Progressive muscular atrophy (PMA) is a rare subtype of amyotrophic lateral sclerosis (ALS) or motor neurone disease (MND) which affects only the lower motor neurones. PMA is thought to account for around 4% of all ALS/MND cases. This is in contrast to the most common form of ALS/MND, amyotrophic lateral sclerosis, which affects both the upper and lower motor neurones, or another rare form of ALS/MND, primary lateral sclerosis, which affects only...
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Progressive muscular atrophy (PMA) is a rare subtype of amyotrophic lateral sclerosis (ALS) or motor neurone disease (MND) which affects only the lower motor neurones. PMA is thought to account for around 4% of all ALS/MND cases. This is in contrast to the most common form of ALS/MND, amyotrophic lateral sclerosis, which affects both the upper and lower motor neurones, or another rare form of ALS/MND, primary lateral sclerosis, which affects only the upper motor neurones. The distinction is important because PMA is associated with a better prognosis than classical ALS/MND.
Despite being rarer than ALS/MND, PMA was actually described earlier, when in 1850 French Neurologist Francois Aran described 11 cases which he termed atrophie musculaire progressive. Contemperorary neurologist Duchenne also claimed to have described the condition one year earlier, but the written report was never found; an archaic term for the disease was once "Aran-Duchenne disease" or "Duchenne-Aran disease".
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