Multiple System Atrophy: A Case Study


ABSTRACT[edit | edit source]

This fictional case study involves an older adult male who is diagnosed with Multiple System Atrophy-Parkinsonism (MSA-P), which is classified as probable. The purpose of this case study is to outline what the neurological disease MSA-P is, including its natural disease course, progressive nature, clinical presentation, as well as the difficulty of distinguishing it from its differential diagnoses (i.e. Parkinson’s Disease). Additionally, the intention was to highlight the impact that the disease had, not only on Mr. L’s physical state, but the impact on his personal relationships, and his mental health. Mr. L first presented 3 years prior with erectile dysfunction and progressed to additional symptoms of balance and coordination difficulties, slurring of speech, postural tremor, gait ataxia, and action myoclonus, among others. He was referred to physiotherapy by his physician. This case study follows Mr. L through his rehabilitation journey, from initial assessment to current therapeutic interventions. These interventions include patient education, resistance training, range of motion exercises, balance and gait functional training, pelvic floor training, and dance therapy. Physiotherapy interventions were determined in collaboration with Mr. L to focus on achieving short-term and long-term goals based on his chief concerns and treatment expectations. Interventions focused on education, resistance training, range of motion, balance/gait training, pelvic floor physiotherapy referral, group rehabilitation dance classes and referral to occupational therapist/speech language pathologist. Upon reassessment 12 weeks into physiotherapy rehabilitation, Mr. L was discharged with significant improvements in areas of prior functional limitation. Mr. L’s discharge was determined  based on improved mobility, functional capabilities and overall quality of life. Upon discharge, Mr. L was still encouraged to seek physiotherapy care as needed regarding any concerns related fo functional/mobility goals or significant changes in disease state. Mr. L was also encouraged to keep in contact with his family physician, occupational therapist and speech language pathologist as needed.

INTRODUCTION[edit | edit source]

Multiple Systems Atrophy (MSA) is a very rare, progressive and degenerative neurological disorder that specifically affects autonomic functions (I.e., blood pressure, bladder function, etc.) of the nervous system.[1] The onset of MSA occurs in older adults in their 50s and 60s and can often be mistaken for Parkinson’s Disease because of cerebellar signs such as cerebellar ataxia.[2] Additionally, since Parkinson’s Disease also affects the nervous system like MSA, the two can often be mistaken for each other. Although there is no cure for MSA, there are various evidence-based treatments that can help maintain quality of life while reducing the impact of the severe symptoms.[3]

There are two main variations of Multiple Systems Atrophy, of which include Parkinsonian and Cerebellar (MSA-P and MSA-C, respectively).[4] Although the two variations are quite similar in nature, the main difference lies in the symptoms that the individual presents with at the time of diagnosis. MSA-P is more common than MSA-C and can be identified by the following symptoms: rigid muscles, difficulty bending limbs, bradykinesia, tremors, and problems with posture and balance.[4] In patients with MSA-C, movement and coordination is impaired, speech is slow and slurred, visual disturbances are noted, and some individuals have difficulty swallowing or chewing.[4] Despite their differences, MSA-P and MSA-C have shared symptoms such as urinary dysfunction, uncontrolled or absent sweating, sleeping disorders, erectile dysfunction, heart problems such as orthostatic hypotension, and some individuals even experience psychiatric disorders.[4] The best way to diagnose MSA is by completing an autopsy on the central nervous system to analyze any neurodegenerative changes in striatonigral or olivopontocerebellar structures.[5] However, since that can only be completed postmortem, the next best way to diagnose MSA is by investigating the sporadic, progressive adult-onset disorder symptoms including rigorously defined autonomic failure and poorly levodopa-responsive Parkinsonism or cerebellar ataxia.[5] The clinical diagnosis of probable MSA requires a reduction of systolic blood pressure by at least 30 mm Hg or of diastolic blood pressure by at least 15 mm Hg after 3 minutes of standing from a previous 3-minute interval in the recumbent position.[5] Lastly, it should be noted that an individual’s MSA-P can change to MSA-C, and vice versa, over the course of the disease, but it is still named based on the symptoms the individual presents with at the time of diagnosis.

Despite a relatively poor prognosis as the disease progresses, there are various evidence-based approaches to help treat the patient. Most treatments are medically based and involve medications to raise blood pressure, reduce Parkinson-like signs and symptoms, and reducing sexual dysfunction symptoms.[2] However, physiotherapists have a major role in treating individuals with MSA since individuals often undergo motor control and muscle degeneration. First off, pelvic floor physiotherapists can assist with urinary retention by prescribing pelvic floor exercises (I.e., Kegels) to strengthen the musculature in the area.[6] Many individuals with MSA that experience urinary retention deficits are often embarrassed to go out in public for this reason, so it is important to target this problem to improve their overall self-esteem and therefore their quality of life. As already mentioned, individuals with MSA undergo motor and muscle degeneration as the disease progresses. Physiotherapists can help maintain muscle range of motion and tone by using passive range of motion in combination with an exercise program that includes resistance training and/or gait/balance training.[7][8] Furthermore, it is crucial that physiotherapists help educate the patient, in conjunction with the interdisciplinary team, on how to optimize functional independence and improve quality of life.[9]

Within this specific case study on Multiple Systems Atrophy, there is a strong focus on the symptoms that Mr. L is presenting with and how they affect his overall quality of life, as well as a strong focus on the evidence-based treatment plan. The objective of this case study is to document Mr. L’s journey with this disease and to maximize his functional independence, all through the eyes of a physiotherapist.

CLIENT CHARACTERISTICS[edit | edit source]

Mr. L is a 62-year-old retired NASA astronaut. He is currently living in California with his husband of 20 years, who is also retired. Mr. L first presented to his family physician three years ago with issues of erectile dysfunction, in which his doctor believed to be result of aging. Over the next year, Mr. L began developing other symptoms seemingly unrelated to his previous health history. He presented with noted difficulty balancing, asymmetric postural tremor (right side), bradykinesia, rigidity, urinary incontinence, and minor slurring of speech – warranting a probable diagnosis of Parkinson’s disease. Mr. L was prescribed oral Levodopa (250 milligrams, four times a day). This medication eased his symptoms for four months, but gradually, he began noticing the return of his symptoms – warranting a reassessment. Despite increasing his medication dosage, Mr. L’s chief complaints were perpetuated. Additionally, he began experiencing gait ataxia with cerebellar dysarthria, action myoclonus, and cold hands and feet. After conducting a thorough physical assessment, it was noted that Mr. L presents with orthostatic hypertension (dropping from 120/94 mmHg in a recumbent position, to 90/68 mmHg, after prolonged standing). Based on the presenting factors, a diagnosis of Multiple System Atrophy-Parkinsonism (MSA-P) was established. The physician referred Mr. L to physiotherapy for gait and mobility training, pelvic floor training, and education on symptom management.

EXAMINATION FINDINGS[edit | edit source]

Subjective[edit | edit source]

Patient Profile[edit | edit source]
  • DOB: November 28, 1958 (62 y/o)
  • Gender: Male
  • Significant Presentation: Probable MSA progressing over 3 years
History of Present Illness[edit | edit source]

Diagnosis of probable MSA-P three weeks ago, right-sided postural tremor, difficulty balancing, bradykinesia, rigidity, urinary incontinence, slurred speech, cerebellar dysarthria, cold hands and feet, action myoclonus, and orthostatic hypotension.

Past Medical History[edit | edit source]

N/A

Previously healthy

Medications[edit | edit source]
  • Levodopa (250 mg 4X/day, for 4 months)
  • Levodopa (500 mg 4X/day, for 2 months)
  • CBD for pain management (occasional)
Health Habits[edit | edit source]

Non-smoker, non-drinker, formerly very active (prior to symptom onset)

Family History[edit | edit source]

No family history of progressive neurological disorders

Psychosocial[edit | edit source]

Patient is experiencing marital issues due to the progressive nature of the disease. Patient also expresses concerns of loneliness, isolation, and depression due to fatality of the diagnosis. Mr. L also worries about burdening his partner and family as the disease progresses and he becomes less independent. Having been previously very active, Mr. L is having trouble coping with his new sedentary lifestyle. Previously, Mr. L volunteered weekly at his local YMCA, running a space exploration program for young children – bringing joy to his life. Due to cerebellar dysarthria, he is having trouble effectively communicating with others. Lastly, he used to really enjoy dancing with his husband but has not been able to do so recently because of his condition.

Living Environment[edit | edit source]

Two-story home with 14 stairs to the second floor. Bedroom located on second floor. Half bathroom on first floor, full bathroom on second floor. Second floor bathroom equipped with a spacious walk-in shower. Hardwood flooring throughout the home.

Functional History[edit | edit source]

Prior to probable MSA-P diagnosis: very active, running 15km a week and walking everywhere within the city. Drove when needed. Able to independently maintain his property and home chores. Able to complete all activities of daily living independently.

Current Functional Status[edit | edit source]

After probable MSA-P diagnosis: able to ambulate 200m independently, but very slowly and unsteadily. Beyond 200m, requires a rollator walker or assist X1. Requires assistance from his husband for ADLs (cooking, cleaning, bathing, shopping, etc.). Trouble controlling urination and bowel movements (requires protective underwear). Able to ascend and descend one flight of stairs with assist X1. Unable to drive. Fine motor activity is limited due to action myoclonus (unable to type). Trouble communicating due to dysarthria.

Imaging[edit | edit source]

MRI: T2 weighted MRI revealed a hyperintense putaminal slit sign bilaterally

Precautions/Contraindications[edit | edit source]

Depression, lack of social interaction (decreased involvement with community), and trouble communicating due to dysarthria (no difficulties with comprehension). Otherwise, no contraindications to a physiotherapy program.

Objective[edit | edit source]

General[edit | edit source]

Minor hypomimia, postural AP tremor, action tremor

Posture[edit | edit source]
Standing Rounded shoulders, right shoulder depression, forward head posture, forward lean, wide base stance
Seated Rounded shoulders, right shoulder depression, posterior pelvic tilt
Gait[edit | edit source]

Festination, shuffling (short stride) wide base gait, bradykinetic ambulation, reduced arm swing bilaterally, action myoclonus more prominent during ambulation

Tone[edit | edit source]

High tone on right side of body in comparison to the left

Active Range of Motion (AROM)[edit | edit source]

Upper Extremity

Left Right
Shoulder flexion 80° 65°
Shoulder extension 40° 30°
Shoulder abduction 150° 110°
Shoulder adduction 75° 70°
Elbow flexion 130° 120°
Elbow extension -5°
Wrist flexion 90° 90°
Wrist extension -10° -20°

Lower Extremity

Left Right
Hip flexion 115° 109°
Hip extension -4° -4°
Hip abduction 40° 38°
Hip adduction 45° 42°
Knee flexion 120° 115°
Knee extension -5° -10°
Ankle dorsiflexion 50° 50°
Ankle plantarflexion
Ankle inversion 34° 35°
Ankle eversion 15°

Trunk

C-Spine T-Spine L-Spine
Flexion 50° 30° 50°
Extension 10° 10° 15°
Side flexion L/R 30/25° 30/25° 25/15°
Rotation L/R 70/65° 30/25° 0/0°
Passive Range of Motion (PROM)[edit | edit source]

PROM limited by muscle rigidity and contracture

Strength[edit | edit source]
Respiratory MIP: 39.08% of normal values

MEP: 49.59% of normal values

Musculoskeletal Manual muscle testing revealed global muscle weakness (4/5)
Neurological Testing[edit | edit source]
Upper Motor Neuron Testing
Lower Motor Neuron Testing Normal
Myotomes Abnormal
Dermatomes Sensation WNL
Self Reported Outcome Measures[edit | edit source]
Activities-Specific Balance Confidence Scale (ABC)[11]
  • Assesses balance (vestibular and non-vestibular, functional mobility)
  • Score = 50% (between low and moderate level of physical functioning
Short Form – 36 (SF36)[12][13]
  • General assessment of overall health function and psychosocial quality of life
  • Score = 12/36
Outcome Measures[edit | edit source]
Unified Multiple System Atrophy Rating Scale (UMSARS)[14]
  • Part 1 -- Historical Review
    • Score = 26
  • Part 2 -- Motor Examination Scale
    • Score = 30
  • Part 3 -- Autonomic Examination
    • 120/94 mmHg (supine) to 90/68 mmHg (standing)
    • Heart rate 98 bpm (supine), 104 bpm (standing)
    • Orthostatic symptoms present
  • Part 4 -- Global Disability
    • Score = 4
  • Overall Score = 60/104
Timed Up and Go (TUG) Test[15]
  • Assesses balance, mobility, ability to walk, and fall risk in older adults
  • Score = 19 seconds
Performance-Oriented Mobility Assessment (POMA)[16]
  • Assesses balance, both static and dynamic
  • Score = 22/28
Functional Reach Test (FRT)[17]
  • Assesses dynamic balance
  • Score = 6" bilaterally
Single Leg Stance (SLS)[18]
  • Assesses static balance on a small base of support
  • Patient unable to stand on one leg

DIAGNOSIS[edit | edit source]

Patient is a 62-year-old male presenting with initial symptoms of bradykinesia, urinary incontinence, difficulty balancing, and increasing issues with ambulation [UMSARS (60/104), TUG (19 seconds), POMA (22/28), SLS (inability), ABC (50%)]. Due to these outcome measure scores and initial presentation the patient is also at an increased risk of falls. The patient was diagnosed with Multiple Systems Atrophy - parkinsonism subtype (MSA-P) and referred to physiotherapy address these issues. The patient expressed concerns regarding their decreasing independence with ADL’s and decreased mental health quality of life due to previously active lifestyle. Although this diagnosis is progressive and will lead to fatality Physiotherapy will be helpful for the patient to improve confidence and functional independence through an exercise program with high focus on balance, mobility, and light resistance training. The patient will also benefit from education surrounding symptom and overall management.

PROBLEM LIST[edit | edit source]

  1. Decreased ambulatory agility and increased fall risk (TUG = 19 seconds)
  2. Moderate fall risk as per his 22/28 POMA score  
  3. Decreased mental health and feeling of independence due to the progression of the disease
  4. Decreased Quality of life 12/36 on the SF-36 questionnaire
  5. Increased right-sided tone and rigidity during PROM  
  6. Bradykinetic ambulation with festination’s during gait and ADLs  
  7. Decreased right sided AROM compared to right (UE’s and LE’s)  
  8. Decreased confidence during gait and ADL’s due to fear of falling (ABC 50%)
  9. Right UE and LE weakness during resisted movements (RIM = 4/5 globally)  
  10. Orthostatic hypotension (130/82mmHg to 98/76mmHg)
  11. Moderate-severe impairment and functioning within various domains (motor, autonomic, and ADL’s) seen during UMSARS Assessment
  12. Urinary incontinence due to autonomic decline associated with MSA progression
  13. Lack of knowledge regarding support/progression of condition
  14. Minor slurring of speech

PATIENT GOALS[edit | edit source]

Short-Term Goals (STG)[edit | edit source]

  • Body Structure/Function
    • Increase shoulder flexion on both left and right sides from 80 and 65 degrees, respectively, to 120 degrees within 3 weeks.
    • Improve right shoulder abduction from 110 degrees to 150 degrees within 3 weeks.
  • Activity
    • Increase Activities-Specific Balance Confidence Scale from 50% to 65% (MDC = 13%) within 4 weeks
    • To be able to stand on one limb within 5 weeks so he can pass the single-leg stance test
  • Participation
    • Gain strength and aerobic capacity through countertop exercises so he will be able to help his husband cook at home within 3 weeks
    • Ensure that the patient has adequate supports and education for both himself and his family within one week of the initial visit to assist with future needs

Long-Term Goals (LTG)[edit | edit source]

  • Body Structure/Function
    • Increase pelvic floor strength through exercise to minimize the risk of urinary incontinence within 8 weeks
    • Improve both upper extremity and lower extremity strength through resisted exercise interventions within 10 weeks
  • Activity
    • Ambulate 500 meters with the use of a quad cane and no extra assistance before having to stop due to fatigue in 8 weeks
    • Ascend one flight of stairs with no additional supports within 8 weeks
  • Participation
    • Encourage Mr. L to go on 3 short walks around the block per week with husband, using rollator to improve daily physical activity  
    • Improve his SF-36 score from 12/36 to 24/36 within 10 weeks which would demonstrate an improvement in overall quality of life

INTERVENTION[edit | edit source]

Treatment Intervention Frequency   Intensity   Time   Rationale
Education:  
  • Role of Physiotherapist and disciplinary team  
  • Importance of daily physical therapy to functional independence and mental health  
  • Environmental modifications in consult with Occupational Therapist  
During appointment 1-2 when deemed appropriate N/A Ongoing throughout intervention Problem List (3, 8, 12)

If the patient understands the role of physiotherapy and other members of the interprofessional team there will be better adherence to interventions. For example, during the first appointment, the Occupational Therapist will assess the environment that the patient encounters daily and determine how they can help Mr. L adapt and ensure functionality in tandem with the Physiotherapist. Providing clear explanations and education about the importance of physical activity and functional independence can ensure the patient and the rest of the circle of care is on the same page for the treatment going forward.[19] This will lead to improved mental health and quality of life for the patient.[20]

Resistance Program:
  • Calf Raises
  • Repeated sit to stand
  • Repeated Knee extension, hip abduction, hip adduction, and knee flexion
  • Kitchen Sink exercises that include reaching in shoulder flexion and abduction
3x/week All exercises can be done with gravity as the resistance but can progress to weights as tolerated. 2 sets of 8 repetitions each Problem List (1, 2, 5, 7, 9, 11)

Since the patient has relatively weaker muscles, which has been causing him many issues such as decreased ambulation and increased fall risks as well as limitations in his activities of daily life, it is important to work on strengthening exercises to try to gain muscle strength. Resistance training has been shown to greatly improve muscle strength, gait, and postures in patients with MSA-P[21]. In terms of the specific exercises that were selected for this intervention, it was important to focus on functional movements that could assist the patient with his ADLs, as well as addressing the lack of range of motion in the shoulder and hip joints. Should this exercise program for the patient become too easy, it can always be progressed by adding more sets and/or repetitions or increasing the resistance.

Range of Motion:  
  • AAROM (shoulder range specifically)
  • PROM
2x/week during appointments Moving into end range/ slight discomfort  
  • AAROM = 2 sets of 8
  • PROM = 2 sets of 8
Problem List (5, 7)

Although range of motion, of which is limited in the patient, can often be addressed through muscle strengthening and functional tasks such as walking, it is still important to address range of motion deficits directly[22]. With muscle rigidity and limited range of motion being predominant characteristics of this patient, similar to most patients with MSA-P, active-assisted range of motion, as well as passive range of motion from a physiotherapist, should be included in the intervention. Passive range of motion can be completed as needed to ensure that there are limited contractures and rigidity while active-assisted range of motion can be done at home to maintain the newly achieved range of motion.

Balance/Gait Specific Exercises:  
  • Kitchen Sink exercises (Sustained marching, SLS)
  • Tandem walking (10m)  
  • Supervised balance board quiet standing  
  • Quiet standing with feet together and eyes closed  
  • Gait training with quad cane (20m)
  • aerobic treadmill training  
3x/week   Light intensity during gait exercises Balance
  • 2 sets of 20 (sustained marching)
  • 2 sets of 10 (SLS)
  • 5 sets of 10 seconds (board standing and standing eyes closed)
  • 5 sets 10m (Tandem walking)


Gait  

  • 5 sets of 20m (quad cane walking)
  • 10 minutes treadmill training at comfortable speed where patient could still have a conversation with therapist
Problem List (1, 2, 3, 4, 6, 8, 11)

Balance exercises (specifically those with a focus on vestibular and visual training) have been shown to increase balance in patients with Parkinson’s disease and therefore patients like Mr. L who have a large component of parkinsonism accompanying his recent diagnosis of MSA-P[23]. There is also evidence that balance exercises combined with resistance training in the lower extremities will increase this benefit further[23]. Gait and balance exercises can decrease the risks of falls by improving TUG, POMA, and SLS while also aid in increasing Mr. L’s confidence while ambulating in the community[24]. Gait training and aerobic exercise will encourage Mr. L to continue his previously active lifestyle.

Pelvic Floor Physio:
  • Kegel Exercises
5x/week Moderate intensity Hold for 3-5 seconds

2 sets of 10 reps

Problem List (3, 4, 12)

Since the patient is experiencing urinary incontinence which ultimately decreases his quality of life, Kegel exercises (pelvic floor exercises) have been incorporated into his intervention and home exercise program. This will ensure that the patient has better self-control with his urinary retention[25]. This should implore Mr. L to resume some of his activities, such as dancing.

Group Rehabilitation Dance Classes:   Once per week Light-moderate intensity 30 minutes to start (can progress) Problem List (1, 4, 3, 8)

Group dance targeted for individuals with neuromuscular diseases can help improve quality of life and sense of community involvement. Classes have also been shown to increase balance, gait, and confidence with movement[26]. Mr. L will especially benefit from this program because he can take what he learns and continue to dance with his husband.

INTERPROFESSIONAL INTERVENTION[edit | edit source]

Treatment Intervention Frequency Intensity Time Rationale
Occupational Therapist (OT)
  • Educate the patient and family members about the importance of OT and how they can help day-to-day living of the individual
  • Continued education as the disease progresses and how to accommodate as motor function continues to regress
  • The OT can provide adaptations for the patient and family as he begins to have difficulty with dressing, eating, and other daily activities
  • At home-visit to provide home alterations to improve ease of day-to-day life
  • During the at home visit, the OT can identify fall risks and give recommendations to ensure patient safety
Once per week N/A
  • 60-minute appointments  
  • 60-minute at home visit every 6 months as disease progresses
Problem List (1, 2, 3, 4, 11, 13)

The OT will implement creative and innovative strategies to aid the patient with day-to-day activities and can help him identify fall risks at his home. As the disease progresses, the patient will begin to have issues with fine motor skills such as writing, dressing, cooking, etc. Also, the OT can address problems with concentration and memory.[27] One strategy the OT will use is Wii-Hab. Research has shown that playing simulated games, such as golfing or bowling, can help improve timing of movements in coordination with sensory stimuli. [28]

Speech-Language Pathologist (SLP)
  • Educate the patient about importance of SLP and how they can help them with their disordered speech and swallowing
  • Provide a variety of oral motor exercises, breathing strategies, and vocal exercises to improve ease of speech
  • Alterations to speech could include speaking with a softer tone, changes in pitch, or giving strategies to help with difficult pronunciations
  • Refer to a communication support group and connect the patient with other individuals suffering from neurodegenerative diseases
  • Once a week for 4 weeks
  • Once a month as disease progresses
N/A
  • 40-minute appointments
  • 60-minute sessions with communication support group
Problem List (13, 14)

Complications with muscles that control speech is a common characteristic of MSA patients including our patient who is dealing with slurred speech, which will progressively decline over time. Complications include changes in voices patterns, hoarseness, monotone, and a quiet mumbling speech pattern. [4] The SLP will use Lee Silverman’s Voice Treatment (LSVT) as an intervention. LSVT has been proven to be effective in enhancing swallowing function and improving dysarthria. [29]

OUTCOMES - Reassessment After 12 Weeks[edit | edit source]

Measures Initial Assessment Reassessment after 6 weeks Reassessment after 12 weeks
AROM


Shoulder flexion

Shoulder extension

Shoulder abduction

Shoulder adduction

Elbow flexion

Elbow extension

Wrist flexion

Wrist extension

Left

80o

40o

150o

75o

130o

0o

90o

-10o

Right

65o

30o

110o

70o

120o

-5o

90o

-20o


Shoulder flexion

Shoulder extension

Shoulder abduction

Shoulder adduction

Elbow flexion

Elbow extension

Wrist flexion

Wrist extension

Left

105o

40o

155o

75o

130o

0o

90o

-5o

Right

90o

32o

140o

72o

123o

-3o

90o

-10o


Shoulder flexion

Shoulder extension

Shoulder abduction

Shoulder adduction

Elbow flexion

Elbow extension

Wrist flexion

Wrist extension

Left

115o

40o

160o

75o

130o

0o

90o

5o

Right

105o

33o

155o

72o

125o

-3o

90o

0o

MSK Strength Global muscle strength – 4/5 Global muscle strength – 4/5 Global muscle strength – 4+/5
ABC Score = 50% Score = 65% Score = 70%
SF36 Score = 12/36 Score = 19/36 Score = 25/36
UMSARS Overall Score = 60/104 Overall Score = 66/104 Overall Score = 70/104
TUG Score = 19 Score = 16 Score = 14
POMA Score = 22/28 Score = 24/28 Score = 25/28
SLS Unable to stand on one limb Able to stand on one limb Able to stand one limb

*Note: Only outcomes related to our STG/LTG were included in the outcome portion

Psychosocial Affect[edit | edit source]

Mr. L’s concerns regarding psychosocial aspects of his life have improved as measured by the SF36. His score at initial assessment improved from 12/36 to 25/36 during reassessment at 12 weeks. Mr. L reports feeling less concerns of loneliness, isolation, depression, and is hopeful for the progress he has been able to make over the past 12 weeks. He reports that the incorporation of group rehabilitation dance classes in his treatment plan is a primary factor regarding his improvement in his SF36 score. Additionally, the education portion of Mr. L’s treatments sessions have played a significant role in his SF36 improvement. Mr. L reports that his confidence in terms of his balance has also Improved as a result of the balance-oriented treatment goals which was showed in his ABC score increase from 50% to 70% at 12 weeks.

Range of Motion/ Strength[edit | edit source]

Mr. L’s AROM has improved significantly since his first visit, as noted in the table above, in shoulder flexion and abduction. His short-term goals were focused on improving right shoulder flexion and right shoulder abduction. Right shoulder flexion and abduction improved from 65 deg to 105 deg and 110 deg to 155 deg, respectively. Mr. L reports that treatment sessions using AAROM and PROM have been very helpful in improving his ROM and decreasing tone in his R extremities. Also, he has learned the importance of ROM maintenance and preventing joint contractures. Mr. L also reports that overall, he feels a bit stronger than how he originally felt on initial assessment, showed by an improvement in his global manual muscle testing improving from 4/5 to 4+/5. He reports that resistance training three times per week has helped him gain strength overall.

Mobility[edit | edit source]

During initial assessment, Mr. L’s functional status was independent with no gait aid, able to ambulate approximately 200m before fatiguing and requiring use of a rollator walker or assist X1. After 12 weeks, Mr. L had an improvement in his TUG from a score of 19 at initial assessment to 14 during reassessment. Despite his improvement, Mr. L is still at an increased risk of fall according to standardized TUG scores.[15] By the end of 12 weeks, Mr. L was able to ambulate 400m using a quad cane before feeling too unsafe to continue or needing assistance. This was achieved by gait re-training using the quad cane in physiotherapy treatment. Mr. L was also able to ascend one flight of stairs (14 steps) with the use of railing without feeling unsafe as shown in the increase in his ABC score.

Balance[edit | edit source]

Mr. L states that he feels much more confident with his balance overall, this is shown by the improvement in his ABC. Increases from his POMA scores after 12 weeks also show his improvement in his balance, his POMA score improved from 22/28 to 25/28. At initial assessment, Mr. L was unable to perform single leg stance (SLS), but after 6 weeks of physiotherapy interventions, he was able to perform it without any issues bilaterally. However, Mr. L still has some concerns regarding his balance that will continued to be monitored.

Neurogenic Bladder[edit | edit source]

Mr. L reports that he has had less issues in regards to urinary incontinence. As previously stated, pelvic floor physiotherapy can reduce occurrences of such issues. Pelvic floor physiotherapists can help with bladder retraining and strengthening of the pelvic floor muscles to help reduce secondary comorbidities such as kidney damage, infections and an overall decreased quality of life. Kegel exercises as prescribed by a trained pelvic floor physiotherapist have helped Mr. L prevent urinary problems from progressing.

Unified Multiple System Atrophy Rating Scale (UMSARS)[edit | edit source]

MSA-P is a progressive neurodegenerative, however, physiotherapy can help with disease management and decrease the rate of decline. On initial assessment, Mr. L scored an overall score of 60/104 on the UMSARS and improved to an overall score of 70/104.

DISCHARGE PLAN[edit | edit source]

Upon reassessment at 12 weeks, it is clear that Mr. L has improved drastically since his initial visit. He reports that he is optimistic about his functional status at 12 weeks and is looking forward to continuing to work on the goals he has set for himself along with physiotherapy. Mr. L reports that he is now able to walk once or twice around the block for daytime strolls using a quad cane along with his husband without any issues of fatigue. However, Mr. L still suffers from symptoms common to MSA-P such as: slight right postural tremor, bradykinesia, and slight slurring of speech. Despite the progressive nature of those symptoms, he has reported that his self-perceived quality of life has significantly increased, as supported by his increase in the SF36. However, there are still some concerns for Mr. L’s safety as he still suffers from slight gait ataxia and balance impairments due to the nature of the disease.

In addition to Mr. L’s physiotherapy related outcomes, time spent with OT and SLP have also contributed to his increase in functional independence. Both in-clinic OT and home OT interventions over the past 12 weeks have greatly improved Mr. L’s ability to adapt to his changing symptoms, given the progressive nature of his disease. During OT assessment and intervention, Mr. L saw great improvements in his fine motor skills as well as coordination of movements with sensory stimuli. He reports that this has helped him significantly with his ADLs and IADLs throughout the past 12 weeks. Mr. L also reports that his time working with the SLP through LSVT interventions have significantly helped with his dysarthria. He states that he is hopeful that he may potentially be able to return to volunteering at his local YMCA in a lesser capacity. The patient states that the increase in his SF36 score is related to his speech progress to an extent.

Due to his improvements over the last 12 weeks, Mr. L will be discharged from bi-weekly physiotherapy sessions but is encouraged to see physiotherapy as needed regarding any issues related to functional/mobility goals or significant changes in disease state. In addition, Mr. L should continue to keep in contact with his family physician or neurologist, OT and SLP to provide him with different interventions/alternatives as the disease progresses.

DISCUSSION[edit | edit source]

MSA is a fatal progressive neurodegenerative disease that occurs sporadically in adults.[30] A study by Watanabe et al. determined that the average time from onset of symptoms to aid-requiring walking, wheelchair dependent, bedridden, and death were 3, 5, 8, and 9 years respectively.[31] Research has determined that majority of MSA patients pass due to secondary conditions of the disease such as pneumonia, pulmonary embolism, aspiration, etc.[32] The goal of physical therapy treatment is to help maintain muscle strength, flexibility, prevent falls, etc., in order to maintain/improve quality of life and functional independence. 3

This case study examines a patient named Mr. L who is a 62-year-old male retired NASA astronaut, who presented with an insidious onset of MSA-P. The patient presented to his family physician with a variety of signs and symptoms such as difficulty balancing, asymmetric postural tremor, bradykinesia, rigidity, erectile dysfunction, urinary incontinence, and minor slurring of speech. The doctor prescribed Levadopa, which initially provided relief of symptoms; however, after four months the patient’s symptoms began to reappear. Mr. L patient began to develop worsening signs and symptoms such as gait ataxia, cerebellar dysarthria, action myoclonus, cold hands/feet, and orthostatic hypotension. Subsequently, the doctor diagnosed the patient with MSA-P and referred him to physiotherapy to aid with disease management and decrease the rate of physical decline due to the progressive nature of this disease.  

After a thorough subjective and objective assessment, the physiotherapist and patient collaborated to develop patient-focused short-term and long-term goals based on his chief concerns and treatment expectations. The treatment focused on patient education, muscle strengthening, range-of-motion/flexibility, functional balance, gait training, and pelvic floor strengthening. Also, we incorporated the Mr. L’s favourite pastime into his rehabilitation by having the patient join a dance class with fellow individuals suffering from neurodegenerative diseases. The focus of the patient’s treatment was to maintain/improve his quality of life and function and lessen the burden of the progressively neurodegenerative nature of this disease.

Since the 12-week intervention, the patient’s quality of life and function has improved significantly. Since the treatment was designed specifically for the patient, he will be more likely to adhere to his treatment after his soft discharge from the physiotherapy clinic. The patient should continue to keep in contact with his family doctor, PT, OT, and SLP to help to provide him with different interventions/alternatives as the disease progresses. Unfortunately, MSA is a progressive neurodegenerative disease that results in death; however, our goal as health care professionals is to optimize his quality of life in the present day.

The implications of this case study propose that there are various modes of treatment (e.g., exercise, education, etc.) that can have a significant positive effect on quality of life and everyday function to lessen the burden of the progressively debilitating nature of this disease. For many, the diagnosis of MSA can be overwhelming; however, this case provides some optimism by bringing to light several treatment options that can help maintain the patients' functional abilities. Since MSA is a very rare disease, the research on treatment options is sparse. In the future, research should focus on providing quality research on specific treatments for individuals diagnosed with MSA.

SELF STUDY QUESTIONS[edit | edit source]

  1. Which of the following is false about MSA-P?
    1. MSA-P stands for Multiple System Atrophy - Parkinsonism
    2. Levodopa is a common form of medication for MSA-P
    3. The cause of MSA-P is known
    4. Physiotherapy is a good treatment option for individuals with MSA-P
  2. All of the following are characteristics of both MSA-P and MSA-C except...
    1. Urinary urgency
    2. Erectile dysfunction in males
    3. Significant orthostatic hypotension
    4. Bradykinesia
  3. Which of the following is required for a diagnosis of MSA?
    1. Unresponsive to levodopa
    2. Orthostatic hypertension
    3. Pill rolling tremor
    4. Age of onset is in the 50s

References[edit | edit source]

  1. Wenning GK, Colosimo C, Geser F, Poewe W. Multiple system atrophy. The Lancet Neurology. 2004 Feb 1;3(2):93-103.
  2. 2.0 2.1 Köllensperger M, Geser F, Ndayisaba JP, Boesch S, Seppi K, Ostergaard K, Dupont E, Cardozo A, Tolosa E, Abele M, Klockgether T. Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Movement Disorders. 2010 Nov 15;25(15):2604-12.
  3. Colosimo C, Pezzella FR. The symptomatic treatment of multiple system atrophy. European Journal of Neurology. 2002 May;9(3):195-9.
  4. 4.0 4.1 4.2 4.3 4.4 Kawai Y, Suenaga M, Takeda A, Ito M, Watanabe H, Tanaka F, Kato K, Fukatsu H, Naganawa S, Kato T, Ito K. Cognitive impairments in multiple system atrophy: MSA-C vs MSA-P. Neurology. 2008 Apr 15;70(16 Part 2):1390-6.
  5. 5.0 5.1 5.2 Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, Wood NW, Colosimo C, Dürr A, Fowler CJ, Kaufmann H. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008 Aug 26;71(9):670-6.
  6. Price N, Dawood R, Jackson SR. Pelvic floor exercise for urinary incontinence: a systematic literature review. Maturitas. 2010 Dec 1;67(4):309-15.
  7. 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 Jan 1;31(2):79-83.
  8. Hirsch MA, Toole T, Maitland CG, Rider RA. The effects of balance training and high-intensity resistance training on persons with idiopathic Parkinson’s disease. Archives of physical medicine and rehabilitation. 2003 Aug 1;84(8):1109-17.
  9. Giladi N, Manor Y, Hilel A, Gurevich T. Interdisciplinary teamwork for the treatment of people with Parkinson’s disease and their families. Current neurology and neuroscience reports. 2014 Nov;14(11):1-7.
  10. http://www.ant-tnsjournal.com/Mag_Files/17-1/dw2008530184030_17-1-P74.pdf
  11. Powell LE, Myers AM. The activities-specific balance confidence (ABC) scale. The Journals of Gerontology Series A: Biological Sciences and Medical Sciences. 1995 Jan 1;50(1):M28-34.
  12. Ware Jr JE. SF-36 health survey.
  13. Lins L, Carvalho FM. SF-36 total score as a single measure of health-related quality of life: Scoping review. SAGE open medicine. 2016 Sep 30;4:2050312116671725.
  14. Wenning GK, Tison F, Seppi K, Sampaio C, Diem A, Yekhlef F, Ghorayeb I, Ory F, Galitzky M, Scaravilli T, Bozi M. Development and validation of the unified multiple system atrophy rating scale (UMSARS). Movement Disorders. 2004 Dec;19(12):1391-402.
  15. 15.0 15.1 Podsiadlo D, Richardson S. The timed “Up & Go”: a test of basic functional mobility for frail elderly persons. Journal of the American geriatrics Society. 1991 Feb;39(2):142-8.
  16. Tinetti ME, Speechley M. Prevention of falls among the elderly. New England journal of medicine. 1989 Apr 20;320(16):1055-9.
  17. Duncan PW, Weiner DK, Chandler J, Studenski S. Functional reach: a new clinical measure of balance. Journal of gerontology. 1990 Nov 1;45(6):M192-7.
  18. Vellas BJ, Wayne SJ, Romero L, Baumgartner RN, Rubenstein LZ, Garry PJ. One‐leg balance is an important predictor of injurious falls in older persons. Journal of the American Geriatrics Society. 1997 Jun;45(6):735-8.
  19. Sluijs EM. Patient education in physiotherapy: Towards a planned approach. Physiotherapy. 1991 Jul 10;77(7):503-8.
  20. Hätönen H, Kuosmanen L, Malkavaara H, Välimäki M. Mental health: patients’ experiences of patient education during inpatient care. Journal of Clinical Nursing. 2008 Mar;17(6):752-62.
  21. Scandalis TA, Bosak A, Berliner JC, et al. Resistance training and gait function in patients with Parkinson's disease. Am J Phys Med Rehabil. 2001;80:38-43.
  22. Mazzoni, P., Shabbott, B., & Cortés, J. C. (2012). Motor control abnormalities in Parkinson's disease. Cold Spring Harbor perspectives in medicine, 2(6), a009282. https://doi.org/10.1101/cshperspect.a009282
  23. 23.0 23.1 Keus SH, Bloem BR, Hendriks EJ, Bredero‐Cohen AB, Munneke M, Practice Recommendations Development Group. Evidence‐based analysis of physical therapy in Parkinson's disease with recommendations for practice and research. Movement disorders. 2007 Mar 15;22(4):451-60.
  24. Mak MK, Pang MY. Balance confidence and functional mobility are independently associated with falls in people with Parkinson’s disease. Journal of neurology. 2009 May;256(5):742-9.
  25. Cavkaytar, S., Kokanali, M. K., Topcu, H. O., Aksakal, O. S., & Doğanay, M. (2015). Effect of home-based Kegel exercises on quality of life in women with stress and mixed urinary incontinence. Journal of Obstetrics and Gynaecology, 35(4), 407-410.
  26. Ciantar SR, Bearss KA, Levkov G, Bar RJ, DeSouza JF. Investigating Affective and Motor Improvements with Dance in Parkinson’s Disease. bioRxiv. 2019 Jan 1:665711.
  27. Jain S, Dawson J, Quinn NP, Playford ED. Occupational therapy in multiple system atrophy: A pilot randomized controlled trial. Mov Disord. 2004 Jun 11.
  28. Gargin K, Pizzi L. Wii-HAB: Using the Wii Video Game System as an Occupational Therapy Intervention with Patients in the Hospital Setting. Health Policy Newsletter. 2010 Mar.
  29. Park A, Jang SJ, Kim NE, Kim TH, Sohn YH, Kim HH, et al. Swallowing Outcomes Following Voice Therapy in Multiple System Atrophy with Dysphagia: Comparison of Treatment Efficacy with Parkinson’s Disease. NewsRX LLC. 2021 Mar 25. 502- p.
  30. Fanciulli A, Stankovic I, Krismer F, Seppi K, Levin J, Wenning GK. Multiple system atrophy. Int Rev Neurobiol. 2019 Nov 21.
  31. Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, et al. Progression and prognosis in multiple system atrophy: An analysis of 230 Japanese patients. Brain. 2002 May:1070-83.
  32. Classen D, Mayeux A, Hoy A, Lopez A. Multiple System Atrophy: What You Need To Know. MSA Coalition. 2019 Apr 4.