Progressive Supranuclear Palsy: A Case Study

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Title: A case study of progressive supranuclear palsy responding to physiotherapy interventions targeting balance and gait as well as participation in tai chi and qi gong classes

Abstract:

67 Year old retired farmer with suspected progressive supranuclear palsy was referred to physiotherapy by his family physician after the patient was unresponsive to pharmaceutical intervention (Levodopa). The patient’s chief complaints included loss of balance and falls at home, decreased gait speed, minor bilateral tremors, mild blurred vision, and neck rigidity. Physio treatment included gait training, as well as exercises addressing balance and neck rigidity. Our patient was referred to tai chi and qi gong group classes in the community. A four wheeled walker  was also prescribed to support ambulation and decrease risk of future falls. After 16 weeks, re-assessment revealed improvements in the patient's Progressive Supranuclear Palsy Rating Scale score, Timed Up and Go score, and Berg Balance Scale.

Introduction:

Progressive supranuclear palsy (PSP) is an atypical parkinsonian syndrome, or otherwise known as a Parkinson-plus disorder (https://www.ncbi.nlm.nih.gov/books/NBK526098/). PSP is a rare neurological disorder that is usually associated with impaired gait and movement, balance issues, axial rigidity, vision problems, speech and swallowing deficits, as well as mood and cognitive impairments or changes (https://www.ncbi.nlm.nih.gov/books/NBK526098/).

The chief purpose of this report is to demonstrate a possible course of physiotherapy management for patients with progressive supranuclear palsy relatively earlier in the course of the disease (approximately 1-2 years post-onset), and what outcomes might be expected from physiotherapy based on the research we found. Previous case studies have described outcomes in patients with PSP following physiotherapy interventions, however our case also included the prescription of tai chi and qi gong, which was recommended due to research showing its effectiveness in balance rehabilitation in those with non-atypical Parkinson’s patients. (source?). With PSP as a parkinson-plus disorder that has some similar characteristics such as balance and motor control deficits we predict that this will also be beneficial in this patient’s case. Additionally,  we believe that group exercise classes such as these will also provide the patient with an important opportunity for socializing and provide a supportive environment due to the patient’s general isolation.

Our case study is distinguishable in that our patient was referred for physiotherapy treatment for treatment of PSP relatively early in the onset of the disease (approximately within 1-2 years post onset), and presently without the manifestation of significantly abnormal eye movements. The patient presents with some spastic speech, however the issue is not yet severe, and swallowing difficulties are also still not evident.

(https://www.researchgate.net/publication/237085429_PHYSIOTHERAPY_MANAGEMENT_FOR_PROGRESSIVE_SUPRA-_NUCLEAR_PALSY).

One challenge presented to students in the completion of this case was completing the discharge form: due to the rapid progression and chronic nature of PSP, it was challenging to determine when and how exactly a hypothetical patient with PSP would be discharged from PT. We determined in this case that the patient would be discharged following 4 months of intervention, due to re-assessment at that time revealing improvements to gait and balance, and patient having established an effective group exercise and at-home therapy program that they were able to continue without the need for ongoing physiotherapy appointments. A similar case study found that their patient with PSP achieved improvements in gait and balance, as assessed by PSP Rating Scale, following 15 weeks of physiotherapy interventions. Therefore, our hypothetical timeline for PT treatment of 4 months seemed reasonable, before the patient transitioning to exercise management independent of PT, at least for the time being. (https://www.researchgate.net/publication/237085429_PHYSIOTHERAPY_MANAGEMENT_FOR_PROGRESSIVE_SUPRA-_NUCLEAR_PALSY).

Client Characteristics:

Jim is a 67 year old retired farmer that lives with his wife Julia. During the past 2 years Jim has noticed a gradual decline in his balance, culminating in 3 falls at home this month. Jim’s wife Julia has also noticed that Jim is walking much more slowly during their walks together. In addition, Jim has noticed increased difficulty reading his newspaper due to self-reported blurred vision, and he also experiences some mild tremors in his right and left hands, which he finds most noticeable when he holds the newspaper. Jim’s relationship with his wife is becoming strained, which Julia attributes to Jim being much more irritable than he used to be. Jim has also complained of an increasingly stiff neck over the past year, which he finds uncomfortable and which interferes with his ability to sleep at night.

With symptoms becoming increasingly difficult to manage and significant concern over increasing frequency of falls, Jim went to see his family doctor, who initially suspected Parkinson’s disease. Jim underwent MRI imaging which showed the “hummingbird sign”, a sign associated with progressive supranuclear palsy (PSP) (SOURCE-physiopedia). Jim was also prescribed Levodopa but was unresponsive, another sign supporting the differential diagnosis of PSP as opposed to Parkinson’s. The physician suspected that Jim was experiencing PSP, and due to a lack of pharmaceutical treatment options available for this condition, referred Jim to physio with the request that PT should target balance rehabilitation (SOURCE).

Examination Findings

Subjective Findings:

SHx/FHx:

-Patient lives in a 2-storey farmhouse with his wife, large property (6 acres), 4 steps to enter with railing on both sides. Bedroom on 2nd floor, flight of 10 stairs to 2nd floor with railing on left side and wall on right side. Farm supplied with well water and propane for electricity.

-Has 2 children (son and daughter) in mid 40’s, 4 grandchildren. Both live in the US, visit a few times a year for holidays. Patient reports that they talk to their kids/grandkids regularly via Skype.

-Friends with neighbors in surrounding farms; the closest neighbor is ~1km drive away.

PMHx:

Hypertension, Dislipidemia, previous L scapular fracture from MVA (2014)

Medication:

Hydrochlorothiazide, Atorvastatin (Lipidor), Acebutolol (Sectral)

Functional Status:

-Patient repeated primary of neck stiffness and balance issues due to repeated falls at home, reported stiffness currently negatively affecting sleep

-Self-reported decrease in balance and coordination when working around the farm, Patient reports that he has stopped walking down to the fields (approximately 300m) for fear of falling/inability to complete the trip safely.

-Self-reported mild intermittent bilateral tremor in hands, no clear aggravating/relieving factors. Patient reports random onset of tremor, can last for several minutes to several hours

-explains that he fell backwards during the most recent set of falls (3 this last month). Self-reported no injuries from fall, but patient is fearful of falling again and those falls are becoming more frequent.

-Patient’s wife mentions that Jim has been more irritable this past month, as well Jim being uncharacteristically apathetic towards getting the day to day farm work completed. Patient’s wife mentions that she has taken on almost all of the duties around the house (cooking and cleaning).

-Patient reports that they have stopped driving, wife is now doing all shopping and drove patient into today’s appointment.

Objective Findings

-Patient’s speech is slightly spastic while answering questions.

-Patient’s face shows a ”startled” appearance (widened eyes, furrowed brows)

-Baseline VAS score of 2/10 in neck

Posture Analysis:

Jim has a stooped, kyphotic posture in quiet stance

AROM:

Cervical Spine ROM:

Flexion	22°
Extension	29°
Right Side Flexion	12°
Left Side Flexion	10°
Right Rotation	19°
Left Rotation	14°

(Cervical Spine AROM severely diminished in all directions)

Cervical PROM = AROM

UE & LE AROM: All WNL except L shoulder flex + adduction both limited ~130° (-50°)

Neurological assessment:

Babinski’s sign (+)

Clonus (-)

Hoffman’s (-)

Reflex Grading:

U/E:	L	R
Bicep (C6)	2	2
Tricep (C7)	3	3+
L/E:
Patellar (L4)	2	3
Tib Post (L5)	3	2
Achilles (S1)	3+	3

Finger to nose test:

-Patient able to complete repetitive finger to nose task with minimal over/under shooting in movement with bradykinesia, particularly when approaching target

Finger Opposition:

-Patient able to complete task with observed bradykinesia, patient reported large cognitive demand to complete task and only completed 1 full cycle before stopping

BERG Balance Scale: 36/56 (see attached)

-patient able to sit and stand independently, requires use of hands for transfers, observed bradykinesia in transfers (sit ↔ stand, chair ↔ chair) and in gait while stepping

Timed up and go (TUG): 30s

-Observed bradykinesia in movement, patient requires several attempts to go from sit to stand without use of hands

Progressive Supranuclear Palsy(PSP) Rating scale: Total Score 37/100

Total Score PSP-RS	36/100
History	7/24
Mental	4/16
Bulbar	2/8
Ocular	3/16
Limb	6/16
Gait	14/20

Gait Analysis:

Revealed a slow, stiff, drunk-like stepping pattern with wide and uncertain steps. Patient pivots quickly, with visible instability and walks with a stiff upright trunk with arms slightly abducted. Observed rigidity in trunk and neck in both standard gait and in pivot (failure to turn head towards pivot direction).

Clinical Impression

Jim is a 67 y/o male who is presenting with a decline in balance culminating in several falls recently, decreased gait speed, neck stiffness that also interferes with sleep, and spastic speech. Jim’s wife reports that Jim is also increasingly irritable, suggesting a possible cognitive-behavioural impact in addition to physical manifestation.

Problem list

-Patient is at risk for falls due to reduced balance, which can lead to a future injury. Use of a a four wheeled walker is reccomended.

-Patient has high degree of neck rigidity, which has affected his range of motion

-Patient has decreased gait speed which affects his ability to participate in meaningful social functions as well as activities of daily living

-Difficulty with sleeping, which patient attributes to neck rigidity

-Patient is experiencing spastic speech, making him unable to efficiently communicate and express how he is feeling

Intervention:

Participation Goals:

Long-term Goal # 1)  In 2 months Jim wants to be able to walk outdoors with his wife for 15 minutes with the use of a 4 wheeled walker without losing balance. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5291505/)

-To help Jim accomplish his goal we recommend that he attends physiotherapy twice a week. We chose treadmill training as an intervention for Jim because it has been used as a treatment for patients with progressive supranuclear palsy to help with balance (Source). Treadmill training in conjunction with traditional physiotherapy treatment can lead to improvements in Berg Balance Scores, 6 minute walk test scores, and lead to decreased PSP rating scale scores (source). Each week we will increase his time on the treadmill by 1 minute. Each physiotherapy session we will include treadmill training.

-Treadmill speed 1.0-1.5 km/hr slowly progressed to 2.5 km/hr (source)

-Heart rate reserve goal to 70-80% (source)

Week 1: at 7 minutes of treadmill activity.

Week 2: at 8 minutes of treadmill activity.

Week 3: at 9 minutes of treadmill activity.

Week 4: at 10 minutes of treadmill activity.

Week 5: at 11 minutes of treadmill activity.

Week 6: at 12 minutes of treadmill activity.

Week 7: at 13 minutes of treadmill activity.

Week 8: at 14 minutes of treadmill activity

Week 9: at 15 minutes of treadmill activity

Before starting treadmill training we felt it was appropriate to include warm up activities to build strength balance and flexibility.

Warmup Exercises:10-15 minute warm-up before going on the treadmill.

-Hip Bridges to stretch hip flexors and strengthen hip extensors

-Marching exercise 10 times on each foot 2 sets

-Single leg balance

-side stepping exercise

-Side stretch holding for 20 seconds each side

-trunk rotation 3 times on each side.

A) Treadmill

(source) found that the use of treadmill training as an intervention can improve balance in patients with PSP. Based on Jim’s goal to improve his balance, we feel this is an important intervention to build aerobic endurance gradually, and improve ambulation, and balance.

B) Parallel Bars

We feel the use of parallel bars is appropriate at this stage of Jim’s treatment plan because he is able to walk independently without the use of a gait aid however he often stumbles and loses balance when walking for longer durations. Our intention behind using parallel bars is to help Jim learn strategies to fall safely. Furthermore the use of parallel bars allows Jim to practice walking moving forward, backward and sideways without fear of falling backwards. During our treatment we alternated between the use of 2kg ankle weights and also walking without ankle weights.

FITT parameters:

Walking forward:

3 sets slow pace

3 sets moderate-fast walking speed

Walking Backward:

2 sets: Patient holding on to parallel bars. While going backward, the patient is instructed to turn his head and look back to right and left.

Walking sideways

3 times back and forth

Walking in Tandem stance

3 times back and forth

Gait training intervention included educating Jim on the probable course of the disease. Research was communicated to the patient that demonstrated that gait aids were required on average within 3 years of onset of PSP, and that therefore Jim may need to transition to the use of a gait aid in the future (SOURCE).

Long term Goal #2) In the next month Jim wants his physical therapist to help him find a safe exercise program in the local community center to help improve his balance and flexibility. Jim and his wife have a goal of doing this exercise routine together 3 times a week. Jim and Julia both also believe that having a group to socialize with would be helpful.

-The use of Tai chi and Qi gong has been studied extensively as an intervention for patients with parkinson's disease Qi gong is essentially a full body workout which incorporates a repetition of simple movements done slowly while incorporating the breath. Tai chi is actually a martial art and is a low impact activity that uses a series of movements together. In contrast, Qi gong does the same movement repeatedly, where tai chi movements flow from one movement sequence to the other. Several Studies have shown the effectiveness of both tai chi and chi gong on improvements with berg balance scale in patients with parkinson's disease SOURCE). For this reason we felt it was appropriate to use as an intervention. We have referred Jim to a combined Tai chi and Qi gong group exercise program offered at the local YMCA, which Julia will drive Jim to for three sessions weekly.

Activity Related Goal:

Jim wants to be able to transfer from bed to chair without feeling like he is going to fall down. Each physiotherapy session we practiced timed up and go 3 times to help train Jim move and transfer on-command (with use of gait aid and also without use of gait aid) to replicate how he would move around at home to get from one place to another. We also challenged Jim to stop on command and maneuver around objects to make the timed up and go intervention more challenging.

Body/Structure Function Goals:

Jim has a goal of reducing neck stiffness and improving Neck ROM.

-Chin tuck exercise in combination with forward flexion 3 sets

-neck extension 3 sets

-Side flexion both sides- 3 sets

-Neck rotation both side - 3 sets

-Neck 360 degrees rotation- 3 sets.

Outcomes (assessed @ 16 week follow-up)

TUG: 25 seconds

Berg Balance Scale: <INSERT>

PSP-RS: 26/100

Total Score PSP-RS (Follow-Up @ 16 weeks)	26/100
History	6/24
Mental	2/16
Bulbar	2/8
Ocular	4/16
Limb	4/16
Gait	8/20

Gait Assessment:

-Patient more confident in gait albeit still broad and uncertain comparative to healthy age-matched population

-Patient reports more confident, less fearful of falls while walking

Discharge Notes

<INSERT>

Referrals

Referral to occupational therapist primarily to

Speech language pathologist for concerns regarding spastic speech

Discussion

Jim achieved improvements in the TUG, Berg, and PSP Rating Scale outcome measures as a result of physiotherapy interventions and referral to group tai chi and qi gong exercise programs. This case study serves as a hypothetical scenario where the role of physiotherapist was demonstrated effectively in the case of a patient presenting relatively early in the onset of PSP (around 1-2 years post-onset). In alternative cases where other distinguishing symptoms of PSP are already present, including more severe speech and swallowing problems as well as abnormal eye movements, other interventions would need to be emphasized. From our research, symptoms such as abnormal eye movements are typical later in the course of PSP, whereas Jim is a hypothetical patient recognized and referred to physiotherapy quite early, when falls, bradykinesia, and axial rigidity are still presently the chief symptoms.

Self-study questions

What is a characteristic that distinguishes progressive supranuclear palsy from Parkinson’s Disease?

A. Kyphotic posture

B. Falling backwards as opposed to forwards*

C. Extreme Tremors

D. Sleep difficulties due to decreased balance ability

When treating patients with PSP, which management technique would most likely be beneficial, particularly when targeting balance deficits?

A. Manual Therapy

B. Strength training program

C. Tai chi and Qi Gong*

D. Bedrest (no intervention)

What is the minimal clinically important difference (MCID) for the Progressive Supranuclear Palsy Rating Scale (PSPRS)?

1. 6.5
2. 5.5
3. 5.7*
4. 4.9

References