Multiple System Atrophy

A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)

Also Known As:

Networked: 1045 relevant articles (17 outcomes, 104 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Parkinson Disease (Parkinson's Disease)
2.	Orthostatic Hypotension (Postural Hypotension)
3.	Overactive Urinary Bladder (Overactive Bladder)
4.	Atrophy
5.	Parkinsonian Disorders (Parkinsonism)

Experts

1.	Wenning, Gregor K: 41 articles (01/2016 - 09/2002)
2.	Poewe, Werner: 34 articles (10/2015 - 09/2002)
3.	Stefanova, Nadia: 28 articles (01/2016 - 09/2002)
4.	Goldstein, David S: 25 articles (11/2015 - 07/2002)
5.	Masliah, Eliezer: 19 articles (01/2015 - 06/2002)
6.	Wakabayashi, Koichi: 18 articles (12/2015 - 02/2002)
7.	Rockenstein, Edward: 18 articles (01/2015 - 06/2002)
8.	Takahashi, Hitoshi: 16 articles (12/2015 - 03/2002)
9.	Mori, Fumiaki: 15 articles (12/2015 - 03/2002)
10.	Holmes, Courtney: 15 articles (11/2015 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Multiple System Atrophy:

1.	Levodopa (L Dopa)FDA LinkGeneric 05/15/2009 - "Progressive degeneration of striatal projection neurons is thought to account for the loss of L-Dopa response observed in the majority of patients with the parkinsonian variant of multiple system atrophy (MSA-P). " 01/01/2009 - "Does clinical intolerance to a diagnostic acute levodopa challenge differentiate multiple system atrophy from PD?" 05/15/2007 - "High dose levodopa therapy is not toxic in multiple system atrophy: experimental evidence." 08/01/2006 - "Recent observations suggest that levodopa can induce irresistible sleep onset in multiple system atrophy (MSA). " 08/01/2006 - "Levodopa-induced sleepiness in the Parkinson variant of multiple system atrophy."
2.	alpha-SynucleinIBA 03/09/2007 - "To investigate the precise role of Hsc70 and Hsp70 in patients with multiple system atrophy (MSA), which is another alpha-synuclein-related disease, we performed immunohistochemical studies on Hsc70 and Hsp70 using autopsied brains from 7 normal subjects and 15 patients with MSA. " 10/01/2006 - "In this study, we measured the plasma alpha-synuclein levels in 105 patients with PD, 38 patients with multiple system atrophy (MSA), and 51 age-matched controls. " 02/01/2003 - "In this semiquantitative study based on 26 post-mortem cases, we describe the involvement of precerebellar nuclei in multiple system atrophy (MSA), a progressive degenerative disorder of the human central nervous system characterized by abnormal, argyrophilic and alpha-synuclein immunopositive intracellular inclusions within selectively vulnerable oligodendrocytes and nerve cells. " 10/01/2002 - "Alpha-synuclein immunoreactivity and ultrastructural study of glial cytoplasmic inclusions in multiple system atrophy." 07/19/2002 - "This cell system of oligodendroglial alpha-synuclein expression is a useful system to study alpha-synuclein metabolism in the cell type affected in multiple system atrophy. "
3.	3-Iodobenzylguanidine (Iobenguane)IBA 08/15/2010 - "Abnormal cardiac [(123)I]-meta-iodobenzylguanidine uptake in multiple system atrophy." 05/15/2008 - "Low myocardial MIBG uptake in multiple system atrophy with incidental Lewy body pathology: an autopsy case report." 01/01/2007 - "A slight decrease in cardiac uptake of MIBG has also been reported in some patients with multiple system atrophy (MSA). " 05/01/2005 - "MIBG uptake of the thyroid on early images decreased significantly also in multiple system atrophy compared to controls (p = 0.027). " 05/09/2000 - "Cardiac uptake of [123I]MIBG separates PD from multiple system atrophy."
4.	Rifampin (Rifampicin)FDA LinkGeneric 03/01/2014 - "We aimed to assess the safety and efficacy of rifampicin in patients with multiple system atrophy. " 02/01/2015 - "Optimizing clinical trial design for multiple system atrophy: lessons from the rifampicin study." 03/01/2014 - "Efficacy and safety of rifampicin for multiple system atrophy: a randomised, double-blind, placebo-controlled trial." 03/01/2014 - "Rifampicin for multiple system atrophy." 03/01/2014 - "Our results show that rifampicin does not slow or halt progression of multiple system atrophy. "
5.	Minocycline (Cyclops)FDA LinkGeneric 01/15/2010 - "The aim of the study was to investigate the efficacy of the antibiotic minocycline as a drug treatment in patients with Multiple-System-Atrophy Parkinson-type (MSA-P). " 01/15/2010 - "Minocycline 1-year therapy in multiple-system-atrophy: effect on clinical symptoms and [(11)C] (R)-PK11195 PET (MEMSA-trial)." 10/01/2004 - "The purpose of the present study was to analyze whether minocycline exhibits neuroprotective activity in a rat model of striatonigral degeneration (SND), the core pathology underlying levodopa-unresponsive parkinsonism associated with multiple system atrophy (MSA). "
6.	rasagiline (Azilect)FDA Link 02/01/2015 - "Efficacy of rasagiline in patients with the parkinsonian variant of multiple system atrophy: a randomised, placebo-controlled trial." 04/01/2008 - "The present study was performed to test the potential of rasagiline as a disease-modifying agent in multiple system atrophy (MSA) using a transgenic mouse model previously described by our group. " 02/01/2015 - "In this population of patients with the parkinsonian variant of multiple system atrophy, treatment with rasagiline 1 mg per day did not show a significant benefit as assessed by UMSARS. " 02/01/2015 - "Eligible participants aged 30 years or older with possible or probable parkinsonian variant multiple system atrophy were randomly assigned (1:1), via computer-generated block randomisation (block size of four), to receive either rasagiline 1 mg per day or placebo. " 02/01/2015 - "We aimed to assess the effect of rasagiline on symptoms and progression of the parkinsonian variant of multiple system atrophy. "
7.	Type A Botulinum Toxins (Botulinum A Toxin)FDA Link 10/01/2009 - "The new beneficial effect that we report in a small study sample encourages larger trials to confirm botulinum toxin type A injection into the detrusor muscle as an effective and safe treatment for refractory overactive bladder symptoms and detrusor overactivity related to Parkinson's disease and multiple system atrophy." 10/01/2009 - "We investigated the effectiveness and safety of botulinum toxin type A injected into the detrusor muscle in patients with Parkinson's disease and multiple system atrophy who had refractory overactive bladder symptoms and detrusor overactivity. "
8.	CamptocormiaIBA 10/01/2006 - "Camptocormia or Pisa syndrome in multiple system atrophy." 08/01/2005 - "We describe a case of progressive camptocormia as part of the initial presentation of a patient with multiple system atrophy." 08/01/2005 - "Camptocormia in a patient with multiple system atrophy." 10/01/2006 - "Authors present two cases of probable multiple system atrophy (MSA) with predominant parkinsonism and Pisa syndrome (or camptocormia). " 03/01/2006 - "Camptocormia associated with focal myositis in multiple-system atrophy."
9.	Dopamine beta-HydroxylaseIBA 07/15/2003 - "Variations in the dopamine beta-hydroxylase gene are not associated with the autonomic disorders, pure autonomic failure, or multiple system atrophy." 07/15/2003 - "Recent findings of an association between human NE deficiency and variants at the dopamine beta-hydroxylase (DBH) gene [Kim et al., 2002] prompted us to investigate these markers in patients with autonomic disorders; 38 with orthostatic intolerance (OI), 26 with pure autonomic failure (PAF), and 39 with multiple system atrophy (MSA). " 08/01/1991 - "The effect of a balanced liquid meal on supine and postural blood pressure (BP) responses was investigated in three groups of patients with chronic autonomic failure; 10 with associated neurological impairment (multiple system atrophy (MSA), Shy-Drager syndrome) and seven without (of which five had pure autonomic failure (PAF); and two had a deficiency of the enzyme dopamine beta hydroxylase, DBH-deficiency). " 01/01/2006 - "DOPS improved orthostatic hypotension in patients with familial amyloid polyneuropathy, congenital deficiency of dopamine beta hydroxylase, pure autonomic failure and multiple system atrophy. " 04/01/1995 - "The effect of supine exercise on blood pressure (BP), measured while recumbent and after head-up postural change, was investigated in three groups with marked postural hypotension due to chronic autonomic failure: 15 with associated neurological impairment (Shy-Drager syndrome, SDS, multiple system atrophy); 15 with pure autonomic failure (PAF) and two with a deficiency of the enzyme dopamine beta hydroxylase (DBH deficiency). "
10.	Trimethaphan (Trimetaphan)IBA 10/22/2002 - "We infused trimethaphan at increasing doses (0.5 to 10 mg/min IV) while monitoring heart rate, heart rate variability spectra, QT interval, and blood pressure in 10 normal volunteers, 9 patients with multiple system atrophy (MSA), and 8 patients with pure autonomic failure (PAF). " 05/01/2002 - "In patients with multiple system atrophy (Shy-Drager syndrome), supine hypertension is explained by residual sympathetic tone because it can be eliminated with the ganglionic blocker trimethaphan. " 06/13/2000 - "In patients with multiple system atrophy (MSA) or pure autonomic failure (PAF), we studied the effect of oral yohimbine on seated systolic blood pressure (SBP), the effect of ganglionic blockade (with trimethaphan) on supine SBP and plasma catecholamine levels, and the effect of alpha(1)-adrenoreceptor blockade (phentolamine) on supine SBP. "

Therapies and Procedures

1.	Continuous Positive Airway Pressure 09/01/2014 - "Discontinuation of continuous positive airway pressure treatment in multiple system atrophy." 07/01/2005 - "Continuous positive airway pressure for sleep-related breathing disorders in multiple system atrophy: long-term acceptance." 09/14/2004 - "The authors found that continuous positive airway pressure (CPAP) eliminated video-polysomnographic documented stridor in 13 multiple system atrophy (MSA) subjects with vocal cord abnormalities. " 09/01/2014 - "Continuous positive airway pressure (CPAP) is used for the management of sleep-disordered breathing in patients with multiple system atrophy (MSA). " 02/15/2009 - "Effects of continuous positive airway pressure on stridor in multiple system atrophy-sleep laryngoscopy."
2.	Deep Brain Stimulation 01/01/2012 - "This study summarizes the positive and negative effects of deep brain stimulation treatment in two pathologically confirmed multiple system atrophy patients from the University of Florida Deep Brain Stimulation-Brain Tissue Network. " 01/01/2012 - "A commentary on the lead positioning for deep brain stimulation in the pedunculopontine tegmental nucleus in a patient affected by multiple system atrophy." 01/01/2012 - "Deep brain stimulation has not been recommended for treatment in multiple system atrophy patients. " 01/01/2012 - "Deep brain stimulation response in pathologically confirmed cases of multiple system atrophy." 04/01/2008 - "The experience with deep-brain stimulation (DBS) in multiple-system atrophy (MSA) is sparse and generally disappointing. "
3.	Transplants (Transplant) 08/01/2000 - "The purpose of the present study was to evaluate embryonic neuronal grafts in a novel rat model of multiple system atrophy (MSA) with the help of in vivo magnetic resonance imaging (MRI) and to correlate imaging with histological parameters. " 09/01/2009 - "Striatal transplantation for multiple system atrophy--are grafts affected by alpha-synucleinopathy?" 01/01/2000 - "Our study confirms that functional improvement may be obtained from embryonic neuronal grafts in a double-lesion rat model of multiple system atrophy/striatonigral degeneration type. " 08/01/2000 - "In vivo magnetic resonance imaging of embryonic neural grafts in a rat model of striatonigral degeneration (multiple system atrophy)." 07/01/2000 - "Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy)."
4.	Cell Transplantation 01/01/2014 - "This study was designed to evaluate the effects of cord blood mononuclear cell transplantation in multiple system atrophy (MSA). " 01/01/2013 - "The aim of this study is to explore the safety and therapeutic effect of multiple cell transplantations on patients with multiple system atrophy. " 01/01/2014 - "The cord blood mononuclear cell transplantation was repeated 30 days after the first treatment in patients 1 and 2; it was repeated twice in patient 3. The clinical outcomes of treatment were used to assess the Unified Multiple System Atrophy Rating Scale (UMSARS) before, 90 and 180 days after the cell transplantation. " 01/01/2013 - "Ten patients suffering from multiple system atrophy were treated by multiple cell transplantations from August 2005 to March 2011. "
5.	Denervation 06/17/2014 - "Postganglionic sudomotor denervation in patients with multiple system atrophy." 06/01/2012 - "In contrast, most patients with multiple system atrophy, which can resemble PD+OH clinically, do not have evidence for cardiac or extra-cardiac noradrenergic denervation. " 12/01/2011 - "Subclinical nigrostriatal dopaminergic denervation in the cerebellar subtype of multiple system atrophy (MSA-C)." 01/01/1981 - "In five patients with multiple system atrophy, electromyographic studies using a new surface electrode technique, showed unequivocal evidence of denervation of the posterior crico-arytenoid muscle, and in three of them, of the interarytenoid muscle or crico-pharyngeal sphincter. " 09/01/1988 - "In patients with progressive autonomic failure and multiple system atrophy, the bladder neck was incompetent in every case and the striated muscle of the urethra was affected by a process of denervation and re-innervation. "