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Spinal Muscular Atrophy (Progressive Muscular Atrophy)

A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
Also Known As:
Progressive Muscular Atrophy; Adult Spinal Muscular Atrophy; Distal Spinal Muscular Atrophy; Spinal Muscular Atrophy, Distal; Muscular Atrophy, Spinal; Adult-Onset Spinal Muscular Atrophy; Amyotrophy, Neurogenic Scapuloperoneal, New England Type; Hereditary Motor Neuronopathy; Muscular Atrophy, Adult Spinal; Myelopathic Muscular Atrophy; Myelopathic Muscular Atrophy, Progressive; Progressive Myelopathic Muscular Atrophy; Progressive Proximal Myelopathic Muscular Atrophy; Proximal Myelopathic Muscular Atrophy, Progressive; Scapuloperoneal Spinal Muscular Atrophy; Spinal Amyotrophy; Spinal Muscular Atrophy, Oculopharyngeal; Spinal Muscular Atrophy, Scapuloperoneal; Spinal Muscular Atrophy, Scapuloperoneal Form; Adult Onset Spinal Muscular Atrophy; Amyotrophies, Spinal; Amyotrophy, Spinal; Atrophies, Progressive Muscular; Atrophy, Myelopathic Muscular; Atrophy, Progressive Muscular; Atrophy, Spinal Muscular; Bulbospinal Neuronopathies; Hereditary Motor Neuronopathies; Motor Neuronopathies, Hereditary; Motor Neuronopathy, Hereditary; Muscular Atrophies, Progressive; Muscular Atrophy, Myelopathic; Muscular Atrophy, Progressive; Neuronopathies, Bulbospinal; Neuronopathies, Hereditary Motor; Neuronopathy, Bulbospinal; Neuronopathy, Hereditary Motor; Progressive Muscular Atrophies; Spinal Amyotrophies; Bulbospinal Neuronopathy; Oculopharyngeal Spinal Muscular Atrophy; Scapuloperoneal Form of Spinal Muscular Atrophy
Networked: 2240 relevant articles (110 outcomes, 202 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Duchenne Muscular Dystrophy (Muscular Dystrophy, Becker)
2. Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
3. Muscular Dystrophies (Muscular Dystrophy)
4. Motor Neuron Disease (Primary Lateral Sclerosis)
5. Disease Progression

Experts

1. Swoboda, Kathryn J: 44 articles (01/2022 - 05/2005)
2. Mercuri, Eugenio: 42 articles (07/2022 - 02/2005)
3. Wirth, Brunhilde: 42 articles (01/2022 - 03/2006)
4. Gillingwater, Thomas H: 32 articles (01/2021 - 12/2008)
5. Muntoni, Francesco: 30 articles (01/2022 - 09/2011)
6. Finkel, Richard S: 29 articles (01/2022 - 08/2007)
7. Burghes, Arthur H M: 25 articles (01/2021 - 11/2003)
8. Darras, Basil T: 24 articles (01/2022 - 12/2007)
9. Pellizzoni, Livio: 24 articles (01/2021 - 08/2002)
10. Kolb, Stephen J: 23 articles (01/2022 - 07/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Spinal Muscular Atrophy:
1. nusinersenIBA
2. Antisense OligonucleotidesIBA
3. ZolgensmaIBA
4. Proteins (Proteins, Gene)FDA Link
5. RisdiplamIBA
6. Biomarkers (Surrogate Marker)IBA
7. Thyrotropin-Releasing Hormone (Protirelin)FDA Link
8. Valproic Acid (Depakote)FDA LinkGeneric
9. Histone Deacetylase InhibitorsIBA
10. OligonucleotidesIBA

Therapies and Procedures

1. Therapeutics
2. Noninvasive Ventilation
3. Tracheotomy
4. Mechanical Ventilators (Ventilator)
5. Drug Therapy (Chemotherapy)