Four patients presented with slowly progressive, bilateral, asymmetric weakness and muscle atrophy in the lower extremities, accompanied by cramps and fasciculations. Sensory symptoms were insignificant. There was no bladder or bowel disturbance. Upper extremities and cranial nerves were normal. Weakness was found in lumbosacral myotomes, ranging from L2 to S1. The tendon reflexes varied, and extensor plantar responses were found in one case with proximal leg involvement. Nerve conduction studies were normal, but segmental chronic and often active denervation confined to the weak myotomes in the lower extremities was found in the electromyogram. Magnetic resonance imaging showed evidence of spondylotic lumbosacral myelopathy associated with disc herniation or osteophytic arthropathy at the T11/T12 spinal level in all patients, with increased signal within the adjacent cord. This unusual purely motor presentation may result from ischemic myelopathy secondary to compression of the anterior spinal artery.