Multiple System Atrophy: Difference between revisions

No edit summary
m (Text replacement - "[[Cognitive Deficits" to "[[Cognitive Impairments")
 
(143 intermediate revisions by 9 users not shown)
Line 1: Line 1:
[[Category:Bellarmine Student Project]]<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div> <div class="editorbox">
<div class="editorbox">
'''Original Editors '''- [[Pathophysiology of Complex Patient Problems|Students from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
'''Original Editors '''- [[User:Emily Nicklies|Emily Nicklies]] from [[Pathophysiology of Complex Patient Problems|Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  


'''Lead Editors''' - Your name will be added here if you are a lead editor on this page.&nbsp; [[Physiopedia:Editors|Read more.]]
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
</div>  
</div>  
== Definition/Description<br>  ==
== Introduction  ==
[[File:Parkinson's man sketches.jpg|right|frameless|209x209px]]
Multiple System Atrophy (MSA)&nbsp;is defined as a sporadic, fatal, progressive, [[Neurodegenerative Disease|neurodegenerative]] (specifically a [[synucleinopathy]]) adult-onset disorder. It is the second most common neurodegenerative movement disorder with an average annual incidence rate of 3 per 100000 person-years after [[Parkinson's|Parkinson’s disease]] (PD)<ref name=":3">Dash S, Mahale R, Netravathi M, Kamble NL, Holla V, Yadav R, Pal PK. [https://www.cureus.com/articles/83909-cognition-in-patients-with-multiple-system-atrophy-msa-and-its-neuroimaging-correlation-a-prospective-case-control-study Cognition in Patients With Multiple System Atrophy (MSA) and Its Neuroimaging Correlation: A Prospective Case-Control Study]. Cureus. 2022 Jan 29;14(1). Available:https://www.cureus.com/articles/83909-cognition-in-patients-with-multiple-system-atrophy-msa-and-its-neuroimaging-correlation-a-prospective-case-control-study (accessed 14.3.2022)</ref>. It can affect the
* Autonomic system causing autonomic failure, causing eg.fainting spells and problems with [[Heart Arrhythmias|heart rate]], erectile dysfunction, and [[Urinary Incontinence|bladder control]].
* [[Basal Ganglia|Basal ganglia]] causing [[Parkinson's|parkinsonism]], causing eg tremor, rigidity, and/or loss of muscle coordination as well as difficulties with speech and [[gait]].
* [[Cerebellum]] causing [[ataxia]] <ref name="Lundy Eckman">Lundy-Eckman, L.  Neuroscience: Fundamentals for Rehabilitation.  3rd ed. St. Louis: Saunders Elsevier, 2002.</ref>
The symptoms reflect the progressive loss of function and death of different types of nerve cells in the [[Brain Anatomy|brain]] and [[Spinal cord anatomy|spinal cord]].


Multiple System Atrophy (MSA)&nbsp;is defined as a sporadic, progressive, neurodegenerative adult-onset disorder that can affect the autonomic system, basal ganglia causing parkinsonism, and/or cerebellum causing ataxia in any combination.(Lundy-Eckman)<ref name="Lundy Eckman">Lundy-Eckman, L.  Neuroscience: Fundamentals for Rehabilitation. 3rd ed. St. Louis: Saunders Elsevier, 2002.</ref>  
Some of these features are similar to those seen in Parkinson’s disease, and early in the disease course it often may be difficult to distinguish these disorders. This 1 minute video is titled Differentiating Multiple System Atrophy From Parkinson's Disease.
{{#ev:youtube |FZlk8a064N4|width}}<ref>MSA coalition Differentiating Multiple System Atrophy From Parkinson's Disease (PSA) Available: https://www.youtube.com/watch?v=CD7UL12GSCM<nowiki/>(accessed 14.3.2022)</ref>


This disorder is called Multiple System Atrophy, because there is a combination of symptoms arising from each system just mentioned (autonomic system, basal gangila, and cerebellum). &nbsp;In order for a patient to be diagnosed with MSA, there have to be at least two systems involved.&nbsp;There can be any combination of symptoms present from the three systems. (Swan)<ref name="Swan">Swan L, Dupont J.  Multiple System Atrophy.  Journal of Physical Therapy 1999;79:488-94.</ref>  
=== Etiology ===
The causes of MSA are unknown; however, as for other neurodegenerative diseases, a complex interaction of genetic and environmental mechanisms seems likely.<ref name=":1">Jellinger KA. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870010/ Multiple system atrophy: an oligodendroglioneural synucleinopathy]. Journal of Alzheimer's Disease. 2018 Jan 1;62(3):1141-79 Available from.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870010/ (last accessed 16.1.2020)</ref><br>Research has suggested that MSA is characterized by a progressive loss of neuronal and oligodendro[[Glial Cells|glial cells]] (myelin producing cells in CNS) in numerous sites in the CNS (basal ganglia, cerebellum). This damage is said to occur from the formation of glial cytoplasmic inclusions (GCI’s).<ref name="Diedrich">Diedrich A, Robertson D.  Multiple System Atrophy.  Vanderbilt University School of Medicine 2009. http://emedicine.medscape.com/article/1154583-overview (accessed on 24 Jan 2010).</ref>


*In MSA, patients often have symptoms that are characteristic of autonomic dysfunction, parkinsonism, or cerebellar dysfunction. As the disease progresses, additional symptoms emerge (Swan).<ref name="Swan" />
== Classification ==
*Some patients may initially have autonomic features and may be diagnosed with pure autonomic failure. (Swan)<ref name="Swan" /><br>o Patients who are initially misdiagnosed with PAF may later be correctly diagnosed with MSA as the neurological symptoms of parkinsonism and/or cerebellar ataxia appear. (Swan).<ref name="Swan" />
MSA is classified by the most dominant symptom exhibited by the patient.


There are three different categories or types of MSA, based upon what areas of the nervous system or autonomic system are affected and to what degree:  
# Parkinsonian type (MSA-P): this includes symptoms similar to Parkinson's disease eg tremors at rest, rigidity of muscles, and slow movements, including gait
# Cerebellar type (MSA-C): involves difficulty walking (ataxia), issues maintaining [[balance]], and trouble [[Coordination Exercises|coordinating]] voluntary movements<ref name=":0">Very well health MSA Available: https://www.verywellhealth.com/multiple-system-atrophy-2860862<nowiki/>(accessed 14.3.2022)</ref>.


*Clinically, MSA is dominated by autonomic failure (MSA-A subtype) (also referred to as ''Shy Drager Syndrome''), which may be associated with either:
=== Epidemiology ===
*the movement dysfunction of parkinsonism (MSA-P subtype) (also referred to as ''Striatonigral degeneration'') in 80% of cases. 2,3<ref name="Wenning">Wenning GK, Braune S.  Multiple System Atrophy: Pathophysiology and Management.  CNS Drugs 2001;15:839-48.</ref><ref name="Wedge">Wedge F.  The Impact of Resistance Training on Balance and Functional Ability of a Patient with Multiple System Atrophy.  Journal of Geriatric Physical Therapy 2008;31:79-83.</ref>
[[File:MSE Dementia pic.jpg|thumb]]
*or with cerebellar ataxia (MSA-C subtype) (also referred to as ''Sporadic Olivopontocerebellar atrophy OPCA''), which will result in difficulty with postural control and coordination in 20-50% of cases. 2,3,<ref name="Wenning" /><ref name="Wedge" />
MSA is an orphan disease with an incidence of up to 2.4 cases per 100 000 persons per year, while the prevalence may reach up to 7.8 patients per 100 000 population over the age of 40. The MSA‐P variant seems to be more common in the western hemisphere, whereas MSA‐C appears to be more frequent in Asia<ref name=":4">Virtual strategy Multiple System Atrophy Epidemiology Forecast to 2030 Available: https://virtual-strategy.com/2020/07/16/multiple-system-atrophy-epidemiology-forecast-to-2030/ (accessed 14.3.2022)</ref> .


== Prevalence  ==
There is about 1 living case of MSA in the population for every 40 cases of Parkinson’s disease, but because survival in MSA is shorter than for PD, about 1 new MSA case presents every year for about every 20 who present with PD.<ref name=":4" />


The prevalence of MSA has been estimated to be 4.4 per 100,000 people. (Diedrich).<ref name="Diedrich">Diedrich A, Robertson D.  Multiple System Atrophy.  Vanderbilt University School of Medicine 2009. http://emedicine.medscape.com/article/1154583-overview (accessed on 24 Jan 2010).</ref>
=== Symptoms ===
The symptoms reflect the progressive loss of function and death of different types of [[Neurone|nerve cells]] in the brain and spinal cord. The initial symptoms of MSA are often difficult to distinguish from the initial symptoms of Parkinson’s disease and include: slowness of movement, tremor, or rigidity (stiffness); clumsiness or incoordination; impaired speech, a croaky, quivering voice; fainting or lightheadedness due to [[Orthostatic Hypotension|orthostatic hypotension]]; bladder control problems, eg a sudden urge to urinate or difficulty emptying the bladder<ref name=":4" />.


<br>The mean age at onset has been reported to be between 52.5 and 55 years (Diedrich).<ref name="Diedrich" />
The 2 minute video below gives a good introduction to MSA
{{#ev:youtube|https://www.youtube.com/watch?v=mVqiCgaLSf4&app=desktop|width}}<ref>MSA coalition.


<br>MSA is found more often in men than in women (Diedrich).<ref name="Diedrich" />
What is MSA available from:https://www.youtube.com/watch?v=mVqiCgaLSf4&app=desktop (last accessed 17.1.2020)</ref>
=== Cognition ===
[[File:Cerebrum animation small2.gif|thumb|150x150px|Frontal lobe red Temporal lobe green]]
[[Cognitive Impairments|Cognitive deficits]] occur in all cognitive domains. The impaired cognitive domains had a significant correlation with atrophy of frontotemporal [[Cerebral Cortex|cortical]] areas, [[Basal Ganglia|basal ganglia]], [[thalamus]], and [[cerebellum]]<ref name=":3" />. Cognitive impairment, particularly seen in executive function, may occur in up to 75% of patients The [[Mini-Mental State Examination]], or MMSE, yielded 3% of participants showing signs of cognitive decline while a test called the Frontal Assessment Battery (FAB) categorized 41% of participants as being cognitively impaired. The FAB is a cognitive test that incorporates several clinical assessments to screen for [[Frontotemporal Dementia|frontotemporal dementia]] (FTD)


<br>Wenning et al- completed an analysis of 100 patients with MSA and measured their disability. They found that patients with MSA become disabled at a faster rate than patients with Parkinson's Disease.<ref name="Wenning" />  
* Emotional instability may occur later in the progression of the disease
* Depression, anxiety, panic attacks, and suicidal ideation may present in MSA  
* Patients with MSA can have great difficulty switching attention from one stimulus to another,<ref name="Swan">Swan L, Dupont J.  Multiple System Atrophy.  Journal of Physical Therapy 1999;79:488-94.</ref>&nbsp;with a&nbsp;decrease in goal-oriented cognitive ability.


<br>
== Diagnosis ==
At this time, there are no specific symptoms, blood tests or imaging studies that distinguish MSA<ref name=":2">MSA Coalition. [https://www.multiplesystematrophy.org/wp-content/uploads/2019/09/What-You-Need-to-Know-9.4.19-DSC-AM-NV-Edits2.pdf MSA what you need to know] 4.4.2019. Available from: https://www.multiplesystematrophy.org/wp-content/uploads/2019/09/What-You-Need-to-Know-9.4.19-DSC-AM-NV-Edits2.pdf (last accessed 17.1.2020)</ref>. It can take a while to receive an MSA diagnosis because of the similarities between MSA and other conditions, eg Parkinson's disease. MSA is usually diagnosed around 50 years of age and is seen in people of all ethnic backgrounds. Once symptoms begin, the disease tends to progress quite rapidly.


== Characteristics/Clinical Presentation  ==
The Mini-Mental State Examination, or MMSE, yielded 3% of participants showing signs of cognitive decline while a test called the Frontal Assessment Battery (FAB) categorized 41% of participants as being cognitively impaired<ref>MAS coalition Depression and Cognitive Impairment in MSA Available:https://www.multiplesystematrophy.org/about-msa/depression-cognitive-impairment/ (accessed 14.3.2022)</ref>. The FAB is a cognitive test that incorporates several clinical assessments to screen for frontotemporal dementia (FTD),


MSA is classified by the most dominant symptom exhibited by the patient.  
'''Diagnostic Challenge''' There is no “typical” presentation for this disorder, and MSA can often be masked by other diagnoses. Patients with MSA are most commonly misdiagnosed with [[Parkinsons Disease|Parkinson’s Disease]]. About 1/3 of people with MSA die while still misdiagnosed.<ref name="Lundy Eckman" /> Only 25% of patients with MSA are correctly diagnosed at their first neurological visit. The correct diagnosis is usually established on an average of 4 to 5 years after the disease onset.<ref name="Wenning">Wenning GK, Braune S.  Multiple System Atrophy: Pathophysiology and Management.  CNS Drugs 2001;15:839-48.</ref>
=== Management ===
Currently, there is no cure for MSA, nor are there any treatments specifically designed to reverse or stop disease progression.  


*When Parkinson-like movement dysfunction is the more prominent feature, the disease is categorized as MSA-P. Parkinsonism has been identified as the initial feature in 46% of patients with MSA, but eventually over 80% of patients with MSA will develop parkinsonian features.(wedge)<ref name="Wedge" />
* Movement disorders can be treated with levodopa and carbidopa (Sinemet), but this usually has limited results.
*Characteristics of parkinsonism include bilateral involvement, bradykinesia, impaired writing, slurred speech, and rigidity (Swan)<ref name="Swan" />, postural and rest tremor disequilibrium and gait unsteadiness (Diedrich).<ref name="Diedrich" />
* Other medications such as trihexyphenidyl (Artane), benztropine mesylate (Cogentin), and amantadine (Symmetrel), may also offer some symptom relief. Several medications exist to treat orthostatic hypertension.
* Physical and occupational therapy, eg aqua therapy, can help maintain muscle function, and speech therapy can help improve any difficulties swallowing or speaking<ref name=":0" />.


<br>  
=== Physical Therapy Management ===
[[File:Man-walker dementia.jpg|thumb|Walking Aid]]
Physical therapies offer drug-free tools to maintain their function for as long as possible, by helping keep muscles strong and flexible and helping prevent [[falls]]. Encouraging mobility also lowers the risk of [[Pulmonary Embolism|pulmonary embolism]] which can be fatal<ref name=":2" />.


*When cerebellar dysfunction is more prominent, the disease is labeled as MSA-C. &nbsp;Over 50% of individuals with MSA will present with cerebellar dysfunction. (wedge)<ref name="Wedge" />
Ideally a neurological physiotherapist will assess the client and then make a tailored program. It incorporates a combination of progressive fitness, balance, movement and strength training and stretching, as well as education on how to manage symptoms. Make PT goals realistic.  Base the goals upon current function and take into consideration the activities most important to the patient.  
*Cerebellar features that are characteristic of MSA have their initial manifestations in the trunk and lower extremities, leading to disturbances in gait. (Swan)<ref name="Swan" />. Also display limb kinetic ataxia and scanning dysarthria as well as cerebellar oculomotor disturbances. (Diedrich).<ref name="Diedrich" />


<br>
[[Gait]]/Mobility Training:


*Autonomic dysfunction, primarily orthostatic hypotension, is present in approximately 75% of people with MSA. (Wedge)<ref name="Wedge" />
# Fit the patient for the most appropriate [[Walking Aids|walking aids]] in order to gain independent mobility. &nbsp;This will depend upon the patient’s level of function and safety.
*Autonomic dysfunction commonly appears as postural/orthostatic hypotension associated with impaired or absent reflex tachycardia upon standing, bowel and bladder incontinence, impotence, hypohydrosis. (Swan and Diedrich).<ref name="Swan" /><ref name="Diedrich" />
# If a [[Wheelchair Assessment - Assessment Interview|wheelchair]] is most appropriate, ensure the chair is one that will best benefit their posture and mobility based upon the function of patient.&nbsp;As a PT, the need to promote independent functional mobility and optimal posture with safety in mind is most important.


<br>  
[[File:Hip exercise 6.png|right|frameless]]
Therapeutic Exercise/Activities:
*[[Moving and Handling|Transfer training]]
*[[Balance]] activities<ref name="Wedge">Wedge F.  The Impact of Resistance Training on Balance and Functional Ability of a Patient with Multiple System Atrophy.  Journal of Geriatric Physical Therapy 2008;31:79-83.</ref>eg [[Otago Exercise Programme|Otago balance porogram]]
*[[Strength Training|Strength training]] focusing  on  [[Knee Extensors|knee extensors]] and [[Knee Flexors|flexors]], [[Hip Abductors|hip abductors]] and [[Hip Adductors|adductors]], and ankle plantar flexors .These muscle groups are chosen because of their importance to balance.<ref name="Wedge" /> Can lead to improvements in the patient's gait pattern (better at maintaining good step height and length even) and reduces festinating gait.
*Postural muscle strengthening to improve eg head and trunk alignment in both sitting and standing).<ref name="Wedge" />


*Dysfunction to the pyramidal/corticospinal tracts can also occur, but does not have its own subtype. This will often result in impaired muscle performance. (Wedge).<ref name="Wedge" />
Education
*&nbsp;Signs of corticospinal tract dysfunction (hyperreflexia, spasticity, and a positive Babinski sign) are features characteristic of MSA, but are not used as criteria for diagnosis. (Swan).<ref name="Swan" />


<br>
*Education of the patient and family on benefits of PT is important for patient/family knowledge and compliance.


There is no “typical” presentation of this disorder, which makes it hard to pick up, and patients often go misdiagnosed. MSA may also goes misdiagnosed because the disorder initially (as mentioned previously) manifests with S/S of only one system. (Lundy-Eckman)<ref name="Lundy Eckman" />  
=== Differential Diagnosis ===
MSA often presents as the following disorders: Pure [[Parkinson's|Parkinsonism]]<ref name="Hardy">Hardy, Joanne.  Multiple System Atrophy: Pathophysiology, Treatment and Nursing Care.  Nursing Standard 2008;22:50-6.</ref>; Pure Autonomic Failu<nowiki/>re (PAF)<ref name="Hardy" />; [[Progressive Supranuclear Palsy]]<ref name="O'Sullivan">O'Sullivan SS, Massey LA, Williams DR, Silveira-moriyama L, Kempster PA, Holton JL, Revesz T,  Lees, AJ.  Clinical Outcomes of Progressive Supranuclear Palsy and Multiple System Atrophy. Brain.  2008;131:1362-72</ref>; Corticobasal Ganglionic Degeneration<ref name="Hain">Hain TC.  Multiple System Atrophy.  2010.  http://www.dizziness-and-balance.com/disorders/central/movement/msa.html (accessed on 5 april 2010).</ref>


*Parkinson’s is the most common misdiagnosis. About 1/3 of people with MSA die while still misdiagnosed. (Lundy-Eckman).<ref name="Lundy Eckman" />
=== Prognosis and Outlook ===
*Only 25% of patients with MSA are correctly diagnosed at their first neurological visit. The correct diagnosis is usually established on average 4 to 5 years after disease onset (Wenning).<ref name="Wenning" />
Prognosis is currently guarded, with most MSA patients passing away from the disease or its complications within 6-10 years after the onset of symptoms. Nonetheless, there is reason for hope for MSA research goes on. Since the biology of MSA may be related to other neurodegenerative diseases like Parkinson's disease, it is possible that therapies designed for other conditions will also prove helpful for patients with MSA.<ref name=":2" />
 
== References ==
<br>
 
It is important to differentiate MSA from pure autonomic failure (PAF) and pure Parkinsonism.
 
*According to Lundy-Eckman, the distinction between MSA and Parkinsonism&nbsp;is made by exclusion. &nbsp;If no autonomic or cerebellar signs are present, then the patient has pure Parkinsonism.<ref name="Lundy Eckman" />
*It is also important to note that up to 90% of patients with MSA-P treated with levodopa (L-Dopa) fail to show a sustained, long-term response. (Wenning)<ref name="Wenning" /> This is a sign that this patient does not have pure Parkinsonism, as L-Dopa is not effective.  
*In addition, orthostatic hypotension, difficulty urinating, rapid progression of functional limitations, loud breathing,and impotence indicate that MSA is a more likely Dx than Parkinson’s Disease.<ref name="Wenning" />
*According to Lundy-Eckman, a patient has pure autonomic failure if ANS signs (such as orthostatic hypotension) are present, but signs of parkinsonism or cerebellar are not, then most likely PAF.<ref name="Lundy Eckman" />
 
<br>
 
Level of cognition:
 
*&nbsp;While there is generally an absence of marked cognitive impairment, emotional instability may occur later in the progression of the disease. (Wedge)<ref name="Wedge" />
*Patients with MSA can have great difficulty switching attention from one stimulus to another (Swan)<ref name="Swan" /> and a decrease in goal-oriented cognitive ability has also been noted in MSA patients (Lundy-Eckman).<ref name="Lundy Eckman" /><br>
 
Bowel and Bladder Dysfunction:
 
*Genitourinary dysfunctions are common symptoms of MSA. Urinary bladder dysfunction may include frequency, urgency, incontinence, and retention. &nbsp;Men may experience erectile difficulties early in the course of the disease. &nbsp;Constipation is also common problem. (Swan).<ref name="Swan" />
 
Symptoms Occurring Later as the Disease Progresses:  
 
*Dysphagia and dysarthria are symptoms that occur later in the disease (Swan)<ref name="Swan" /> and should be treated by speech therapy.
 
<br>
 
As there is no “typical” presentation for this disorder, and MSA can often be masked by other diagnoses, it is important if one has a patient with pure Parkinson’s, PAF, etc to keep a watchful eye of any suspicious S/S that are not associated with that system alone. This is pertinent so that these patients do not go misdiagnosed.
 
<br>  
 
<br>
 
== Associated Co-morbidities ==


add text here <br>
<references />  
 
== Medications  ==
 
Since MSA is a variable disorder and can present in many different ways, treatment is symptomatic.<br>
 
'''Pharmacological Treatment:'''
 
MSA-A
 
*To treat orthostatic hypotension: (is defined as a fall in BP on standing of more than 20mmHg systolic or 10 mmHg diastolic) (Hardy)<ref name="Hardy">Hardy, Joanne.  Multiple System Atrophy: Pathophysiology, Treatment and Nursing Care.  Nursing Standard 2008;22:50-6.</ref> Drugs can be prescribed to enhance vasoconstriction and increase the blood volume, which will increase the blood pressure (''Fludrocortisone'' and ''Midodrine'') (Lundy-Eckman).<ref name="Lundy Eckman" />
*To treat constipation: Stool softener medications<ref name="Hardy" />
*To treat urinary dysfunction:&nbsp;Pharmacological intervention does not adequately reduce post-void residual volume in patients with MSA, but anticholinergic agents like ''Oxybutynin'' can improve symptoms of hyper-reflexia. (Wenning).<ref name="Wenning" />
*To treat impotence:&nbsp;''Sildenafil'' (25 to 75mg) may be successful in treating erectile failure. (Wenning).<ref name="Wenning" />
 
<br>
 
MSA-C
 
*There are no currently established pharmacological treatment strategies for cerebellar ataxia and pyramidal dysfunction (Wenning).<ref name="Wenning" />
 
 
 
MSA-P
 
*''Dopamimetics'' (''Levodopa'', ''Dopamine''&nbsp;''Agonists'') and ''Anticholinergics''/''Amantadine''. (Wenning).<ref name="Wenning" />
 
<br>
 
== Diagnostic Tests/Lab Tests/Lab Values  ==
 
add text here <br>
 
== Causes  ==
 
add text here <br>
 
== Systemic Involvement  ==
 
add text here
 
== Medical Management (current best evidence)  ==
 
add text here
 
== Physical Therapy Management (current best evidence)  ==
 
add text here
 
== Alternative/Holistic Management (current best evidence)  ==
 
add text here
 
== Differential Diagnosis  ==
 
add text here
 
== Case Reports  ==
 
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
 
== Resources <br>  ==
 
add appropriate resources here
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
 
see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]
<div class="researchbox">
<rss>Feed goes here!!|charset=UTF-8|short|max=10</rss>
</div>  
== References  ==


see [[Adding References|adding references tutorial]].
[[Category:Neurological - Conditions]]
[[Category:Bellarmine_Student_Project]]  


<references />
[[Category:Occupational Health]]

Latest revision as of 11:15, 17 February 2023

Original Editors - Emily Nicklies from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Emily Nicklies, Lucinda hampton, Admin, Kim Jackson, Dave Pariser, Wendy Walker, WikiSysop, Amrita Patro, Elaine Lonnemann and Naomi O'Reilly  

Introduction[edit | edit source]

Parkinson's man sketches.jpg

Multiple System Atrophy (MSA) is defined as a sporadic, fatal, progressive, neurodegenerative (specifically a synucleinopathy) adult-onset disorder. It is the second most common neurodegenerative movement disorder with an average annual incidence rate of 3 per 100000 person-years after Parkinson’s disease (PD)[1]. It can affect the

The symptoms reflect the progressive loss of function and death of different types of nerve cells in the brain and spinal cord.

Some of these features are similar to those seen in Parkinson’s disease, and early in the disease course it often may be difficult to distinguish these disorders. This 1 minute video is titled Differentiating Multiple System Atrophy From Parkinson's Disease.

[3]

Etiology[edit | edit source]

The causes of MSA are unknown; however, as for other neurodegenerative diseases, a complex interaction of genetic and environmental mechanisms seems likely.[4]
Research has suggested that MSA is characterized by a progressive loss of neuronal and oligodendroglial cells (myelin producing cells in CNS) in numerous sites in the CNS (basal ganglia, cerebellum). This damage is said to occur from the formation of glial cytoplasmic inclusions (GCI’s).[5]

Classification[edit | edit source]

MSA is classified by the most dominant symptom exhibited by the patient.

  1. Parkinsonian type (MSA-P): this includes symptoms similar to Parkinson's disease eg tremors at rest, rigidity of muscles, and slow movements, including gait
  2. Cerebellar type (MSA-C): involves difficulty walking (ataxia), issues maintaining balance, and trouble coordinating voluntary movements[6].

Epidemiology[edit | edit source]

MSE Dementia pic.jpg

MSA is an orphan disease with an incidence of up to 2.4 cases per 100 000 persons per year, while the prevalence may reach up to 7.8 patients per 100 000 population over the age of 40. The MSA‐P variant seems to be more common in the western hemisphere, whereas MSA‐C appears to be more frequent in Asia[7] .

There is about 1 living case of MSA in the population for every 40 cases of Parkinson’s disease, but because survival in MSA is shorter than for PD, about 1 new MSA case presents every year for about every 20 who present with PD.[7]

Symptoms[edit | edit source]

The symptoms reflect the progressive loss of function and death of different types of nerve cells in the brain and spinal cord. The initial symptoms of MSA are often difficult to distinguish from the initial symptoms of Parkinson’s disease and include: slowness of movement, tremor, or rigidity (stiffness); clumsiness or incoordination; impaired speech, a croaky, quivering voice; fainting or lightheadedness due to orthostatic hypotension; bladder control problems, eg a sudden urge to urinate or difficulty emptying the bladder[7].

The 2 minute video below gives a good introduction to MSA

[8]

Cognition[edit | edit source]

Frontal lobe red Temporal lobe green

Cognitive deficits occur in all cognitive domains. The impaired cognitive domains had a significant correlation with atrophy of frontotemporal cortical areas, basal ganglia, thalamus, and cerebellum[1]. Cognitive impairment, particularly seen in executive function, may occur in up to 75% of patients The Mini-Mental State Examination, or MMSE, yielded 3% of participants showing signs of cognitive decline while a test called the Frontal Assessment Battery (FAB) categorized 41% of participants as being cognitively impaired. The FAB is a cognitive test that incorporates several clinical assessments to screen for frontotemporal dementia (FTD)

  • Emotional instability may occur later in the progression of the disease
  • Depression, anxiety, panic attacks, and suicidal ideation may present in MSA
  • Patients with MSA can have great difficulty switching attention from one stimulus to another,[9] with a decrease in goal-oriented cognitive ability.

Diagnosis[edit | edit source]

At this time, there are no specific symptoms, blood tests or imaging studies that distinguish MSA[10]. It can take a while to receive an MSA diagnosis because of the similarities between MSA and other conditions, eg Parkinson's disease. MSA is usually diagnosed around 50 years of age and is seen in people of all ethnic backgrounds. Once symptoms begin, the disease tends to progress quite rapidly.

The Mini-Mental State Examination, or MMSE, yielded 3% of participants showing signs of cognitive decline while a test called the Frontal Assessment Battery (FAB) categorized 41% of participants as being cognitively impaired[11]. The FAB is a cognitive test that incorporates several clinical assessments to screen for frontotemporal dementia (FTD),

Diagnostic Challenge There is no “typical” presentation for this disorder, and MSA can often be masked by other diagnoses. Patients with MSA are most commonly misdiagnosed with Parkinson’s Disease. About 1/3 of people with MSA die while still misdiagnosed.[2] Only 25% of patients with MSA are correctly diagnosed at their first neurological visit. The correct diagnosis is usually established on an average of 4 to 5 years after the disease onset.[12]

Management[edit | edit source]

Currently, there is no cure for MSA, nor are there any treatments specifically designed to reverse or stop disease progression.

  • Movement disorders can be treated with levodopa and carbidopa (Sinemet), but this usually has limited results.
  • Other medications such as trihexyphenidyl (Artane), benztropine mesylate (Cogentin), and amantadine (Symmetrel), may also offer some symptom relief. Several medications exist to treat orthostatic hypertension.
  • Physical and occupational therapy, eg aqua therapy, can help maintain muscle function, and speech therapy can help improve any difficulties swallowing or speaking[6].

Physical Therapy Management[edit | edit source]

Walking Aid

Physical therapies offer drug-free tools to maintain their function for as long as possible, by helping keep muscles strong and flexible and helping prevent falls. Encouraging mobility also lowers the risk of pulmonary embolism which can be fatal[10].

Ideally a neurological physiotherapist will assess the client and then make a tailored program. It incorporates a combination of progressive fitness, balance, movement and strength training and stretching, as well as education on how to manage symptoms. Make PT goals realistic.  Base the goals upon current function and take into consideration the activities most important to the patient.

Gait/Mobility Training:

  1. Fit the patient for the most appropriate walking aids in order to gain independent mobility.  This will depend upon the patient’s level of function and safety.
  2. If a wheelchair is most appropriate, ensure the chair is one that will best benefit their posture and mobility based upon the function of patient. As a PT, the need to promote independent functional mobility and optimal posture with safety in mind is most important.
Hip exercise 6.png

Therapeutic Exercise/Activities:

Education

  • Education of the patient and family on benefits of PT is important for patient/family knowledge and compliance.

Differential Diagnosis[edit | edit source]

MSA often presents as the following disorders: Pure Parkinsonism[14]; Pure Autonomic Failure (PAF)[14]; Progressive Supranuclear Palsy[15]; Corticobasal Ganglionic Degeneration[16]

Prognosis and Outlook[edit | edit source]

Prognosis is currently guarded, with most MSA patients passing away from the disease or its complications within 6-10 years after the onset of symptoms. Nonetheless, there is reason for hope for MSA research goes on. Since the biology of MSA may be related to other neurodegenerative diseases like Parkinson's disease, it is possible that therapies designed for other conditions will also prove helpful for patients with MSA.[10]

References[edit | edit source]

  1. ↑ 1.0 1.1 Dash S, Mahale R, Netravathi M, Kamble NL, Holla V, Yadav R, Pal PK. Cognition in Patients With Multiple System Atrophy (MSA) and Its Neuroimaging Correlation: A Prospective Case-Control Study. Cureus. 2022 Jan 29;14(1). Available:https://www.cureus.com/articles/83909-cognition-in-patients-with-multiple-system-atrophy-msa-and-its-neuroimaging-correlation-a-prospective-case-control-study (accessed 14.3.2022)
  2. ↑ 2.0 2.1 Lundy-Eckman, L. Neuroscience: Fundamentals for Rehabilitation. 3rd ed. St. Louis: Saunders Elsevier, 2002.
  3. ↑ MSA coalition Differentiating Multiple System Atrophy From Parkinson's Disease (PSA) Available: https://www.youtube.com/watch?v=CD7UL12GSCM(accessed 14.3.2022)
  4. ↑ Jellinger KA. Multiple system atrophy: an oligodendroglioneural synucleinopathy. Journal of Alzheimer's Disease. 2018 Jan 1;62(3):1141-79 Available from.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870010/ (last accessed 16.1.2020)
  5. ↑ Diedrich A, Robertson D. Multiple System Atrophy. Vanderbilt University School of Medicine 2009. http://emedicine.medscape.com/article/1154583-overview (accessed on 24 Jan 2010).
  6. ↑ 6.0 6.1 Very well health MSA Available: https://www.verywellhealth.com/multiple-system-atrophy-2860862(accessed 14.3.2022)
  7. ↑ 7.0 7.1 7.2 Virtual strategy Multiple System Atrophy Epidemiology Forecast to 2030 Available: https://virtual-strategy.com/2020/07/16/multiple-system-atrophy-epidemiology-forecast-to-2030/ (accessed 14.3.2022)
  8. ↑ MSA coalition. What is MSA available from:https://www.youtube.com/watch?v=mVqiCgaLSf4&app=desktop (last accessed 17.1.2020)
  9. ↑ Swan L, Dupont J. Multiple System Atrophy. Journal of Physical Therapy 1999;79:488-94.
  10. ↑ 10.0 10.1 10.2 MSA Coalition. MSA what you need to know 4.4.2019. Available from: https://www.multiplesystematrophy.org/wp-content/uploads/2019/09/What-You-Need-to-Know-9.4.19-DSC-AM-NV-Edits2.pdf (last accessed 17.1.2020)
  11. ↑ MAS coalition Depression and Cognitive Impairment in MSA Available:https://www.multiplesystematrophy.org/about-msa/depression-cognitive-impairment/ (accessed 14.3.2022)
  12. ↑ Wenning GK, Braune S. Multiple System Atrophy: Pathophysiology and Management. CNS Drugs 2001;15:839-48.
  13. ↑ 13.0 13.1 13.2 Wedge F. The Impact of Resistance Training on Balance and Functional Ability of a Patient with Multiple System Atrophy. Journal of Geriatric Physical Therapy 2008;31:79-83.
  14. ↑ 14.0 14.1 Hardy, Joanne. Multiple System Atrophy: Pathophysiology, Treatment and Nursing Care. Nursing Standard 2008;22:50-6.
  15. ↑ O'Sullivan SS, Massey LA, Williams DR, Silveira-moriyama L, Kempster PA, Holton JL, Revesz T, Lees, AJ. Clinical Outcomes of Progressive Supranuclear Palsy and Multiple System Atrophy. Brain. 2008;131:1362-72
  16. ↑ Hain TC. Multiple System Atrophy. 2010. http://www.dizziness-and-balance.com/disorders/central/movement/msa.html (accessed on 5 april 2010).
Retrieved from "https://www.physio-pedia.com/index.php?title=Multiple_System_Atrophy&oldid=327247"