Although respiratory failure is the primary cause of death in patients with amyotrophic lateral sclerosis (ALS), the management of nutritional status is important to enhancing the quality of life and optimising the timing of interventive techniques. Progressively weakening muscles impair the patient's ability to eat, and nearly all patients with ALS develop severe dysphagia. If nutritional support is not provided, food and fluid consumption may be greatly restricted, leading to weight loss and malnutrition. This may be compounded by impaired respiratory functions, which place increased energy demands on the patient. This paper describes the nutritional needs of ALS patients from a worldwide and cross-cultural perspective. In particular, the differences between a paternalistic and a patient-centred approach to treatment are addressed. The need for further study into the nutritional status of ALS patients and the issue of parenteral and enteral nutritional therapy, particularly percutaneous endoscopic gastrostomy, are discussed.