Multiple System Atrophy Student - PowerPoint Presentation

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Multiple System Atrophy

Student

Inservice

by

kellie

adams

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Learning Objectives

The rehabilitation specialist will be able to identify the two different types of Multiple System Atrophy and the distinguishing characteristics of each type to ensure proper rehabilitation treatment based on impairment

The rehabilitation specialist will be able to identify the three distinguishable clinical signs for diagnosis of Multiple System Atrophy to allow contribution to diagnosis in a multidisciplinary team

The rehabilitation specialist will be able to discuss the neurodegenerative changes in the brain that cause the clinical symptoms associated with Multiple System Atrophy to allow for formation of a focused plan of care in rehabilitation

The rehabilitation specialist will be able to describe appropriate physical therapy interventions at each stage of mobility decline in MSA: preserved mobility, impaired mobility, and severely compromised mobility to ensure maintenance of patient independence and quality, patient centered care

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History of Multiple Systems atrophy

First described in 1960 by Dr. Milton Shy and Dr. Glen Drager, leading to the less common title of Shy-Drager Syndrome

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The term “Multiple System Atrophy” was first used in 1969 to include three previously described neurologic disorders: olivopontocerebellar atrophy, autonomic dysfunction, and

striatonigral

degeneration2

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Types of Multiple system atrophy

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MSA- P:

Primary characteristics similar to Parkinson’s Disease:

Tremors

StiffnessBalance problems IncoordinationANS dysfunction

MSA- C:

Primary characteristics due to cerebellar dysfunction:

Ataxia

Difficulty swallowing

Speech abnormalities

Abnormal eye movements

ANS dysfunction

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Diagnosis of multiple system atrophy

Key distinguishing clinical signs at diagnosis include

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:

Autonomic failure

Poor response to LevodopaAkinetic rigid Parkinsonism OR cerebellar ataxia Presence of widespread Glial Cytoplasmic inclusions are the only reliable criteria for the

definitive diagnosis of MSADiagnosis proposed from clinical history and brief neurological screen5Autonomic testing

Bladder function screenMRIPET scanLevodopa trial

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Diagnosis of msa

: hot cross bun sign

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“Hot cross bun” sign found on T2 weighted MRI was said to be highly specific for diagnosis of MSA

Due to loss of pontine neurons and myelinated transverse pontocerebellar fibers with preservation of corticospinal tracts

Recent findings have shown the hot cross bun sign to be diagnostic of patients with Parkinsonism secondary to vasculitis and spinocerebellar atrophy as well

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Glial cytoplasmic inclusions

Neurodegenerative changes in the brain leading to clinical symptoms

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Signs and symptoms of msa3

Autonomic Symptoms:

Fainting spells

Difficulty controlling heart rate

Bladder control

Erectile dysfunctionOrthostatic hypotension

Motor Symptoms:

Loss of muscle control and movementTremorRigidityMuscle incoordinationSpeech difficultiesGait abnormalities

Contractures

Antecollis

Pisa syndrome

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Prevalence of msa

15,000-50,000 Americans suffer from MSA

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1,900 new cases per year in the U.S.

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Incidence of 0.6 per 100,000 people worldwide2 Affects men and women equally across all racial groups

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etiology of msa

Unknown etiology

Nicotine and alcohol consumption are less common among patients with MSA as well as Parkinson’s Disease

Majority of cases are sporadic with no known cause

In some reported European and Japanese families, MSA has been transmitted in an autosomal recessive or autosomal dominant inheritance pattern2Study hypothesized that increased expression of the gene ABCA8 played a role in pathogenesis of MSA

8:ABCA8 is a protein encoded by the ABCA8 gene and was found to be highly expressed in oligodendrocyte-rich white matter regions of the brainMeasured expression of ABCA8, alpha synuclein and p25a in MSA brains in disease affected gray matter, disease affected white matter, and an unaffected brain region

Findings suggested direct relationship between the levels of ABCA8 and expression of alpha synuclein and p25a; therefore, increased ABCA8 may precipitate MSA pathology

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Prognosis of MSA

Onset is typically in the 50s with a rapid decline over 5-10 years with loss of motor function

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Some patients reported to live as long as 18 years after diagnosis, with majority of patients with MSA passing away 6-10 years after diagnosis

950% of patients require an assistive device for walking within 3 years of diagnosis, 60% require a wheelchair after 6 years, with patients bedridden at a median time after diagnosis of 6-8 yearsNegative prognostic factors: older age at onset, MSA-P phenotype, and early development of severe autonomic failure

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Treatments for msa

There are currently no medications to slow the progression of MSA or treat the disease, only medications to treat symptoms

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Drugs that are used to treat Parkinson’s Disease, such as Levodopa, may be prescribed; however, in many cases patient’s respond poorly and effectiveness decreases over time

7Physical therapyOccupational therapy

Speech language pathology

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Differential diagnosis: msa

vs. Parkinson’s disease

Multiple System Atrophy

Rapid motor decline after diagnosis including postural instability

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Accumulation of alpha-synuclein in glia2 Levodopa ineffective7

Dementia uncommon, but some cases present with mild loss of cognitive abilityDecreased putaminal volume, glucose metabolism, postsynaptic D2 receptor density10

Autonomically preganglionic disorder11

Parkinson’s Disease

Slower decline in function and motor symptoms

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Accumulation of alpha synuclein in nerve cells (Lewy Bodies)

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Levodopa effective in most cases

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Characteristic pill rolling tremor present

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Can be associated with dementia

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Autonomically postganglionic disorder

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Study: prospective differentiation of multiple system atrophy from

parkinson’s

disease, with and without autonomic failure

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Hypothesis

: The severity, distribution, and pattern of autonomic failure at entry into study will separate MSA from PDFor MSA patients with mild autonomic disorder, the increase in autonomic deficit at one year follow up will differentiate these patients from patients with PD

Used COMPASS and CASS testing to evaluate autonomic severity and deficits and UMSARS to assess functional status Autonomic scores were significantly greater in patients with MSA, as well as functional status scoresChange in autonomic symptoms 12 months later was almost 3 fold greater in patients with MSA than patients with PD, with 2 times greater scores on UMSARS

Anhidrosis in MSA tended to affect entire regions, while in PD tended to be distal (due to autonomic pathology in PD being primarily postganglionic)Conclusion: Functional status and certain autonomic symptoms and deficits will differentiate MSA from PD

Follow up in 12 months will further differentiate PD and MSA due to more rapid progression of MSA

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Parkinson plus disorders12

Multiple System Atrophy considered to be a “Parkinson Plus Syndrome”

Group of heterogeneous neurological disorders

Differ from idiopathic Parkinson’s disease in clinical features, poor response to levodopa, distinctive pathological characteristics, and poor prognosis

Dementia with Lewy Body Disease

Multiple System Atrophy

Progressive Supranuclear Palsy Final diagnosis based on autopsy study

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Rehabilitation strategies for patients with

msa

Multiple System Atrophy Coalition

https://

www.multiplesystematrophy.org

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Physical therapy4

Stage 1: Preserved Mobility

Management of autonomic symptoms

Encourage active lifestyle and exercise:

pilates

, tai chi, walking the dogExercise should focus on high level balance, strength, aerobic trainingIdentify falls riskAvoid compensatory motor patterns at this stage

MSA-C: focus on posture, balance, and gait while considering role of vision

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Physical therapy

Stage II: Impaired Mobility

Encourage maintenance of activity

May include compensations and/or assistive devices

Remove causes of falls including creating a safer environment

Stretching and assess for contractures Gait training: focus on taking large steps and heel strike, safe turns while avoiding pivoting and crossing one foot over the other Orthotics Seated exercises

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Physical therapy

Stage III: Severely Compromised Mobility

Wheelchair assessment

Skills for transfer and bed mobility

Positioning

Caregiver support and educationBreathing techniques, assisted cough if appropriate

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Physical therapy

LSVT Big Program

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Increased amplitude movements in limb and body centered around patients with PD

Documented improvements in amplitude and speed of movement, balance, and quality of life

16 sessions over a 1 month period- 4 hour long sessions per weekMay help patients with Multiple System Atrophy in the short term

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Occupational therapy14

Teach compensatory strategies as needed:

Adaptive devices

Arm positioning when eating

Educate on placing less blankets or using satin sheets in the bed to allow for easier bed mobility

Dress affected limb firstSit when dressing, bathing, self-care Supporting with one hand on sturdy surface while reaching

Side step when working at the counter-space or sink

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Outcome measure: unified multiple system atrophy rating scale

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UMSARS

Assesses 4 domains: impairment and functional tasks, motor ability, autonomic function, global disability

Good psychometric properties including: internal consistency, interrater reliability, and criterion validity Scores range from 0-104 with higher score= greater impairmenthttps://

www.movementdisorders.org/MDS-Files1/PDFs/MDS-UPDRS-Rating-Scales/scale_UMSARS.pdf

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Patient case: Mr. S

64 years old

Diagnosed in 2013 at 59 years old

Worked as a nuclear physicist for the CIA for 23 years

Retired early due to progression of disease- having difficulty driving, walking, typing

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Patient Case: Mr. S

Signs and symptoms began prior to diagnosis:

Difficulty swimming (collegiate swimmer, swam regularly for exercise)

Swayed to the right when walking

Grabbed onto sturdy objects for stability- poor balance

Erectile dysfunctionBladder control problems at night

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Patient Case: Mr. S

Evaluated by neurologists, urologists, movement disorder specialists

Sought numerous opinions from specialists at John Hopkins, Georgetown, NIH

Multiple specialists suspected a Parkinson Plus syndrome, came to eventual diagnosis of MSA

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Patient Case: Mr. S

Current:

No longer to negotiate stairs- moved to a single-story home

Primary form of mobility is a manual wheelchair with others pushing him

Gait is very slow with poor balance,

uncoordinationRequires assistance eating, bathing, shaving, dressing, using the bathroomNo longer able to self-cath

- uses a supra-pubic catheter Suffers from orthostatic hypotension- dizziness, fainting, falling

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Patient Case- Mr. S

Caregiver comes daily for 4 hours

Lives with wife, 3 sons live close by and come over daily to check on him

Receives home health PT, OT, SLP twice weekly- focus on functional mobility and preserving strength

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Patient case- mr.

s

Challenges:

Mr. S is very angry about his

circumstances,“his

world has become very small”Personality has changed- has become self-absorbed and doesn’t always recognize what his family members do for him on a daily basis Hobbies: watches movies, playing chess, listens to podcasts about physics

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References

History of Multiple System Atrophy (MSA), A Neurodegenerative Disorder. Multiple System Atrophy: The MSA Coalition. https://

www.multiplesystematrophy.org

/about-

msa

/history-of-msa. Accessed April 19, 2018.

Longo DL, Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl

J Med. 2015;372(3):249-263. http://libproxy.lib.unc.edu/login?url=https://search-proquest-

com.libproxy.lib.unc.edu

/

docview

/1645924181?accountid=14244.

Multiple System Atrophy Fact Sheet. National Institute of Neurological Disorders and Stroke. https://

www.ninds.nih.gov

/Disorders/Patient-Caregiver-Education/Fact-Sheets/Multiple-System-Atrophy. Modified January 23, 2018. Accessed April 19, 2018.

Lord S,

Mhiripiri

D. A Guide to Multiple System Atrophy for: Physiotherapists. Multiple System Atrophy Trust. https://

www.msatrust.org.uk

/

wp

-content/uploads/2015/11/FS830-Guide-for-Physiotherapists_v1.2.pdf. Published November 2014. Accessed April 19, 2018.

Ciolli

L,

Krismer

F, Nicoletti F,

Wenning

GK. An update on the cerebellar subtype of multiple system atrophy. Cerebellum & Ataxias. https://

cerebellumandataxias.biomedcentral.com

/articles/10.1186/s40673-014-0014-7. Published October 10, 2014. Accessed April 19, 2018.

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References

Muqit

MM, Mort D,

Miszkiel

KA, Shakir RA. "Hot cross bun" sign in a patient with parkinsonism secondary to presumed vasculitis. Journal of Neurology, Neurosurgery & Psychiatry. http://

jnnp.bmj.com/content/71/4/565. Published October 1, 2001. Accessed April 19, 2018.

Multiple System Atrophy. NORD (National Organization for Rare Disorders). https://rarediseases.org/rare-diseases/multiple-system-atrophy/. Accessed April 19, 2018.

Bleasel JM, Hsiao JH, Halliday GM, Kim WS. Increased expression of ABCA8 in multiple system atrophy brain is associated with changes in pathogenic proteins. Journal of Parkinson's disease. https://www.ncbi.nlm.nih.gov/pubmed

/23948991. Published January 1, 2013. Accessed April 19, 2018.

Differential Diagnosis for Multiple System Atrophy, a Disorder Similar to Parkinson's Disease. Multiple System Atrophy: The MSA Coalition. https://

www.multiplesystematrophy.org

/about-

msa

/differential-diagnosis. Accessed April 19, 2018.

Ghaemi

M,

Hilker

R, Rudolf J,

Subesky

J,

Heiss

WD. Differentiating multiple system atrophy from Parkinson's disease: contribution of striatal and midbrain MRI

volumetry

and multi-tracer PET imaging. Journal of Neurology, Neurosurgery & Psychiatry. http://

jnnp.bmj.com

/content/73/5/517?utm_source=

TrendMD&utm_medium

=

cpc&utm_campaign

=J_Neurol_Neurosurg_Psychiatry_TrendMD_1. Published November 1, 2002. Accessed April 19, 2018.

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References

Lipp

A, Sandroni P,

Ahlskog

JE, et al. Prospective Differentiation of Multiple System Atrophy from Parkinson's Disease, with and without Autonomic Failure. Archives of neurology. https://

www.ncbi.nlm.nih.gov/pmc/articles/PMC2838493/. Published June 2009. Accessed April 19, 2018.

Mitra K. Parkinson plus syndrome- a review. Neurology India. 2003;51(2):183-188. doi:10.1075/ps.5.3.02chi.audio.2f.

What is LSVT BIG? LSVT Global - What is LSVT BIG? https://www.lsvtglobal.com/patient-resources/what-is-lsvt-big. Accessed April 19, 2018.

A Guide to Multiple System Atrophy for: Occupational Therapists. Multiple System Atrophy Trust. https://

www.msatrust.org.uk

/

wp

-content/uploads/2015/11/FS810-Guide-to-MSA-for-Occupational-Therapists.pdf. Published February 2016. Accessed April 19, 2018.

Wenning

G, et al. Development and Validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Movement

Disorderes

. 2004;19:1391-1402. https://

www.movementdisorders.org

/MDS-Files1/PDFs/Rating-Scales/

umsars.pdf

.

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Questions?