Ten new cases of distal amyotrophy localized in an upper limb are reported. In all cases, patients were young males. The onset of the neurological impairment was always progressive during one to three years. The disease remained stable with a follow-up study of at least three years. Electrophysiological and neuroradiologic results were in keeping with an involvement of the anterior horn cell, in agreement with similar studies found in the literature. The relatively benign evolution of this syndrome, in regard to the usual prognosis of the other forms of motor neuron disease, especially amyotrophic lateral sclerosis, has to be outlined.