Spinal Muscular Atrophy (Progressive Muscular Atrophy)
418
relevant articles (5 outcomes,
28 trials/studies)
found for this Disease
Description:
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; Syndrome, Kennedy; Distal Spinal Muscular Atrophy; Spinal Muscular Atrophy, Distal; Muscular Atrophy, Spinal; Bulbo-Spinal Atrophy, X-Linked; Myelopathic Muscular Atrophy; Myelopathic Muscular Atrophy, Progressive; Progressive Myelopathic Muscular Atrophy; Progressive Proximal Myelopathic Muscular Atrophy; Proximal Myelopathic Muscular Atrophy, Progressive; Spinal Muscular Atrophy, Oculopharyngeal; Spinal Muscular Atrophy, Scapuloperoneal Form; X-Linked Bulbo-Spinal Atrophy; Atrophies, X-Linked Bulbo-Spinal; Atrophy, Myelopathic Muscular; Atrophy, Spinal Muscular; Atrophy, X-Linked Bulbo-Spinal; Bulbo Spinal Atrophy, X Linked; Bulbo-Spinal Atrophies, X-Linked; Bulbospinal Neuronopathies; Muscular Atrophy, Myelopathic; Neuronopathies, Bulbospinal; Neuronopathy, Bulbospinal; X Linked Bulbo Spinal Atrophy; X-Linked Bulbo-Spinal Atrophies; Bulbospinal Neuronopathy; Kennedy Syndrome; Oculopharyngeal Spinal Muscular Atrophy; Scapuloperoneal Form of Spinal Muscular Atrophy
Relationship Network
Disease Context: Research Results
Related Diseases
Experts
| 1. | Wirth, Brunhilde:
6 articles
(04/2008 - 06/2006)
|
| 2. | Brichta, Lars:
4 articles
(03/2008 - 06/2006)
|
| 3. | Rossoll, Wilfried:
3 articles
(04/2008 - 01/2002)
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| 4. | Burghes, Arthur H M:
3 articles
(02/2008 - 11/2003)
|
| 5. | Melki, Judith:
3 articles
(10/2007 - 10/2003)
|
| 6. | Sumner, Charlotte J:
3 articles
(03/2007 - 11/2003)
|
| 7. | Fischbeck, Kenneth H:
3 articles
(03/2007 - 05/2003)
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| 8. | Fischer, U:
3 articles
(10/2001 - 03/2000)
|
| 9. | Holker, Irmgard:
2 articles
(03/2008 - 06/2006)
|
| 10. | Butchbach, Matthew E R:
2 articles
(02/2008 - 01/2007)
|
Drugs and Biologics
Drugs and Important Biological Agents (IBA) related to Spinal Muscular Atrophy:
| 1. | gabapentin (Neurontin)FDA LinkGeneric
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| 2. | Thyrotropin-Releasing Hormone (Prolactin Releasing Hormone)FDA Link
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| 3. | OxygenIBA
12/01/1994
- " In a polysomnographic study of 32 neuromuscular patients-22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome--of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2), cardiac arrhythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring" 03/01/2007
- " OBJECTIVE: Our aim was to assess the efficacy of noninvasive ventilation (NIV) for the treatment of thoracoabdominal asynchrony during sleep in children with spinal muscular atrophy (SMA) types 1 and 2. DESIGN: Nine subjects underwent assessment for sleep apnea/hypopnea index (AHI), mean oxyhemoglobin saturation (SpO2), oxygen desaturation index, transcutaneous carbon dioxide tension (tcpCO2), and mean phase angle during sleep as a measure of thoracoabdominal coordination"
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|
| 4. | Carbon DioxideIBA
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| 5. | Oxyhemoglobins (Oxyhemoglobin)IBA
|
| 6. | Erythromycin (Erycette)FDA LinkGeneric
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| 7. | DNA (Deoxyribonucleic Acid)IBA
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| 8. | Neuronal Apoptosis-Inhibitory ProteinIBA
10/01/2007
- " The spinal muscular atrophy candidate interval genes including survival motor neuron, the responsible gene in spinal muscular atrophy phenotype expression, neuronal apoptosis inhibitory protein, and P44, potential modifying genes, are located on chromosome 5q13 in two highly homologous copies (telomeric and centromeric) within the spinal muscular atrophy region.METHODS: In this study, the neuronal apoptosis inhibitory protein gene deletion was analyzed in 34 spinal muscular atrophy families, with the consanguinity rate of 65% (22/34), in whom exon 7 of the survival motor neuron-1 gene was already confirmed and was deleted in 79% of the affected individuals" 06/01/2001
- " OBJECTIVE: To study the absence of survival motor neuron(SMN) gene exon 7,8 and neuronal apoptosis inhibitory protein(NAIP) gene exon 5,6 in Chinese patients with type I - IV spinal muscular atrophy (SMA) and to confirm the relationship between the deletions of SMN, NAIP and SMA phenotype" 06/01/2001
- " A study of survival motor neuron and neuronal apoptosis inhibitory protein gene in spinal muscular atrophy]" 10/01/2007
- " CONCLUSION: The neuronal apoptosis inhibitory protein gene deletion in spinal muscular atrophy-I was higher than the other spinal muscular atrophy types" 10/01/2007
- " Molecular analysis of the neuronal apoptosis inhibitory protein gene in families with spinal muscular atrophy."
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| 9. | Genetic Markers (Genetic Marker)IBA
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| 10. | Albuterol (Salbutamol)FDA LinkGeneric
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| 216 Drugs and Important Bio-Agents
and
356
more articles analyzed in the Research Interface, order at left...
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Therapies and Procedures
| 1. | Walkers
|
| 2. | Denervation
05/01/2005
- " Denervation was assessed in 89 spinal muscular atrophy (SMA) 1, 2, and 3 subjects via motor unit number estimation (MUNE) and maximum compound motor action potential amplitude (CMAP) studies, and results correlated with SMN2 copy, age, and function" 01/01/2008
- " These findings indicate that apoptosis, although probably a secondary phenomenon following denervation, plays a role in the progress of spinal muscular atrophy." 06/01/2001
- " The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy" 01/01/1989
- " The presence of partial denervation in the EMG led to a mistaken diagnosis of distal chronic spinal muscular atrophy" 07/01/1982
- " Moderate or marked fibrosis was infrequent in patients lacking electrodiagnostic, clinical, or muscle biopsy evidence of denervation but was present in 90% or more of patients with either spinal muscular atrophy or peripheral neuropathy; fibrosis correlated well with the presence of muscle fiber type grouping and grouped atrophy"
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| 3. | Therapeutics
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| 4. | Tracheostomy
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| 5. | Palliative Care (Palliative Medicine)
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| 37 Therapies and Procedures
and
71
more articles analyzed in the Research Interface, order at left...
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