Progressive muscular atrophy

Progressive muscular atrophy
Other namesDuchenne–Aran muscular atrophy, others
SpecialtyNeurology

Progressive muscular atrophy (PMA), also called Duchenne–Aran disease and Duchenne–Aran muscular atrophy, is a disorder characterised by the degeneration of lower motor neurons, resulting in generalised, progressive loss of muscle function.

PMA is classified among motor neuron diseases (MND) where it is thought to account for around 4% of all MND cases.[1]

PMA affects only the lower motor neurons, in contrast to amyotrophic lateral sclerosis (ALS), the most common MND, which affects both the upper and lower motor neurons, or primary lateral sclerosis, another MND, which affects only the upper motor neurons. The distinction is important because PMA is associated with a better prognosis than ALS.

  1. ^ Wijesekera LC, Mathers S, Talman P, Galtrey C, Parkinson MH, Ganesalingam J, Willey E, Ampong MA, Ellis CM, Shaw CE, Al-Chalabi A, Leigh PN (Mar 2009). "Natural history and clinical features of the flail arm and flail leg ALS variants". Neurology. 72 (12): 1087–1094. doi:10.1212/01.wnl.0000345041.83406.a2. PMC 2821838. PMID 19307543.