Progressive muscular atrophy (PMA) is a form of motor neuron disease, but its outcome is not well defined, and the aim was to study the pattern of clinical progression in PMA. We studied 10 patients prospectively for 12 months. None showed clinical signs of upper motor neuron involvement at presentation or during follow-up, and all had normal transcranial magnetic stimulation studies. Four had upper limb onset, four lower limb onset and two axial onset. We used neurophysiological and strength measurements and a clinical rating scale (ALS-FRS). Seven other patients presenting with a PMA syndrome developed upper motor neuron signs during a one-year period of observation and were excluded from the study. The rate of progression was variable. At six months, only motor unit number estimation (MUNE) and ALS-FRS had decreased significantly. The Neurophysiological Index (NI) and M-wave amplitude measurements decreased at 12 months. Two patients with axial-onset disease progressed rapidly to respiratory failure. Overall the pattern of change resembled that of ALS, although some patients progressed very slowly. Axial onset, however, predicts the early onset of respiratory failure, and a poor prognosis.