ABSTRACT

Motor neuron disease (MND) is characterized by the progressive degeneration of the upper and lower motor neurons. Approximately 90% of cases are sporadic, with an incidence rate of 1-2/100 000. The disease typically affects adults in late middle life, and 75% of patients present with symptoms in the limbs, while 25% present with bulbar signs. The disease typically progresses to affect other regions, and death usually occurs between 1 and 5 years from the onset of symptoms.