The history of motor unit number estimation (MUNE) is given, together with brief descriptions of the various methods presently available. A small muscle of the hand contains about 100 motor units and greater numbers are found in larger muscles; beyond 60 years the numbers begin to decline. In ALS approximately half the motor units cease to function within 6 months of the involvement of the motoneuron pool, while in adult spinal muscular atrophy further loss may not occur over several years. The reduction in MUNE values in myotonic dystrophy remains an enigma, but even more curious are the losses and subsequent recoveries occasionally observed in hyperthyroidism and chronic renal failure; possibly, nontransmitting (“silent”) synapses are involved. MUNE may also be used to study CNS problems such as hemiplegia and congenital brachial palsy. The availability of more powerful computers for EMG should lead to advances in MUNE. © 1995 John Wiley & Sons, Inc.