A Case Study about Progressive Supranuclear Palsy

Introduction

Progressive Supranuclear Palsy or PSP is a neurological disorder that affects your movement, such as eye movement, walking, balance, and extremities. Additionally, it can also affect your cognition and behaviour. People diagnosed with PSP typically are middle aged, however the actual cause of this disorder is not known. Upon diagnosis, the disease worsens quickly, and can turn severe within 5 years. Although PSP shares similar symptoms to those with Parkinson’s disease, there are some key differences such as axal rigidity, early onset eye movement dysfunction, and severe complications with speech and swallowing. Treatment options are scarce for individuals with PSP, and little research supports the use of medications as an effective treatment technique. The purpose of this case study is to identify ways that physiotherapists can help diagnose individuals with PSP, and treat this condition using an evidence-based approach. Additionally, this case study will look at PSP 1-2 years after diagnosis and aim to treat patient specific goals for common PSP symptoms such as balance and gait, as well as go through a typical physiotherapist’s plan with a patient with PSP.

Client Characteristics

William Quillesburg is a 62 year old male patient who was referred to our private clinic from a family physician to help manage William’s symptoms of PSP. William first began experiencing signs of PSP on June 20th 2021 while walking his dog one morning. That morning, William noticed his head shift backwards inadvertently and lost his balance which caused him to fall on his back. He did not think much of this, however over the next year he experienced similar falls while walking his dog. Furthermore, the frequency at which the falls started to occur increased. It was not until the morning of April 1st 2023 that he finally decided to seek help from the physician because over the course of a few months William developed inability to control his eyelids, neck stiffness and difficulty swallowing. William was diagnosed with Progressive Supranuclear Palsy, one and a half years following the initial onset of symptoms. Due to the lack of evidence supporting the use of pharmaceuticals for treatment, William has been referred to physiotherapy by the physician to help manage his current symptoms and prevent further onset of this disease.

Examination Findings

Subjective Findings

Past Medical History (PMHx)

One common comorbidity in patients with PSP that can be screened for is hypertension. William has had hypertension and high cholesterol levels for the past 7 years. No other significant medical history before PSP diagnosis. Poor response to L-Dopa. MRI identified hummingbird sign which highly suggests PSP (Pandey, 2012).

Medications (Meds)

Lisinopril for hypertension (20 mg daily), atorvastatin for high cholesterol (40 mg daily)

Health Habits (HH)

No history of smoking or drinking.

Family History (FHx)

His father had Alzheimer’s and his mother suffered a right MCA stroke in her 60s

Social History (SHx)

Currently retired and lives alone. Used to play Bingo every second day at the local Bingo center (15-minute walk away), and walk his dog everyday; not as able to anymore.  

Functional History (FnHx)

Prior to the injury, was independent and had no difficulties with ADLs. No gait aids.

Current Functional Status (FnSt)

PSP affects his ability to walk and maintain balance, leading to falls. Uses a single He has developed some fear of falling and loss of confidence navigating more uneven terrain. Unable to get into and stand in shower without proprioceptive feedback; relies on handrail. Inhibited eyelid control leaves his eyes dry and often tired. Difficulty swallowing is starting to affect his ability to eat and socialize with others. Sleep is affected.

Objective Findings

Observation: presents with stooped posture and tilts head. Looks fatigued/anxious.  

Gait: reduced stride length and decreased swinging of arms bilaterally. Requires some assistance to maintain balance when walking. Recruits a wide base of support.

AROM/PROM:

Cervical ROM

• Flexion: 24°

• Extension: 30°
• Left side flexion: 16°
• Right side flexion: 15°
• Left rotation: 18°
• Right rotation: 21°

Shoulder ROM was limited bilaterally in external rotation (41°) and flexion (62°). ROM at other UE and LE joints WNL. AROM and PROM were equally limited.

Neurological testing:

• Dermatomes and myotomes intact bilaterally. Slump was positive bilaterally.

Reflex Test	Left	Right
Babinski	+	+
Oppenheimer	+	+
Clonus	-	-
Biceps (C6)	3	3
Triceps (C7)	2	2
Patellar (L3-L4)	2	2
Achilles	2	2

MMT: bilateral weakness (3-) in knee extension. Bilateral weakness (4-) in hip flexion and ankle dorsiflexion.

TUG: required 29 seconds to complete.

Berg Balance Scale: 35/56. Difficulty maintaining balance with feet together and eyes closed. Requires support in tandem stance.

Progressive Supranuclear Palsy Rating Scale (PSP-RS): scored 44/100 on the PSP-RS.

History	10/24
Mentation	5/16
Bulbar	3/8
Ocular motor	3/16
Limb motor	9/16
Gait and midline	14/20

PSP-QoL: 64/100

Clinical Hypothesis

William McQuabbie is a 62 year-old male experiencing impaired balance leading to frequent falls which disrupt his ability to walk his dog and get to the local bingo center. He demonstrates symmetrical axial rigidity, muscle stiffness associated with dysphagia, issues with gait, and starts to face some mental challenges.

Prognostically, the disease is progressive with most surviving up to 5-7 years after diagnosis. However, he is starting from a good baseline as an active individual who walked his dog everyday previously.

Problem List

• Impaired balance leading to falls. Berg Balance Score of 35/56, indicating moderate falls risk.
• Decreased ambulation tolerance. TUG requires 29 s to complete.
• Social isolation and increased withdrawal from hobbies like playing Bingo and walking his dog. 64/100 on PSP-QoL score which could be improved.

Intervention

While understanding that William presented with PSP symptoms such as impaired balance leading to falls, axial rigidity, and muscle stiffness, it is important to note that William was not considered “severe” and can follow similar treatment guidelines to Idiopathic Parkinson’s disorder as certain treatment techniques have shown to positively benefit both disorders.

Our intervention approach aimed to be client-centered by creating specific short-term and long-term goals based on William’s current phenotype and problem list, while maintaining an evidence-based intervention style. It was important to attempt to get William back to doing his regular routine within limits, such as walking his dog, and participating in his bingo sessions. With these factors in mind, an intervention program was developed.

The intervention program involved short-term and long-term goals for the following ICF domains:

1. Body Function and Structure
2. Participation
3. Activities

The goals for each domain are listed below:

Short-term Goal #1 for Body Function and Structure:

William will score at least 38 on the Berg Balance Scale after 4 weeks of Physiotherapy.

Long-term Goal #1 for Body Function and Structure:

William will be able to walk his dog again with no falls, and the assistance of a mobility aid, after 5 months of physiotherapy.

Short-term Goal #2 for Participation:

William will be able to maintain at least 63/100 on the PSP-QoL scale after 3 weeks of Physiotherapy.

Long-term Goal #2 for Participation:

William will be able to navigate to weekly bingo sessions, play independently, and return home safely using his mobility aid after 5 months of Physiotherapy.

Short-term Goal #3 for Activities:

William will be able to complete the TUG test in 25 seconds within 4 weeks of Physiotherapy treatment.

Long-term Goal #3 for Activities:

William will be able to safely travel 400m with his mobility aid, with no complications, after 12 months of Physiotherapy treatment.

Treatment:

First, we fitted William with a quad point cane so that we can reduce the falls risk present in First, we recommended that William ideally attended physiotherapy sessions 3 times a week, in order to have the best chance to maintain or improve his current presentation. The core of our treatment involves music-cueing to help facilitate effective gait technique. This form of treatment was conducted in conjunction with regular physiotherapy treatment techniques in order to provide William with the best outcome possible. Music-cued exercise training has been shown to be an effective and engaging way for individuals with PSP to improve aerobic endurance and retrain gait patterns. Furthermore, it can prove to be an optimal technique for individuals with PSP because the part of the brain responsible for rhythm, are not associated with the basal ganglia which is heavily affected in individuals with PSP.

Physical Therapy sessions consisted of:

• Warm-up (10 minutes)
• Lower limb strengthening (20 minutes)
• Gait training with music (20 minutes)

Warm-up (10 minutes - Choose exercise based on patient’s current presentation)

• Single leg balance each leg 2x20 sec
• Right + Left torso rotation 2x10
• Quad & hamstring stretch 1x20 until feeling of discomfort
• Neck left + right rotation 1x10
• Neck flexion and extensions 1x10
• Heel tapping, hand clapping 1x10

Strengthening plan (20 minutes)

• Scapular squeezes 2x8
• Supported calf raises 2x8
• Knee flexion & extension 3x10 each leg
• Supported sit to stands 3x (to simulate the TUG)

Gait training with music (30 minutes)

• Patients are given a choice of music genre selection ranging from country to pop to classical music, in hopes to increase satisfaction and adherence to the program. Each song choice presented contains a cadence that can be adjusted to the patient's ability level.
• When the music begins, patients listen to the music beats/rhythms and are instructed to perform specific movements on cue with the beat/rhythm heard in the song playing, that relate to walking, or build up to walking techniques, such as knee raises or arm swings.
• Each session, the music can be adjusted to fit the ability of William, and the specific movements performed can be progressed or regress in difficulty.

The Use of Innovative Technology for Individuals with PSP

Innovative technology is being used more frequently as we begin to better understand Progressive Supranuclear Palsy as a disease, and continue to determine the best options for treatment. One example of an innovative technology used for patients with PSP in recent history is the use of gaming systems to facilitate increase in motor control, awareness and improvement in gait patterns. In the specific case of William, the use of this gaming system, specifically the XBox Kinect, several activity-based games could be chosen, therefore tailoring the treatment to the patient. Furthermore, each activity based game can be adjusted in difficulty, and potentially mimic functional tasks based on the goals of the patient. In William’s case, we could use XBox Kinect to improve gait, improve cardiovascular health, and potentially aim to get him walking his dog again, through virtual dog walking games. However, it is important to note that there are some challenges to incorporating these types of technological tools into intervention plans. First, it is important to understand that innovative technology is still a relatively new approach to treatment in individuals with PSP, therefore for patients who are considered elderly, it may present a challenge for them to understand treatment and adhere to the program in general. Furthermore, the platform of XBox Kinect has recently stopped receiving support from its original developer for the development of its product. Although XBox Kinect can facilitate physical activity in a variety of different populations, there is question on how tailored an intervention program can be, especially in clinical practice, if a decreased number of games and updates are being made. Regardless, these challenges can be mitigated by providing an educational seminar to patients interested in XBox Kinect therapy so that they can better understand their treatment options and the technology itself. Additionally, there are other gaming systems that support exercise-based treatment that are consistently being updated. It is possible that if a clinical environment does not deem XBox Kinect to be suitable for use in clinical practice, another platform such as Playstation VR can be used.