Progressive Muscular Atrophy.
Progressive muscular atrophy (PMA) is a rare, sporadic, adult-onset motor neuron disease, clinically characterized by isolated lower motor neuron features; however, clinically evident upper motor neuron signs may emerge in some patients. Subclinical upper motor neuron involvement is identified pathologically, radiologically, and neurophysiologically in a substantial number of patients with PMA. Patients with subclinical upper motor neuron involvement do not fulfill the revised El Escorial criteria to participate in amyotrophic lateral sclerosis clinical trials. Intravenous immunoglobulin therapy is only marginally beneficial in a small subgroup of patients with lower motor neuron syndrome without conduction block.
['Liewluck|Teerin|T|', 'Saperstein|David S|DS|']
D003937:Diagnosis, Differential D019468:Disease Management D006801:Humans D016756:Immunoglobulins, Intravenous D016472:Motor Neuron Disease D009134:Muscular Atrophy, Spinal D059906:Neuroimaging D012720:Severity of Illness Index
Progressive muscular atrophy PMA Lower motor neuron syndrome Lower motor neuron-onset ALS