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Neurol Clin. 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005.

Progressive Muscular Atrophy.

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Department of Neurology, University of Colorado School of Medicine, Anschutz Medical Campus, 12631 East 17th Avenue, Mail Stop B-185, Aurora, CO 80045, USA; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address:
Phoenix Neurological Associates, University of Arizona College of Medicine, 5090 North 40th Street, Suite 250, Phoenix, AZ 85018, USA.


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.


Lower motor neuron syndrome; Lower motor neuron-onset ALS; PMA; Progressive muscular atrophy

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