Motor Neurone Disease Research — Selection and Evaluation of Model Neurones

Abstract

The most remarkable feature of motor neurone disease (MND) is that it is virtually confined to the voluntary motor system. Its specificity of attack is not seen in any other disease of the nervous system (Appel, 1981). One hundred and fifty years after the condition was first described, little is known about its aetiology (Munsat et al., 1984). For example, particular groups of motor cells are spared at either end of the spinal column (controlling eye movement and the rectal sphincters, respectively), a phenomenon which has not been explained. The limited data on nerve cell physiology has allowed Weiner (1980) to suggest that this may be based on a difference in sensitivity towards androgenic hormones, receptors for which are found only on the cells which succumb to the disease. This hypothesis may not be correct, but there must be some biochemical or physiological basis to the selectivity. Others have suggested a toxic environmental factor as being responsible for MND (Yoshimasu et al., 1982). Detailed examination of motor neurones themselves may help to answer these questions.