Primary-Progressive Multiple Sclerosis (PPMS) - A Case Study

Abstract

Purpose: The case study was designed for Queen’s University PT858 Neurorehabilitation II course. The purpose of this fictional case study is to understand and explore an individual diagnosed with Primary Progressive Multiple Sclerosis (PPMS). Additionally, we investigated and applied a research-based treatment approach while integrating the role of technology in rehabilitation.

Case Presentation: This fictional case study focuses on Michelle White a female 47 year old work from home accountant who recently progressed from active primary progressive multiple sclerosis (PPMS) without progression to active PPMS with progression. Her primary complaints were fatigue, difficulty with normal gait, impaired balance, weakness and decreased attention and memory.

Intervention: Patient was prescribed activities and exercises that will maintain and improve her function as her MS continues to progress. These interventions are designed to target the short and long term goals of the patient to allow her to regain and maintain function that is meaningful to the patient. Prescribed exercises include aerobic exercise, resistance training, balance training, fatigue management, education as well as referrals to target problems that are outside the scope of physiotherapy. The use of technology in rehabilitation is used throughout the different aspects of intervention.

Outcomes: At the 4 month reassessment, Michelle White was reassessed in the following tests; Multiple Sclerosis Walking Scale, 6 Minute Walk, mini BESTest, Activities Balance Confidence Scale, 5 times sit to stand test and the Modified Fatigue Impact Scale. After completing her physiotherapy sessions, her home exercise routine and additional appointments with members of her inter-professional team, Michelle was able to improve her ability and confidence with ambulation, balance and activities of daily living.

Introduction

Multiple sclerosis (MS) is a neurological, autoimmune disease that affects the central nervous system (HOPKINS). Though the exact etiology of MS is unknown, it occurs when the immune system attacks nerve fibers and the myelin sheath of neuronal axons, leading to inflammation and demyelination of the central nervous system (TAFTI ET AL., 2022). As a result, it alters electrical signaling in the brain and leads to a variety of symptoms, such as difficulty with walking, blindness, paralysis, etc. In Canada, over 90,000 people are living with MS, which equates to 1 in every 400 people (MS SOCIETY). Annually, 4,377 Canadians are diagnosed with MS, which equates to almost 12 people per day (MS SOCIETY).

A thorough medical history in conjunction with a neurological examination and imaging are used to diagnose MS, and MS is diagnosed once other potential reasons for symptoms are ruled out (MS SOCIETY). MS can be classified into four types of diagnoses: clinically isolated syndrome, relapsing remitting, secondary progressive and primary progressive (NATIONAL MS SOCIETY).

Primary progressive multiple sclerosis (PPMS) is characterized by a gradual deterioration in functional capacity due to neurological deficits, without the occurrence of early relapses (exacerbation or flare-ups). Furthermore, this type of MS can be categorized as active (confirmed via an occasional relapse and/or imaging) or inactive, as well as progressive (evident decrease in functional capacity) or non-progressive (NATIONAL MS SOCIETY). Globally, 10-15% of individuals diagnosed with MS present with PPMS (TAFTI).

The fictional case study will highlight the experience of Michelle White, a 47-year-old female who was diagnosed with PPMS. Michelle was referred to physiotherapy due to an acute exacerbation of PPMS, and her primary goals with rehabilitation are to improve her walking capacity and ability to perform activities of daily living. The purpose of this case study is to provide evidence-based information from the literature regarding symptoms, objective findings, and interventions for PPMS.

Client Characteristics  

Michelle White is a 47-year-old female (DOB March 14th, 1970) who was diagnosed with primary progressive MS 2 years ago. Since the onset of her symptoms, Michelle’s status has changed from being in the active without progression PPMS stage (increasing accumulation of disability), to active (new MRI changes) with progression (increasing accumulation of disability). Michelle was diagnosed with clinical depression 1 year ago and has been managing hypertension for the last 5 years. Michelle is a work-from-home accountant, and currently resides in a detached home in Mississauga, Ontario with her husband, 2 children, and family dog. Michelle was referred to rehabilitation by her neurologist to reduce the risk of a relapse, as well as to help improve her mobility (walking with the use of a mobility aid and balance training) and function (activities of daily living).

Examination Findings

Subjective Assessment

Chief Complaint: Michelle has concerns about taking her dog for daily walks in the summer. She is afraid of losing her balance and worried she won’t have enough energy to complete the walks. Michelle is also having trouble with the stairs in her house. She finds her legs weak and she is tired by the time she reaches the top of the stairs.

History of Present Illness:

• Medical diagnosis: PPMS active with progression
  • MRI to confirm and identify areas of lesions in the brain
  • Positive lumbar puncture test
  • Positive evoked potentials test

• Treatment to Date: No previous history of physiotherapy treatment.

• Current Symptoms:
  • Impaired mobility
  • Weakness and stiffness in the legs, the right leg worse than the left.
  • Decreased attention, and memory
  • Pins and needles in the feet
  • Urgency incontinence  
  • Fatigue
  • Difficulty with normal gait
  • Balance impairments

Past Medical History:

• Diagnosed with active PPMS without progression 2 years ago
• Diagnosed with active PPMS with progression 2 weeks ago
• Comorbidities:
  • Depression - diagnosed 1 year ago
    • Prescribed medications
    • Weekly visits to psychiatrist to help with depressive symptoms
  • Hypertension - diagnosed 5 years ago

Medications:

• Oral Paroxetine - 20mg, once a day for depressive symptoms
• Norvasc (Amlodipine) - 5mg, once per day for hypertension
• Ocrevus - 600 mg intravenous infusion every six months

Social History: Michelle lives in Mississauga in a house with 2 steps to get into the house as well as 12 steps to get to the second floor. She lives there with her husband, 2 children and family dog. She has been a work-from-home accountant for the last 15 years. Michelle enjoys working from home but has noticed in the last few months that she has trouble concentrating on her work and thinks her memory has gotten worse. Her family recently moved to Mississauga from Ottawa in the last year, and friends and  distant family are still located in Ottawa. Michelle enjoys walking her dog daily, reading and watching movies. However, she finds when she sits in the same position for a long period her pain and discomfort increases.

Family History: History of hypertension on maternal side

Functional History: Michelle was previously able to ambulate 45 minutes with dog without use of gait aid. She was also less fearful of community ambulation. Michelle also previously enjoyed gentle weight-bearing resistance training at her local gym, she would often go twice a week for about 30-45 minutes. Michelle was also able to walk up a flight of stairs without fatigue. She had no problems with her daily activities such as grooming, cleaning and cooking. However, she found carrying laundry up the stairs quite difficult but often received help from her husband or kids.

Current Functional Status: Michelle has been cutting back on the length of her workouts at the gym as she now finds resistance training more painful and difficult due to balance impairments. Michelle has also become more fearful of community ambulation due to fear of falling, and currently walks around the block. She only walks the dog twice a week due to her balance and stability deficits and will only go if her husband or one of her children accompanies her. Michelle is often fatigued and feels weak after walking up a flight of stairs and is unable to carry her laundry up the stairs anymore. She still manages her daily activities well, but finds herself tired if she does a large clean of the house.

Patient Goals:

1. Michelle would like to get back to walking her dog daily, independently.
2. Michelle would also like to limit the progression of her symptoms.
3. Michelle would like to improve her strength to be able to walk up stairs better.

Objective Assessment

Range of Motion

• UE - WNL
• LE - Table #1

Table #1: Joint Range of Motion

Joint	Movement	Active ROM - LEFT	Passive ROM - LEFT	Active ROM - RIGHT	Passive ROM -RIGHT
Hip	Flexion	100	105	90	100
Hip	Extension	10	15	10	15
Hip	Adduction	20	30	20	30
Hip	Abduction	40	45	40	45
Hip	External Rotation	30	40	30	40
Hip	Internal Rotation	30	40	30	40
Knee	Flexion	125	130	115	125
Knee	Extension	0	-5	-5	-5
Ankle	Plantar flexion	40	45	35	45
Ankle	Dorsiflexion	10	15	5	10
Ankle	Inversion	15	20	10	15
Ankle	Eversion	5	10	5	10

Spasticity

Modified Ashworth Scale

• UE - WNL
• LE - Table #2

Table #2: Modified Ashworth Scale

Muscle Group	Left Limb	Right Limb
Hip Flexors	0	0
Hip Extensors	0	0
Hip Adductors	0	0
Hip Abductors	0	0
Knee Flexors	1	1
Knee Extensors	1	1
Ankle Plantar Flexors	1+	2
Ankle Dorsiflexors	1	1+

Strength

5 Times Sit to Stand Test: 7.3 seconds

Manual Muscle Testing (MMT)

• UE - WNL
• LE - Table #3

Table #3: Manual muscle testing

Muscle Group	Score Left Limb	Score Right Limb
Hip Flexors	4+	4+
Hip Extensors	4	4
Hip Adductors	4	4
Hip Abductors	4	3+
Hip External Rotators	4	3+
Hip Internal Rotators	4	3+
Knee Flexors	4	4-
Knee Extensors	4	4-
Ankle Plantar Flexors	4	4
Ankle Dorsiflexors	4-	3+
Ankle Inverters	4-	4-

Neurological

• Dermatomes - WNL

• Myotomes - WNL
• Reflexes
  • UMN
    • Clonus - postitive
    • Babinski - positive
  • LMN
    • Patellar - 3/4
    • Achilles - 3/4

• Sensation testing - decreased pinprick sensation to dorsal feet bilaterally

• Proprioception - contralateral joint matching task - reduced proprioception found in the ankle joint

Gait

• Gait Deviations:
  • Foot drop during swing - right worse than left
  • Poor heel strike during initial contact
  • Shortened step length
  • Knee buckling and hyperextension in mid-stance - right more than left
• Assessment Measures
  • 6 Minute Walk Test: 348m
  • Multiple Sclerosis Walking Scale – 12 (MSWS-12): 36/60 (60%)
  • See Figure 1

Figure 1: Multiple Sclerosis Walking Scale 12

Balance

Assessments:

• Mini BESTest: 11/28
  • See Figure 2

Figure 2: Mini BESTest

• Activities Balance Confidence Scale (ABC): 69%
  • See Figure 3

Figure 3: Activities Specific Balance Scale

Fatigue

• Modified Fatigue Impact Scale (MFIS): 50/84
  • See Figure 4

Figure 4: Modified fatigue impact scale

Clinical Impression

Patient Profile

Mrs. White is a 47-year-old female with PPMS that has been experiencing declining function since her disease progressed to active with progression. Currently, Mrs. White is presenting to outpatient rehabilitation with significantly impaired mobility and balance as a result of lower extremity weakness and spasticity, reduced sensation of the feet, gait deviations and fatigue. Additionally, Mrs. White is also experiencing urgency incontinence and cognitive deficits that are also impacting her quality of life.

Problems list

• Body structure function
  • Reduced balance due to decreased proprioception and weakness in lower extremities.
  • Impaired memory due to disease progression.
  • Impaired sensation due to irreversible destruction of the myelin sheath and axons of nerves.
  • Mild drop foot gait with poor heel strike due to weak dorsiflexors.
  • Increased muscle tone/spasticity around ankle and knee joint due to demyelinated nerves in brain and spinal cord.
  • Weak hip abductors, hip external and internal rotators, knee extensors and flexors and ankle dorsiflexors due to demyelinated peripheral nerves.
• Activity
  • Difficulty climbing stairs due to fatigue and SOB.
  • Urgency incontinence due to weakened sphincter muscles.
  • Reduced attention span due to fatigue and cognitive deficits.
  • Reduced walking endurance due to gait deviations and fatigue.
• Participation
  • Unable to walk her dog due to impaired mobility and fatigue.
  • Avoids resistance training due to pain and lack of stability.
  • Difficulty concentrating at work due to impaired memory and attention deficits.
• Personal factors
  • Patient is fearful of falling.
  • Reduced productivity at work due to pain during prolonged positioning.
  • Easily fatigued.
  • Diagnosed with depression 1 year ago.
• Environmental
  • The patient's home has 2 steps to enter, and 12 steps to reach the second floor.

Interventions

Patient Centered Treatment Goals

Short Term Goals:

• Patient will achieve a minimum of a 4/5 MMT score for all lower extremity muscle group
• Improvement in Activities Balance Confidence Scale score from 69% to 86% within 2 weeks.
• Be able to ambulate up and down 12 stairs with a single-point cane independently within 2 weeks.

Long Term Goals:

• Patient will be able to lower her score on the MFIS by 10-20 points within 6 weeks .
• Patient will be able to maintain or improve her lower extremity MMT scores at current baseline within 6 months.
• Patient will be able to continue use of single-point cane without requiring a more stable gait aid over the next 4 months.

Strength Training:

• Functional exercises are prioritized, mainly targeting large muscle groups.
• Examples of functional exercises:
  • Farmers Carry
  • Sit to Stand
  • 1/2 Squat
  • Step ups and Step downs
  • Heel walking & Toe walking

• Strengthening muscles of the lower extremity will help with improving gait as well as counteract spasticity present in ankles and knees.
• Muscle groups and examples:
  • Dorsiflexors: Toe raises, resisted dorsiflexion
  • Plantarflexors: Seated heel raises, standing heel raises
  • Knee Flexors: Hamstring curls, RDLs
  • Knee Extensors: resisted knee extension, mini squat
  • Hip Abductors: side-lying hip abduction
• General parameters for strength training, used for all exercises listed above:
  • 8-12 reps at 60-80% of the patients one repetition max performing 2-3 sets, 2-3 times per week. Rest for 2-3 minutes between sets is recommended.
  • Patient should be reaching fatigue within the outlined rep range. If the patient is able to reach 12 reps with minimal to mild fatigue, resistance should be added or increased.

Gait Training:

• Prescribe and fit patient to a single-point cane to improve stability while maintaining as much mobility as possible.

• Gait training may include:
  • Body weight treadmill training
  • Robot-assisted training

• FES can be incorporated to help improve gait pattern in the early stages by facilitating dorsiflexion through activation of the tibialis anterior to improve drop foot gait.
• Ideally continue with gait training until single-point cane is no longer required for stable ambulation. This is dependant solely on the changes that are seen within the patients condition; both improvements in function as well as progressions of PPMS decreasing function.

Aerobic Training:

• Aerobic exercise will improve the patients aerobic capacity and improve her ability to walk for longer periods of time without fatigue.
• Should be performed 3-4 times per week working up to around 20-30 minutes.
• Methods of aerobic training include:
  • Treadmill training, swimming, walking, arm bike
    • From all options listed above, patients goals align most with walking therefore walking will be prioritized.

• Be mindful of the patients level of fatigue throughout training.

Balance Training:

• In clinic exercises, performed with supervision and guarding, roughly 2 minutes of each activity. As the patient gets more comfortable, balance exercises can be performed outside of the clinical setting. Examples include:
  • Standing eyes open, progressing to eyes closed and widening base of support as needed
  • Ankle sways
  • Marching in place
  • Reaching, nudging and leaning in seated progressing to standing as well as to eyes closed as tolerated
  • Adding cognitive task to any or all of the above to increase intensity and difficulty for the patient
• Other alternatives to balance exercises include: Tai Chi, Yoga, Pilates
  • Patient is encouraged to find a program or class in one of the categories listed above that is of most interest to her in order to train balance in a different environment.

Education:

• Reducing fatigue can be addressed with the 4P's: Pacing, Planning, Prioritizing, and Positioning.
  • Any exercise can be broken up throughout the day to further manage fatigue.
  • Educate the patient to take breaks as required.
• Patient is educated on the use of a single-point cane with a 2 point step-to gait pattern, progressing to a step-through pattern as the patient gets comfortable and her balance and stability improves.
• Patient is informed that exercise is important and movement is always encouraged
  • As the disease may progress, exercise is modified to ensure that the patient is doing as much as they can based on their new functional status
• Patient should seek Physiotherapy even when her symptoms are stable to follow up with functional status and make any changes to treatment plan as necessary.

Referrals:

• Patient will be referred to a Pelvic Floor Physiotherapist to help treat her urgency incontinence.
• Patient will be referred to an Occupational Therapist to work on improving memory and attention.
• Patient will be referred to a Psychologist to control her depression if she is not currently seeing one.

Outcome

After Michelle’s initial assessment on May 3, 2022 she was reassessed on September 4th, 2022,  about 4 months later. She was reassessed using the Multiple Sclerosis Walking Scale, 6 Minute Walk, mini BESTest, Activities Balance Confidence Scale, Modified Fatigue Impact Scale and 5 times sit to stand. The table #3 below shows the change in each test. Michelle’s improvement in the Multiple Sclerosis Walking and her 6 minute walk test demonstrates her improvement in her ability to walk, including improving her pace, distance and effort needed to walk. Michelle's improved mini BESTest, Activities Balance Confidence scale and 5 minutes sit to stand demonstrates the positive impact of the interventions on her dynamic balance, functional mobility and confidence in balance. While completing these tests, Michelle experienced less overall fatigue.

Outcome Measure	Initial Assessment: May 3rd, 2022	Reassessment: September 4th, 2022
Multiple Sclerosis Walking Scale	36/60 or 60%	28/60 or 47%
6 Minute Walk	348m	387m
Mini BESTest	11/28	16/28
Activities Balance Confidence Scale	69%	76%
5 times sit to stand test	7.3 seconds	6.9 seconds
Modified Fatigue Impact Scale	50/84	39/84

After the 16-week intervention, we are pleased with the progress Michelle has made and believe she is ready for discharge from physiotherapy. Michelle will continue with her home exercise program and understands she is able to contact the clinic if she has any questions or concerns. No further referrals are needed at this time.

Michelle responded positively to the assessment with a pelvic floor physiotherapist to provide support for her urinary incontinence. She will continue to see the pelvic floor physiotherapist. She will continue to have follow-up appointments with other members of her inter-professional team including her neurologist and general practitioner to follow the progression of her PPMS as well as her psychiatrist to help support her depression.

Self-Study Questions

1.Which of the following is not true related to the Mini-BESTest?

a) It assesses the patient’s compensatory stepping ability

b) It assesses postural control

c) It does not include dynamic gait tests

d) It is frequently used on patients with Multiple Sclerosis

2.Which is true about Multiple sclerosis?

a) MS is more prevalent in males than females

b) Fatigue is not a common symptom of MS

c) MS is more common in Europe

d) Symptoms progress faster in males than females

3.What is the best intervention approach for a physiotherapy session for someone with Multiple sclerosis?

a) High intensity and high repetition during physiotherapy sessions

b) Functional activities that focus on balance and gait

c) Having the physiotherapy session focus only on upper extremities

d) All of the above would be helpful in improving MS symptoms

Answers: (1) C (2) D (3) B

References

Original Editor - Krystin Rudge as part of the Queen's University Neuromotor Function Project Top Contributors - Brooke McIntosh, Krystin Rudge, Erin Locke, Crystal Robello, Cindy John-Chu, Uchechukwu Chukwuemeka, Sahil Sareen, Kim Jackson and Carina Therese Magtibay