Progressive Supranuclear Palsy: A Case Study

Abstract

This is a fictional case study of A 67 Year old retired farmer with suspected progressive supranuclear palsy (PSP) 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 to target balance control and decrease neck rigidity. Additionally, our patient was referred to tai chi and qi gong group classes in the community as a novel treatment for this pathology that has been successful in similar populations[1][2][3]. A four wheeled walker (4WW) 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 (PSP-RS) score, Timed Up and Go (TUG) score, and Berg Balance Scale when compared to baseline.

Introduction

Progressive supranuclear palsy is an atypical parkinsonian syndrome, or otherwise known as a Parkinson-plus disorder[4]. 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[4]. When patients living with PSP fall, they have a tendency to fall backwards, which could be a distinguishable characteristic[4].

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 group classes, which was recommended due to research showing its effectiveness in balance rehabilitation in those with non-atypical Parkinson’s patients[1][2][3]. With PSP being a parkinson-plus disorder that has some characteristics similar to Parkinson's, such as balance and motor control deficits, we predict that tai chi and qi gong 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 for treatment of PSP relatively early in the progression of the disease (approximately within 1-2 years post onset), currently without the manifestation of significantly abnormal eye movements[5]. The patient presents with some spastic speech, however the issue is not yet severe, and swallowing difficulties are also still not evident.

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 some difficulty reading his newspaper due to self-reported mildly blurred vision, and he also experiences some mild intermittent tremors in his right and left hands. Jim finds these tremors most noticeable when he holds the newspaper. Jim’s relationship with his wife is becoming strained, which Julia attributes to Jim being 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), and he had also been prescribed Levodopa but was unresponsive (another sign supporting the differential diagnosis of PSP as opposed to Parkinson’s)[4]. This led the physician to suspect that Jim was experiencing PSP. Due to a lack of pharmaceutical treatment options available for this condition, the physician referred Jim to physiotherapy with the request that the PT should target balance rehabilitation[5].

Examination Findings

Subjective Findings

Social History/Family History

  • 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 and 4 grandchildren. Both children live in the US and visit a few times a year for holidays. Patient reports that they talk to their kids/grandkids regularly via Skype.
  • Friends with neighbours in surrounding farms; the closest neighbour is ~1km drive away

Past Medical History

Hypertension, Dislipidemia, previous Left 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 both falling again and of his current falls becoming more frequent.
  • Patient’s wife mentions that Jim has been more irritable this past month. As well as 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 he has 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

Active Range of Motion (AROM):

Cervical Spine Range of Motion (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[6]

  • Total Score 36/100
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 and 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 interferes with sleep, and spastic speech. Jim’s wife reports that Jim is also increasingly irritable, and apathetic 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 recommended.
  • 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

Intervention consisted of:

  1. Participation Goals
  2. Activity Related Goals
  3. Body/Structure Function Related Goals

Participation Long-Term Goal # 1) : 

In 4 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.

  • Firstly, our gait training intervention included educating Jim on the probable course of the disease. Research was presented to Jim that discussed that, typically, in patients with PSP falls begin in the first year and progress to being common within 3 years of onset unless appropriate precautions are adopted[4]. With Jim's fall history, he will need use a gait aid in the future . Following our assessment we educated, fit and prescribed Jim with a 4 wheeled walker that he is required to use when walking outside the house.
  • To help Jim accomplish his goal we recommended that he attends physiotherapy twice a week. We chose treadmill training as an intervention for Jim as it has been previously used as a treatment for patients with PSP to help improve balance and mobility[5]. 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[5]. Furthermore we wanted to help improve Jim's gait pattern. To accomplish this, during each physiotherapy session we video-recorded Jim walking to monitor his progress.

These Physiotherapy sessions included:

  1. Warmup/exercise routine,
  2. Parallel bar training
  3. Timed up and Go training
  4. Treadmill training.

Warm up Exercises (10-15 minutes):

We chose to include warm up exercises to prevent injury, gradually increase Jim's heart rate, as well as building strength, balance and flexibility.

List of Exercises: Jim did not perform all of the warm up exercises provided on each session. However, this list acted as a guide during physiotherapy treatment, based on Jim's goals.

  • Hip Bridge exercise: 2 sets of 8 repetitions .
  • Hamstring stretch while holding a strap in supine: 1 set on each leg holding for 30 seconds
  • Arm Circles: 2 sets: 5 clockwise and 5 times counter-clockwise
  • Marching exercise: 2 sets: 10 times on each foot (Standing and holding on to parallel bar)
  • Single leg balance exercise: 2 sets 30 seconds each foot . (Holding on to parallel bar)
  • Bilateral Shoulder flexion 1 set of 10 repetitions
  • Trunk rotation 3 times on each side. 1 set.
  • Side stretch holding for 15 seconds each side
  • Chin tuck exercise: 5 repetitions 1 set
  • Neck extension 1 set of 3 repetitions
  • Side flexion both sides- 1 set of 3 repetitions on each side
  • Neck rotation both side (turning head) - 3 repetitions on each side 1 set.
  • Neck 360 degrees rotation- 3 cycles done very slowly.

Parallel Bar Training (10-15 minutes):

Our intention behind using parallel bars is to allow 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[5].

  • Walking forward: (Without holding on to parallel bars) 3 sets slow pace forward and back. Followed by 3 sets moderate-fast walking speed
  • Walking in Tandem stance: 3 times back and forth
  • Walking Backward: (Patient holding on to parallel bars) 2 sets: While going backward, the patient is instructed to turn his head and look back to right and left.
  • Walking sideways: (Without holding on to parallel bars) 3 times back and forth.

Timed up and Go (TUG): 3 sets

We chose TUG as an intervention based on TUG score during assessment of 30 seconds, as well as poor scoring on transfer components of the Berg Balance Scale, which puts him at an increased risk of falls.

4) Treadmill Training:

Treadmill Setting used:

  • Treadmill speed 1.0-1.5 km/hr slowly progressed to 2.5 km/hr[5]
  • Heart rate reserve goal to 70-80% of age expected max HR[5]
  • The time on the treadmill was gradually progressed every 2 weeks by 1
    • Week 1: at 7 minutes of treadmill activity.
    • Week 2: at 7 minutes of treadmill activity.
    • Week 3: at 8 minutes of treadmill activity.
    • Week 4: at 8 minutes of treadmill activity.
    • Week 5: at 9 minutes of treadmill activity.
    • Week 6: at 9 minutes of treadmill activity.
    • Week 7: at 10 minutes of treadmill activity.
    • Week 8: at 10 minutes of treadmill activity
    • Week 9: at 11 minutes of treadmill activity
    • Week 10: at 11 minutes of treadmill activity
    • week 11: at 12 minutes of treadmill activity
    • week 12: at 12 minutes of treadmill activity
    • week 13: at 13 minutes of treadmill activity
    • week 14: at 13 minutes of treadmill activity
    • week 15: at 14 minutes of treadmill activity
    • week 16: at 14 minutes of treadmill activity
    • week 17: at 15 minutes of treadmill activity

Participation Short-Term Goal #1:

In the next month Jim wants his physical therapist to help him find a safe exercise program at the local community center to help improve his balance and flexibility. Jim and his wife plan to join the group exercise program together and attend the program once a week. They 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[1][2][3]. Qi gong is essentially a full body low intensity workout which incorporates a repetition of simple movements done slowly while incorporating breathing control. Tai chi is a low impact martial art 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 Qi gong on improvements with Berg Balance Scale scores in patients with Parkinson's disease[1][2][3]. 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 1 session a week.

Activity Goal: Long Term Goal 1:

In 4 weeks Jim wants to be able to independently transfer from bed to washroom without the use of a gait aid.

  • Short Term Goal #1: In 2 weeks Jim will decrease his TUG score to 28 seconds.

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.

Activity Goal: Long-Term Goal 2:

In 4 weeks Jim wants to be able to ascend/descend 10 steps in his home without feeling fatigued.

  • Short term Goal 1: In 2 weeks Jim will be able to ascend/descend 5 steps without feeling fatigued.

Stair climbing exercises with railing were performed once a week with emphasis on proper stepping strategy and use of railing to offload weight while ascending/descending,

Body/Structure Function Long-Term Goal #1:

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

Outcomes (assessed @ 16 week follow-up):

  • VAS: 0/10 at rest
  • AAROM
  • Neck: no significant change in any direction
  • TUG: 25 seconds
  • Berg Balance Scale[7]: 41
  • PSP-RS[6]: 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

At Jim's 4 month re-assessment there were noted improvements in several areas when compared to Jim's baseline assessment outcome measures. There were significant improvements in his gait pattern, where Jim is able to step more smoothly compared to his initial visit, and is able to walk a greater number of steps before losing balance. In addition, Jim reports more confidence transferring from one area of the house to the other. Jim is able to use different objects in his house to transfer safely around the house and to go from standing to sitting safely. However he does report challenges at times while turning and transferring without support, due to increased stiffness. Jim is also able to ascend/descend 10 steps safely with less fatigue than at baseline. Jim still requires the use of side rails, and this is not expected to change throughout the progression of Jim's PSP. Jim's TUG score decreased from 30 seconds in his initial visit to 25 seconds at his 4 month assessment, This indicates that Jim is still at risk for a future fall and that he still requires use of prescribed 4 wheeled walker and is advised to remain using his 4 wheeled walker when outside of his home.

Jim's PSP-RS score decreased from 36 to 26, which would indicate a clinically significant change (MCID=5.7)[8]. Jim still reports neck stiffness but has no pain as evidence by VAS score. Therefore he is advised to keep doing his neck stretches daily, As well, with PSP being a progressive degenerative pathology we do not expect Jim to return to normal neck ROM throughout treatment. However, we do want to maintain as much neck ROM as possible throughout the PSP progression. This was evidenced with no significant decreases in his neck ROM after our 4 months of treatment. Jim has increased excitement ever since joining the Tai chi and Qi gong classes. He has also met new friends in the community and claims the weekly classes also helped him bond with his wife. With Jim meeting all of his goals after 4 months we decided he was safe to be discharged. However due to the progressive decline in functional status we feel that Jim may need continued physical therapy support at least once every 1-2 months. In addition Jim has been referral to have an occupational therapist come to his home for for monthly home visits to assess his ability to perform ADLs safely and adapt his home space as necessary. In addition, we have arranged for a speech language pathologist to come in once a month for home visits to help Jim with his speech impairments and to appropriately manage the expected decline in function.

Referrals

  • to Occupational Therapist (OT) for at home assessment (e.g. addressing potential fall-risks in the home)
  • to Speech Language Pathologist (SLP) for concerns regarding spastic speech and expected progression to issues with swallowing
  • to Social Worker (SW) to address emergent marriage and behavioural issues as well as to aid with transitioning to retirement and subsequently aid Jim and his wife navigate the healthcare system and apply for any available financial funds that he may qualify for
  • to Neurologist for specialist investigation and to have specialist follow up throughout the progression of Jim's PSP

Discussion

Jim achieved clinically significant 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, which would include more severe speech and swallowing problems, as well as abnormal eye movements, other interventions would need to be emphasized in that case[4][5]. 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 relatively early, when falls, bradykinesia, and axial rigidity are still presently the chief symptoms[4][5].

Resources

Berg Balance on Assessment [7]

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References

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  2. 2.0 2.1 2.2 2.3 Liu XL, Chen S, Wang Y. Effects of health Qigong exercises on relieving symptoms of Parkinson’s disease. Evidence-Based Complementary and Alternative Medicine. 2016;2016.
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  6. 6.0 6.1 Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain. 2007 Jun 1;130(6):1552-65.
  7. 7.0 7.1 Qutubuddin AA, Pegg PO, Cifu DX, Brown R, McNamee S, Carne W. Validating the Berg Balance Scale for patients with Parkinson’s disease: a key to rehabilitation evaluation. Archives of physical medicine and rehabilitation. 2005 Apr 1;86(4):789-92.
  8. Steffen T, Seney M. Test-retest reliability and minimal detectable change on balance and ambulation tests, the 36-item short-form health survey, and the unified Parkinson disease rating scale in people with parkinsonism. Physical therapy. 2008 Jun 1;88(6):733-46.