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*[[ataxia]] (Poor coordination / unsteady walking)
*[[ataxia]] (Poor coordination / unsteady walking)


When [[Autonomic nervous system|autonomic]] failure predominates, the term '''Shy-Drager syndrome''' is often used, although this term is no longer current, given the recent terminology changes which are explained below.<ref>Kurt A. Jellinger, [http://www.springerlink.com/content/03j1p2032866847h/fulltext.pdf?page=1 Lewy bodies in MSA patients]</ref> This syndrome was named after Dr Milton Shy and Dr Glenn Drager, who identified it in 1960, but the American Autonomic Society and the American Academy of Neurology redefined it as multiple system atrophy with autonomic phenomena in 1996.<ref>{{cite journal |author= | title=Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy |journal=Neurology |volume=46 |issue=5 |pages=1470 |year=1996 |pmid=8628505 |doi=}}</ref><ref>{{WhoNamedIt|synd|875}}</ref><ref>{{cite journal |author=Shy GM, Drager GA |title=A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study |journal=Arch. Neurol. |volume=2 |issue= |pages=511–27 |year=1960 |pmid=14446364 |doi=}}</ref> The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.
When [[Autonomic nervous system|autonomic]] failure predominates, the term '''Shy-Drager syndrome''' is often used, although this term is no longer current, given the recent terminology changes which are explained below.<ref>{{cite journal |author=Jellinger KA |title=More frequent Lewy bodies but less frequent Alzheimer-type lesions in multiple system atrophy as compared to age-matched control brains |journal=Acta Neuropathologica |volume=114 |issue=3 |pages=299–303 |year=2007 |doi=10.1007/s00401-007-0227-4 |url=http://www.springerlink.com/content/03j1p2032866847h}}</ref> This syndrome was named after Dr Milton Shy and Dr Glenn Drager, who identified it in 1960, but the American Autonomic Society and the American Academy of Neurology redefined it as multiple system atrophy with autonomic phenomena in 1996.<ref>{{cite journal |author= | title=Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy |journal=Neurology |volume=46 |issue=5 |pages=1470 |year=1996 |pmid=8628505 |doi=}}</ref><ref>{{WhoNamedIt|synd|875}}</ref><ref>{{cite journal |author=Shy GM, Drager GA |title=A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study |journal=Arch. Neurol. |volume=2 |issue= |pages=511–27 |year=1960 |pmid=14446364 |doi=}}</ref> The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.


A variant with combined features of MSA and [[Lewy body dementia]] may also exist. <ref>{{citejournal|author=Sikorska B, Papierz W, Preusser M, Liberski PP, Budka H.|title=Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies.|journal=Neuropathology and Applied Neurobiology|year=2007|PMID=17239015}}</ref>
A variant with combined features of MSA and [[Lewy body dementia]] may also exist. <ref>{{citejournal |author=Sikorska B, Papierz W, Preusser M, Liberski PP, Budka H.|title=Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies. |journal=Neuropathology and Applied Neurobiology |year=2007 |pmid=17239015}}</ref>


==Symptoms==
==Symptoms==
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Ongoing care from a neurologist specialized in "movement disorders" is recommended as the complex symptoms of MSA are often not familiar to less-specialized health care professionals.
Ongoing care from a neurologist specialized in "movement disorders" is recommended as the complex symptoms of MSA are often not familiar to less-specialized health care professionals.


One particularly serious problem, the drop in blood pressure upon standing up (with risk of fainting thus injury from falling) often responds to [[fludrocortisone]], a synthetic [[mineralocorticoid]]. Another common drug treatment is [[midodrine]] (an [[alpha-agonist]].) Non-drug treatments include "head-up tilt" (elevating the head of the whole bed by about 10 degrees), salt tablets or increasing salt in the diet, generous intake of fluids, and pressure (elastic) stockings. Avoidance of triggers of low blood pressure (e.g. hot weather, [[alcohol]], dehydration) are crucial..<ref name="Shy-drager from Clinical Neurology">{{cite web |url=http://www.accessmedicine.com/content.aspx?aID=2082488 |title=Aminoff MJ, Greenberg DA, Simon RP, "Chapter 7. Movement Disorders" (Chapter). Aminoff MJ, Greenberg DA, Simon RP: Clinical Neurology, 6th Edition: http://www.accessmedicine.com/content.aspx?aID=2082488. |work= |accessdate=2009-04-20}}</ref>
One particularly serious problem, the drop in blood pressure upon standing up (with risk of fainting thus injury from falling) often responds to [[fludrocortisone]], a synthetic [[mineralocorticoid]]. Another common drug treatment is [[midodrine]] (an [[alpha-agonist]].) Non-drug treatments include "head-up tilt" (elevating the head of the whole bed by about 10 degrees), salt tablets or increasing salt in the diet, generous intake of fluids, and pressure (elastic) stockings. Avoidance of triggers of low blood pressure (e.g. hot weather, [[alcohol]], dehydration) are crucial..<ref name="Shy-drager from Clinical Neurology">{{cite book |chapterurl=http://www.accessmedicine.com/content.aspx?aID=2082488 |author=Aminoff MJ, Greenberg DA, Simon RP |chapter=Chapter 7. Movement Disorders |title=Clinical Neurology |edition=6th }}</ref>


[[Hospice care|Hospice]]/homecare services can be very useful as disability progresses.
[[Hospice care|Hospice]]/homecare services can be very useful as disability progresses.
Line 67: Line 67:
==Histopathology==
==Histopathology==


In some cases, a diagnosis of MSA can only be confirmed post-mortem. When brain tissue of a person with MSA is examined under a microscope, structures called glial cytoplasmic [[inclusion bodies]] are visible. The presence of these inclusions (also known as Papp-Lantos bodies) in the movement, balance and automatic control centres of the brain are the defining histopathologic hallmark of MSA. Recent studies have shown that major fillamentous component of glial and neuronal cytoplasmic inclusions is [[alpha-synuclein]].<ref>{{cite journal |author=Arima K, Uéda K, Sunohara N, Arakawa K, Hirai S, Nakamura M, Tonozuka-Uehara H, Kawai M |title=NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy |journal=Acta Neuropathol. |volume=96 |issue=5 |pages=439–44|year=1998|month=November |pmid=9829806|doi= 10.1007/s004010050917|url=http://www.ncbi.nlm.nih.gov/pubmed/9829806?ordinalpos=39&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum}}</ref>
In some cases, a diagnosis of MSA can only be confirmed post-mortem. When brain tissue of a person with MSA is examined under a microscope, structures called glial cytoplasmic [[inclusion bodies]] are visible. The presence of these inclusions (also known as Papp-Lantos bodies) in the movement, balance and automatic control centres of the brain are the defining histopathologic hallmark of MSA. Recent studies have shown that major fillamentous component of glial and neuronal cytoplasmic inclusions is [[alpha-synuclein]].<ref>{{cite journal |author=Arima K, Uéda K, Sunohara N, Arakawa K, Hirai S, Nakamura M, Tonozuka-Uehara H, Kawai M |title=NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy |journal=Acta Neuropathol. |volume=96 |issue=5 |pages=439–44|year=1998 |month=November |pmid=9829806 |doi= 10.1007/s004010050917 }}</ref>


==Terminology==
==Terminology==
Other terms have been used to refer to this disorder, based on the predominant systems presented. These terms and their distinctions have been dropped in recent (1996 onwards) medical usage<ref>{{cite journal |author= |title=Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. The Consensus Committee of the American Autonomic Society and the American Academy of Neurology |journal=Neurology |volume=46 |issue=5 |pages=1470 |year=1996 |pmid=8628505 |doi= |url=}}</ref> and replaced with MSA subtype naming, but are helpful to understanding the older literature about this disease:
Other terms have been used to refer to this disorder, based on the predominant systems presented. These terms and their distinctions have been dropped in recent (1996 onwards) medical usage<ref>{{cite journal |author= |title=Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. The Consensus Committee of the American Autonomic Society and the American Academy of Neurology |journal=Neurology |volume=46 |issue=5 |pages=1470 |year=1996 |pmid=8628505 }}</ref> and replaced with MSA subtype naming, but are helpful to understanding the older literature about this disease:


{| class="wikitable"
{| class="wikitable"
Line 77: Line 77:
| ''Striatonigral degeneration'' || predominating [[Parkinson's disease|Parkinson's]]-like symptoms || MSA-p, "p" = parkinsonian subtype
| ''Striatonigral degeneration'' || predominating [[Parkinson's disease|Parkinson's]]-like symptoms || MSA-p, "p" = parkinsonian subtype
|-
|-
| ''Shy-Drager syndrome'' || characterized by Parkinsonism plus a more pronounced failure of the [[autonomic nervous system]]<ref>{{cite journal |author=Shy GM, Drager GA |title=A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study |journal=Arch. Neurol. |volume=2 |issue= |pages=511–27 |year=1960 |pmid=14446364 |doi= |url=}}</ref> || MSA-a, "a" = autonomic dysfunction subtype
| ''Shy-Drager syndrome'' || characterized by Parkinsonism plus a more pronounced failure of the [[autonomic nervous system]]<ref>{{cite journal |author=Shy GM, Drager GA |title=A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study |journal=Arch. Neurol. |volume=2 |pages=511–27 |year=1960 |pmid=14446364 }}</ref> || MSA-a, "a" = autonomic dysfunction subtype
|-
|-
| ''Sporadic [[Olivopontocerebellar atrophy]]'' (OPCA) || characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and dysarthria (difficulty in articulating words) || MSA - c, "c" = cerebellar dysfunction subtype
| ''Sporadic [[Olivopontocerebellar atrophy]]'' (OPCA) || characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and dysarthria (difficulty in articulating words) || MSA - c, "c" = cerebellar dysfunction subtype

Revision as of 04:49, 5 April 2010

Multiple system atrophy
SpecialtyNeurology Edit this on Wikidata

Multiple system atrophy (MSA) is a degenerative[1] neurological disorder. MSA is associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and autonomic functions of the body such as bladder control. The cause of MSA is unknown and no specific risk factors have been identified.[2] Around 55% of cases occur in men, with typical age of onset in the late 50s to early 60s.[3]

The overall prevalence of MSA is estimated at 4.6 cases per 100,000 people.[4]

Presentation

MSA is characterized by a combination of the following, which can be present in any combination:[5][6]

When autonomic failure predominates, the term Shy-Drager syndrome is often used, although this term is no longer current, given the recent terminology changes which are explained below.[7] This syndrome was named after Dr Milton Shy and Dr Glenn Drager, who identified it in 1960, but the American Autonomic Society and the American Academy of Neurology redefined it as multiple system atrophy with autonomic phenomena in 1996.[8][9][10] The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.

A variant with combined features of MSA and Lewy body dementia may also exist. [11]

Symptoms

The most common first sign of MSA is the appearance of an "akinetic-rigid syndrome" (i.e. slowness of initiation of movement resembling Parkinson's disease) found in 62% at first presentation. Other common signs at onset include problems with balance (found in 22%), followed by genito-urinary problems (9%). For men, the first sign can be erectile dysfunction (unable to achieve or sustain an erection). Both men and women often experience problems with their bladders including urgency, frequency, incomplete bladder emptying or an inability to pass urine (retention). About 1 in 5 MSA patients will suffer a fall in their first year of disease.[3]

As the disease progresses three groups of symptoms predominate. These are:

  1. Parkinsonism (slow, stiff movement, writing becomes small and spidery)
  2. Cerebellar dysfunction (difficulty coordinating movement and balance)
  3. Autonomic dysfunction (impaired automatic body functions) including:

Other symptoms such as double vision[12] can occur. Not all patients experience all of these symptoms.

Prognosis

MSA usually progresses more quickly than Parkinson's disease.[13] There is no remission from the disease. The remaining lifespan after the onset of symptoms is on average about 9 years.[14] Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years.[15] Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Treatment

There is no discovered cure for MSA, so treatment involves treating the symptoms.

Management by rehabilitation professionals (physiotherapists, occupational therapists, speech therapists, and others) for problems with walking/movement, daily tasks, and speech problems is essential. Also social workers can help with coping with disability and access to health care services, both for the person with MSA as well as his/her family caregivers.

Ongoing care from a neurologist specialized in "movement disorders" is recommended as the complex symptoms of MSA are often not familiar to less-specialized health care professionals.

One particularly serious problem, the drop in blood pressure upon standing up (with risk of fainting thus injury from falling) often responds to fludrocortisone, a synthetic mineralocorticoid. Another common drug treatment is midodrine (an alpha-agonist.) Non-drug treatments include "head-up tilt" (elevating the head of the whole bed by about 10 degrees), salt tablets or increasing salt in the diet, generous intake of fluids, and pressure (elastic) stockings. Avoidance of triggers of low blood pressure (e.g. hot weather, alcohol, dehydration) are crucial..[16]

Hospice/homecare services can be very useful as disability progresses.

Levdopa (L-Dopa), a drug used in the treatment of Parkinson's disease, fails to improve the parkinsonian symptoms of most MSA patients. A recent trial reported that only 1.5% of MSA patients experienced a > 50% improvement when taking levodopa, and even this was a transient effect lasting less than one year. Poor response to L-Dopa has been suggested as a possible element in the differential diagnosis of MSA from Parkinson's disease.

A recent study conducted in Europe failed to find an effect for the drug riluzole in treating MSA or PSP.[3]

Histopathology

In some cases, a diagnosis of MSA can only be confirmed post-mortem. When brain tissue of a person with MSA is examined under a microscope, structures called glial cytoplasmic inclusion bodies are visible. The presence of these inclusions (also known as Papp-Lantos bodies) in the movement, balance and automatic control centres of the brain are the defining histopathologic hallmark of MSA. Recent studies have shown that major fillamentous component of glial and neuronal cytoplasmic inclusions is alpha-synuclein.[17]

Terminology

Other terms have been used to refer to this disorder, based on the predominant systems presented. These terms and their distinctions have been dropped in recent (1996 onwards) medical usage[18] and replaced with MSA subtype naming, but are helpful to understanding the older literature about this disease:

Name Characteristics Abbreviation
Striatonigral degeneration predominating Parkinson's-like symptoms MSA-p, "p" = parkinsonian subtype
Shy-Drager syndrome characterized by Parkinsonism plus a more pronounced failure of the autonomic nervous system[19] MSA-a, "a" = autonomic dysfunction subtype
Sporadic Olivopontocerebellar atrophy (OPCA) characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and dysarthria (difficulty in articulating words) MSA - c, "c" = cerebellar dysfunction subtype

See also

  • Professor Sydney Selwyn, suffered from MSA.
  • David Sweetman, British poet, writer and broadcaster, suffered from MSA.
  • Glyn Worsnip, British radio and television presenter, suffered from MSA.
  • Carlos Cristo, a Spanish physician who died of MSA in 2008, is the subject of an inspiring documentary film about his coping with the disease, Las Alas de la Vida [The Wings of Life] (2007).

References

  1. ^ "multiple system atrophy" at Dorland's Medical Dictionary
  2. ^ "National Study Seeks Cause of Baffling, Fatal Disorder Called Multiple System Atrophy". UCSD Health Sciences Communications Healthbeat. December 5, 2003. Retrieved 2008-07-01.
  3. ^ a b c Bensimon G, Ludolph A, Agid Y, Vidailhet M, Payan C, Leigh PN (2008). "Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study". Brain. 132: 156. doi:10.1093/brain/awn291.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  4. ^ Prevalence of rare diseases : Bibliographic data (PDF) (Report). Orphanet. November 2008. p. 20. Retrieved 2009-01-19.
  5. ^ Swan L, Dupont J (1999). "Multiple system atrophy". Phys Ther. 79 (5): 488–94. PMID 10331752. {{cite journal}}: Unknown parameter |month= ignored (help)
  6. ^ Burn DJ, Jaros E (2001). "Multiple system atrophy: cellular and molecular pathology". MP, Mol. Pathol. 54 (6): 419–26. PMC 1187133. PMID 11724918. {{cite journal}}: Unknown parameter |month= ignored (help)
  7. ^ Jellinger KA (2007). "More frequent Lewy bodies but less frequent Alzheimer-type lesions in multiple system atrophy as compared to age-matched control brains". Acta Neuropathologica. 114 (3): 299–303. doi:10.1007/s00401-007-0227-4.
  8. ^ "Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy". Neurology. 46 (5): 1470. 1996. PMID 8628505.
  9. ^ synd/875 at Who Named It?
  10. ^ Shy GM, Drager GA (1960). "A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study". Arch. Neurol. 2: 511–27. PMID 14446364.
  11. ^ Sikorska B, Papierz W, Preusser M, Liberski PP, Budka H. (2007). "Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies". Neuropathology and Applied Neurobiology. PMID 17239015.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  12. ^ NINDS NIH MSA with Orthostatic Hypotension
  13. ^ Bower J, Maraganore D, McDonnell S, Rocca W (1997). "Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990". Neurology. 49 (5): 1284–8. PMID 9371909.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  14. ^ msa at NINDS Multiple System Atrophy
  15. ^ Watanabe H, Saito Y, Terao S; et al. (2002). "Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients". Brain. 125 (Pt 5): 1070–83. doi:10.1093/brain/awf117. PMID 11960896. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  16. ^ Aminoff MJ, Greenberg DA, Simon RP. "Chapter 7. Movement Disorders". Clinical Neurology (6th ed.). {{cite book}}: External link in |chapterurl= (help); Unknown parameter |chapterurl= ignored (|chapter-url= suggested) (help)CS1 maint: multiple names: authors list (link)
  17. ^ Arima K, Uéda K, Sunohara N, Arakawa K, Hirai S, Nakamura M, Tonozuka-Uehara H, Kawai M (1998). "NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy". Acta Neuropathol. 96 (5): 439–44. doi:10.1007/s004010050917. PMID 9829806. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  18. ^ "Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. The Consensus Committee of the American Autonomic Society and the American Academy of Neurology". Neurology. 46 (5): 1470. 1996. PMID 8628505.
  19. ^ Shy GM, Drager GA (1960). "A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study". Arch. Neurol. 2: 511–27. PMID 14446364.

External links

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