Study of 962 patients indicates progressive muscular atrophy is a form of ALS
Open accessAbstract
BACKGROUND: Progressive muscular atrophy (PMA) is clinically characterized by signs of lower motor neuron dysfunction and may evolve into amyotrophic lateral sclerosis (ALS). Whether PMA is actually a form of ALS has important consequences clinically and for therapeutic trials. We compared the survival of patients with PMA or ALS to analyze the clinical features that influence survival in PMA. METHODS: We reviewed the medical records of patients with PMA (n = 91) or ALS (n = 871) from our ALS Center and verified survival by telephoning the families or using the National Death Index. RESULTS: In PMA, patients were more likely to be male (p < 0.001), older (p = 0.007), and lived longer (p = 0.01) than in ALS. Cox model analysis suggested that the risk of death increased with age at onset in both patient groups (p < 0.005). Upper motor neuron (UMN) signs developed in 22% of patients with PMA within 61 months after diagnosis. Demographic and other clinical variables did not differ at diagnosis between those who did or did not develop UMN signs. In PMA, the factors present at diagnosis that predicted shorter survival were greater number of body regions affected, lower forced vital capacity, and lower ALS Functional Rating Scale-Revised score. Noninvasive ventilation and gastrostomy were used frequently in PMA. CONCLUSION: Although patients with progressive muscular atrophy (PMA) tended to live longer than those with amyotrophic lateral sclerosis (ALS), shorter survival in PMA is associated with the same risk factors that predict poor survival in ALS. Additionally, PMA is relentlessly progressive, and UMN involvement can occur, as also reported in imaging and postmortem studies. For these reasons, PMA should be considered a form of ALS.
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PMA caregivers are likely to struggle with the same issues as ALS caregivers.
“PMA caregivers are, therefore, likely to struggle with the same issues as ALS caregivers.”
From A blended psychosocial support program for partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: Protocol of a randomized controlled trial (2018) · cites this paper as [5]
The Medical management in PMA and ALS section says the conditions cannot be distinguished by their need for multidisciplinary care, supporting the inference that their caregivers may face similar care demands. It does not examine caregivers or establish that they struggle with the same issues. (LLM, conf=0.82)
Experimental — accuracy not yet measuredPMA shows substantial overlap with ALS and is considered to be a form of ALS.
“PMA shows substantial overlap with ALS and is considered to be a form of ALS [5]”
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- Authors
- Kim, W. K., Liu, X., Sandner, J., Pasmantier, M., Andrews, J., Rowland, L. P., & Mitsumoto, H.
- Affiliations
- Columbia University Irving Medical Center (US)
- Venue
- Neurology
- Published
- 2009-11-17
- Volume
- 73
- Issue
- 20
- Pages
- 1686-1692
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC2788803
- OpenAlex
- W2073063121
- Cited by
- 217
- References
- 28
- Citations by year
- 2026: 12 · 2025: 8 · 2024: 13 · 2023: 15 · 2022: 8 · 2021: 16 · 2020: 15 · 2019: 11 · 2018: 11 · 2017: 22 · 2016: 11 · 2015: 9 · 2014: 15 · 2013: 15 · 2012: 14
- FWCI
- 7.31
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Neurology (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- ALS Association
- Sanofi
- Muscular Dystrophy Association
- Teva Pharmaceutical Industries
- Avanir Pharmaceuticals
- Otsuka Pharmaceutical
- National Institutes of Health (ES09089)
- National Institute of Neurological Disorders and Stroke (R01NS04812)
- National Institute of Environmental Health Sciences (ES 09089, R01ES016348, P30ES009089)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Keywords
- progressive muscular atrophy, amyotrophic lateral sclerosis, survival, prognostic factors, upper motor neuron signs, lower motor neuron dysfunction, age of onset, ALSFRS-R, forced vital capacity, non-invasive ventilation, gastrostomy, disease progression
- MeSH terms
- Age Factors, Amyotrophic Lateral Sclerosis, Female, Follow-Up Studies, Humans, Interviews as Topic, Male, Middle Aged, Muscular Atrophy, Spinal, Prognosis, Retrospective Studies, Risk Factors, Severity of Illness Index, Sex Factors, Proportional Hazards Models, Motor Neuron Disease, Age of Onset, Kaplan-Meier Estimate
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