pressure sores and contractures.

• Weak bulbar muscles may compromise swallowing with consequent malnutrition or pulmonary aspiration. Enteral nutritional support via a nasogastric tube is necessary.

• In cases with coexistent autonomic neuropathy enteral nutrition may be impossible, necessitating parenteral nutritional support. These patients may also suffer arrhythmias and hypotension requiring appropriate support.

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