The reorganisation of motor units in different motor neuron disorders

A study was made of the degree and sequence of neurophysiological changes during motor unit reorganisation in motor neuron disease (MND), spinal muscular atrophy (SMA), and prior polio. Concentric needle EMG was used in conjunction with our own computerized EMG-LAB system. Motor unit action potential (MUAP) parameters were measured in 543 muscles on weak and maximum effort. MUAP amplitude and area were found to increase in the early stages of damage, declining to normal or subnormal values in the course of the disease. It was concluded that in MND there is a pathological sequence: denervation--reinnervation--terminal denervation. The increase in MUAP amplitude and area in the early stages of lesion, reflecting reinnervation, was much greater in SMA than MND and most marked in prior polio. The eventual decrease is an expression of terminal decompensation.