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Many epidemiological studies of MND have been carried out all over the world. These show that race, diet and lifestyle are not factors that determine who develops the disease. It does not occur in epidemics, there is no evidence of it being infectious and it does not appear to be caused by any other disease.

Many people with MND believe that the disease started following a specific event in their lives, but the research does not show any link with events such as traumatic injuries, emotional shock, traffic accidents or exposure to chemicals. The disease is as common in Europe as it is in America, and it is no more widespread in industrial cities than in the countryside.

In every country studied there about two new cases for every 100,000 people in the population every year, and there are between seven and nine people with the disease (1:12,000) at any one time for every 100,000 in the population. There are therefore about 5,000 people in the UK with MND at any one time. The average age of onset is 50 (49 for men; 53 for women), and the male to female ratio is 1.4:1. A suspicion that the disease is increasing in frequency is probably explained by the fact that it is being diagnosed more accurately since the advent of better investigative techniques.

Careful epidemiological studies in several European countries, including Britain, and in Japan, have suggested that within any population there is a group of people who are in some way predisposed to the disease, or at more risk of developing it, while others seem resistant to it. However, the reason for this is so far unknown.