Progressive muscular atrophy
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| Progressive muscular atrophy Classification and external resources | |
| ICD-9 | 335.21 |
|---|---|
| DiseasesDB | 29149 |
| MeSH | D009134 |
Progressive muscular atrophy (PMA) is a rare subtype of Motor neurone disease (MND) which affects only the lower motor neurones. This is contrast to the most common form of MND, amyotrophic lateral sclerosis, which affects both the upper and lower motor neurones.
As a result of lower motor neurone degeneration, the symptoms of PMA include:
However, in contrast to amyotrophic lateral sclerosis or primary lateral sclerosis, PMA is distinguished by the absence of:
To be diagnosed with PMA one must have the LMN symptoms described above in the absence of UMN 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 PMA can progress into typical ALS at any time, even as much as 26 years after the initial diagnosis.
The significance of diagnosing PMA as opposed to ALS is twofold. Crucially, 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. More recently evidence has shown that patients with PMA do not suffer from the cognitive change identified in at least a subgroup of patients with ALS.
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
