Peripheral electrophysiological studies are of particular value for elucidating the anatomy and pathophysiology of neuromuscular diseases, but they can also help in providing clues to the etiology of the disease. Recent studies of the motor units in chronic denervating conditions including amyotrophic lateral sclerosis (ALS) are reviewed. These indicate that reinnervation is a relatively active process which compensates for the progressive loss of motoneurons in ALS until more than 50% of the motoneurons have died. There seems to be no predilection for death of motoneurons of any particular size in ALS. Fasciculations may arise both proximally and distally. The dying-back change is not a major feature of ALS. These and other data cast doubt on the etiological theories that ALS arises from premature aging of motoneurons, deficiency of motoneuron trophic factors, or an inhibitor of a motoneuronal sprouting factor, and point to the need to study metabolic changes intrinsic to the motoneuron in ALS.
ASJC Scopus subject areas
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)