Malnutrition, present in 16-50% of ALS patients, is an independent prognost
ic factor for worsened survival. It is caused primarily by swallowing dysfu
nction, resulting from involvement of the lower sets of cranial nerves, but
hypermetabolism is also implicated. Malnutrition itself can produce neurom
uscular weakness and adversely affect patients' quality of life, thereby cr
eating a vicious circle. The nutritional status of ALS patients can be asse
ssed with dietary review and measurements of weight (W) and height (H), A b
ody mass index (BMI = W/H-2) below 18.5-20 kg/m(2) indicates a state of mal
nutrition. Dietary counselling is important, but rapidly becomes insufficie
nt, particularly in bulbar-onset ALS, where enteral nutritional support is
then necessary. Percutaneous endoscopic gastrostomy tube placement is well
tolerated, and provides more efficient enteral nutrition than nasogastric t
ube feeding. Enteral nutrition support can improve the respiratory status o
f ALS patients. The effect on survival remains to be confirmed.