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This Article Full Text Full Text (PDF) CME: Take the course for this article: Volume 70, Number 9, February 26, 2008 Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Visser, J. Articles by Visser, M. d. Search for Related Content PubMed Articles by Visser, J. Articles by Visser, M. d. Related Collections History of Neurology Anterior nerve cell disease Amyotrophic lateral sclerosis

The history of progressive muscular atrophy

Syndrome or disease?

From the Department of Neurology, Academic Medical Center, University of Amsterdam, The Netherlands.

Address correspondence and reprint requests to Dr. J. Visser, Department of Neurology (H2-235), Academic Medical Center, PO Box 22700, 1100 DE Amsterdam, The Netherlands j.visser{at}amc.uva.nl

Since its first description more than a century ago, there has been much debate about the diagnostic entity progressive muscular atrophy (PMA). Initially, PMA included all forms of progressive amyotrophy. With the identification of several myogenic and neurogenic diseases and the recognition of amyotrophic lateral sclerosis (ALS), PMA was deemed to disappear as a nosologic entity at the end of the 19th century. In the last century, various other lower motor neuron syndromes were distinguished which may previously have been designated as cases of PMA. In contrast, several observations provided evidence that PMA can be linked both clinically and pathologically to ALS. Therefore, PMA should be considered as a syndromal subtype within a clinical spectrum of motor neuron diseases.

Abbreviations: ALS = amyotrophic lateral sclerosis; MMN = multifocal motor neuropathy; MND = motor neuron disease; PBP = progressive bulbar palsy; PLS = primary lateral sclerosis; PMA = progressive muscular atrophy; PSMA= progressive spinal muscular atrophy.

Disclosure: The authors report no conflicts of interest.

Received May 15, 2007. Accepted in final form July 27, 2007.