Progressive muscular atrophy
5663937
205027226
2008-04-11T23:29:26Z
SmackBot
433328
Date the maintenance tags or general fixes
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ICD9 = {{ICD9|335.21}} |
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MeshID = D009134 |
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'''Progressive muscular atrophy''' (PMA) is a rare subtype of [[Motor neurone disease]] (MND) which affects only the [[lower motor neurone]]s. This is in contrast to the most common form of MND, [[amyotrophic lateral sclerosis]], which affects both the [[upper motor neurone|upper]] and [[lower motor neurone|lower]] motor neurones. Famous people to suffer from it include [[Mike Gregory]].
==Symptoms==
As a result of lower motor neurone degeneration, the symptoms of PMA include:
* [[atrophy]]
* [[fasciculation]]s
* [[muscle weakness]]
==Differential diagnosis==
However, in contrast to [[amyotrophic lateral sclerosis]] or [[primary lateral sclerosis]], PMA is distinguished by the absence of:
* brisk [[reflexes]]
* [[spasticity]]
* [[Babinski's sign]]
* [[Emotional lability]]
==Diagnosis==
To be diagnosed with PMA one must have the lower [[motor neuron]] symptoms described above in the absence of upper motor neuron symptoms for a given period of time. Some authors recommend 3 years, others 5 years or even up to 7 years. However it is worth bearing in mind that this illness can progress into typical ALS at any time, even as many as 26 years after the initial diagnosis.{{Fact|date=April 2008}}
==Prognosis==
The significance of diagnosing progressive muscular atrophy as opposed to ALS is twofold.
* 1) the prognosis is better. The typical prognosis for ALS is approximately 2-5 years after initial diagnosis. In PMA survival is in the order of 5-10 years.{{Fact|date=April 2008}}
* 2) Patients with PMA do not suffer from the [[MND#Extra-motor change in MND|cognitive change]] identified in at least a subgroup of patients with ALS.{{Fact|date=April 2008}}
==External links==
* [http://www.alsforums.com/forum/forumdisplay.php?f=39 PMA online support group]
* [http://www.mndassociation.org/life_with_mnd/pmapls/index.html MND Association's web pages for PMA and PLS]
{{Diseases of the nervous system}}
[[Category:Unsolved problems in neuroscience]]