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progressive muscular atrophy : ウィキペディア英語版 | progressive muscular atrophy
Progressive muscular atrophy (PMA), also known as Duchenne-Aran muscular atrophy and Duchenne-Aran disease — is a rare subtype of motor neuron disease (MND) that affects only the lower motor neurones. PMA is thought to account for around 4% of all MND cases. This is in contrast to amyotrophic lateral sclerosis (ALS), the most common form of MND, which affects both the upper and lower motor neurones, or primary lateral sclerosis, another rare MND variant, which affects only the upper motor neurons. The distinction is important because PMA is associated with a better prognosis than classic ALS. ==History== Despite being rarer than ALS, PMA was actually described earlier, when in 1850 French neurologist François Aran described 11 cases which he termed ''atrophie musculaire progressive''. Contemporary neurologist Duchenne de Boulogne also claimed to have described the condition one year earlier, but the written report was never found; hence, the condition was once known as "Aran–Duchenne disease" or "Duchenne–Aran disease".
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