Spinal Muscular Atrophy (Progressive Muscular Atrophy)

455  relevant articles (6 outcomes, 30 trials/studies) found for this Disease

Progressive Muscular Atrophy; Adult Spinal Muscular Atrophy; Distal Spinal Muscular Atrophy; Spinal Muscular Atrophy, Distal; Muscular Atrophy, Spinal; 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; Atrophies, Progressive Muscular; Atrophy, Myelopathic Muscular; Atrophy, Progressive Muscular; Atrophy, Spinal Muscular; Bulbospinal Neuronopathies; Muscular Atrophies, Progressive; Muscular Atrophy, Myelopathic; Muscular Atrophy, Progressive; Neuronopathies, Bulbospinal; Neuronopathy, Bulbospinal; Progressive Muscular Atrophies; Bulbospinal Neuronopathy; Oculopharyngeal Spinal Muscular Atrophy; Scapuloperoneal Form of Spinal Muscular Atrophy

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Muscular Dystrophies (Muscular Dystrophy)
2.	Spinal Dysraphism (Spina Bifida)
3.	Sleep Apnea Syndromes (Sleep Apnea)
4.	Multiple Sclerosis
5.	Duchenne Muscular Dystrophy (Muscular Dystrophy, Becker)

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)
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)
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.	Thyrotropin-Releasing Hormone (Prolactin Releasing Hormone)FDA Link 07/01/1994 - "Efficacy of thyrotropin-releasing hormone in the treatment of spinal muscular atrophy." 09/01/2000 - "OBJECTIVE: To determine whether thyrotropin-releasing hormone (TRH) can increase muscle strength in children with spinal muscular atrophy types 2 and 3. DESIGN: A randomized, double-blinded, controlled, 5-wk drug trial of six subjects and three controls. " 09/01/2000 - "A study of thyrotropin-releasing hormone for the treatment of spinal muscular atrophy: a preliminary report." 07/01/1994 - "Thyrotropin-releasing hormone has a neurotrophic effect on the spinal anterior motor neurons of spinal muscular atrophy patients and thus may be warranted for the management of spinal muscular atrophy." 07/01/1994 - "Children with spinal muscular atrophy were treated by the administration of thyrotropin-releasing hormone. " Order ALL the reference details at left...
2.	gabapentin (Neurontin)FDA LinkGeneric 10/15/2001 - "OBJECTIVE: To evaluate the efficacy of gabapentin in increasing muscle strength of patients with spinal muscular atrophy (SMA). " 08/01/2003 - "Role of gabapentin in spinal muscular atrophy: results of a multicenter, randomized Italian study." 10/15/2001 - "A placebo-controlled trial of gabapentin in spinal muscular atrophy." 08/01/2003 - "We carried out a multicenter, randomized, controlled trial of gabapentin versus no treatment in 120 patients with type II or III spinal muscular atrophy for 12 months. " Order ALL the reference details at left...
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. " Order ALL the reference details at left...
4.	Carbon DioxideIBA 09/01/1976 - "2 cases of progressive spinal muscular atrophy with carbon dioxide narcosis]" 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. " Order ALL the reference details at left...
5.	Oxyhemoglobins (Oxyhemoglobin)IBA 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. " Order ALL the reference details at left...
6.	Erythromycin (Erycette)FDA LinkGeneric 08/10/1992 - "[A case of progressive spinal muscular atrophy with disorder of intestinal motility improved by administration of erythromycin]" Order ALL the reference details at left...
7.	DNA (Deoxyribonucleic Acid)IBA 10/01/1995 - "DNA studies in 103 spinal muscular atrophy (SMA) patients from The Netherlands revealed homozygosity for a survival motor neuron (SMN) deletion in 96 (93%) of 103. " 07/27/1991 - "[DNA studies in proximal spinal muscular atrophy]" 12/01/1986 - "We used probes for DNA polymorphisms on the X chromosome to study genetic linkage in seven families with X-linked adult-onset spinal muscular atrophy. " 01/01/2007 - "DNA analysis confirmed the diagnosis of spinal muscular atrophy on the third day of life. " 07/01/2005 - "DNA analysis revealed homozygous deletion in exons 7 and 8 of SMN1 gene, confirming the diagnosis of spinal muscular atrophy. " Order ALL the reference details at left...
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." Order ALL the reference details at left...
9.	Genetic Markers (Genetic Marker)IBA 11/01/1993 - "2. A genetic linkage study was carried out on 70 individuals using nine polymorphic DNA markers (eight RFLP and one microsatellite) from chromosome 5q11.2-13.3 which have been shown to flank the spinal muscular atrophy (SMA) gene. " 07/01/1993 - "Linkage studies with 9 highly informative DNA markers on the long arm of chromosome 5 were performed in 12 multiplex families (29 patients) with spinal muscular atrophy (SMA) from The Netherlands. " 04/01/1994 - "Use of genetic and physical mapping to locate the spinal muscular atrophy locus between two new highly polymorphic DNA markers." 01/01/1996 - "DNA markers linked to various human genetic neuromuscular diseases were not linked to hereditary canine spinal muscular atrophy which segregates in this kindred. " Order ALL the reference details at left...
10.	Albuterol (Salbutamol)FDA LinkGeneric 01/01/2003 - "Pilot trial of albuterol in spinal muscular atrophy." 08/27/2002 - "Pilot trial of albuterol in spinal muscular atrophy." 08/27/2002 - "The aim of this pilot study was to evaluate the effect of albuterol in children with spinal muscular atrophy (SMA). " 01/01/2008 - "Salbutamol increases SMN mRNA and protein levels in spinal muscular atrophy cells." Order ALL the reference details at left...

Therapies and Procedures

1.	Walkers 08/01/2003 - "Pre-natal screening has dramatically reduced the incidence of spina bifida in the UK and subsequently swivel walkers have been used in a wider range of pathology, including spinal muscular atrophy, multiple sclerosis, muscular dystrophy and other neurological conditions that lead to lower limb dysfunction. " Order ALL the reference details at left...
2.	Intravenous Injections 08/01/2009 - "There is no definitive treatment for spinal muscular atrophy, but recent reports have indicated the efficacy of intravenous injection, but not oral administration, of thyrotropin-releasing hormone (TRH). " Order ALL the reference details at left...
3.	Oral Administration 08/01/2009 - "Oral administration of this thyrotropin-releasing hormone analogue should be noted as a promising therapy for spinal muscular atrophy." 08/01/2009 - "Oral administration of the thyrotropin-releasing hormone (TRH) analogue, taltireline hydrate, in spinal muscular atrophy." 08/01/2009 - "There is no definitive treatment for spinal muscular atrophy, but recent reports have indicated the efficacy of intravenous injection, but not oral administration, of thyrotropin-releasing hormone (TRH). " Order ALL the reference details at left...
4.	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. " 10/01/2009 - "Spinal muscular atrophy (SMA), a recessive genetic disease, affects lower motoneurons leading to denervation, atrophy, paralysis and in severe cases death. " Order ALL the reference details at left...
5.	Therapeutics 01/01/2004 - "Various animal models have been developed to study the pathogenesis of spinal muscular atrophy, as well as to test novel therapeutics. " 07/01/2006 - "PURPOSE: The natural history of spinal muscular atrophy suggests that for maximum effect, therapeutics will need to be administered in the earliest phases of the disease. " 04/01/2006 - "Therapeutics development for spinal muscular atrophy." 03/30/2001 - "This system provides a powerful setting for studying the function of SMN in vivo and for screening for potential therapeutics for spinal muscular atrophy." 04/01/2009 - "In this review, recent developments in splicing therapeutics for the treatment of two specific diseases are described: correcting the alternative splicing of survival of motor neuron (SMN)2 pre-mRNA to compensate for the defective SMN1 gene in spinal muscular atrophy, and re-engineering the splicing of apolipoprotein B pre-mRNA to lower circulating cholesterol levels." Order ALL the reference details at left...