Stiff Person Syndrome: A Case Study

Original Editor - Cassandra Bodrucky, Kathleen Li, Benjamin Prance, Joseph Balasundaram as part of the Queen's University Neuromotor Function Project Top Contributors - Cassandra Bodrucky, Nupur Smit Shah, Kathleen Li, Joseph Balasundaram, Benjamin Prance and Kim Jackson

Introduction

Stiff Person Syndrome (SPS) is a rare neurological condition in which the rate of diagnosis is approximately 1 diagnosis for every 1 million people . The low occurrence of the condition may contribute to there being limited research in regard to the effectiveness of a physiotherapy intervention. In individuals who have SPS it is typical to present with a hyper-lordodic posture, rigidity, and fluctuating muscle spasms . The symptoms typically have an insidious onset with increased tone starting in the paraspinals, proximal lower extremities, and abdominal muscles . Individuals with SPS may also present with episodic spasms particularly in the lower limbs that may also have an associated trigger . The spasms may also cause the patient to fall like tin-man . The typical differential diagnosis for SPS may differ from patient to patient as symptoms vary but it may include: Multiple sclerosis, Parkinson’s disease, or psychiatric disorders secondary to phobias and emotional triggers.

In a case reported by Hegyi in 2011, a 24 year old woman who had also been diagnosed with SPS for approximately one year. In Hegyi’s report, the patient attended 17 sessions across 15 weeks of outpatient physio. The chief concerns of the patient were pain, muscle spasms, gait anomalies and ROM deficits particularly in the left lower extremity. In this case, the patient’s physiotherapy interventions were guided to treat for pain, muscle spasms, gait and range of motion. The particular interventions used were therapeutic ultrasound, soft tissue mobilizations, manual stretching, therapeutic exercise, and fitting for an ankle-foot orthoses. It was found these interventions were successful and improved the symptoms treated.

In a case reported by Potter in 2006, a 33 year old man was being treated in in-patient physiotherapy for 10 days. This man had been diagnosed with SPS for 3 years prior to the recorded physiotherapy intervention. The man presented with decreased static and dynamic balance in standing that was indicated by requiring a rollator walker to ambulate short distances and only being able to maintain a standing position with no handheld supports for 5 seconds . When required to ambulate, the man typically used a wheelchair. The patient also presented with axial rigidity, hyper-lordosis, and bilateral lower extremity rigidity . During the patients past medical history it was indicated that he had suffered from anxiety, depression, substance abuse and chronic bronchitis. The patient also had pain that ranged from 4/10 in quiet sitting that could reach 10/10 depending on the circumstances.

The patient averaged 45 minutes a day of physiotherapy for 10 days with treatment focused on improving functional independence and basic motor skills. The physiotherapy consisted of therapeutic exercises and functional retraining. The patient was given a home exercise program that focused on stretching his lower extremities and flexion based truncal exercises. Although the physiotherapy treatment was scheduled for a longer period, the patient requested early discharge and it was still found there were improvements in his functioning. In this case by Potter in 2006 the patient appears to have a much more progressed case of SPS compared to our patient who will be explored below. However, the goals and physiotherapy management are still similar and will apply in our case. We can use this case to support the efficacy of the physiotherapy techniques used in the case of a patient with SPS which has been questioned before .

In the case to be discussed we will refer to our patient as “Mr. Reed”. Mr. R is a 75 year old man who was diagnosed with classical SPS approximately one year ago. Mr. R presents increased axial stiffness, and bilateral lower extremity stiffness, with his right side more affected compared to his left lower extremity. Mr. R presents with hyper-lordosis, low back pain, hypertonic trunk musculature and hypertonic lower extremity musculature. Although SPS is a rare neurological disorder and there is limited evidence regarding the efficacy of physiotherapy as a treatment, the two cases above support the indication of physiotherapy for Mr. R’s case. The objectives of this case study to provide more information and literature regarding the efficacy  of physiotherapy applied to an individual with SPS in an outpatient setting.

Client Characteristics

Mr. Reed is a 75-year-old male. He is a retired data analyst, who lives at home with his wife in Kingston, Ontario. Mr. R began noticing he had decreased balance two and a half years ago with increased amounts of falls (last fall ~two weeks ago) which has progressively gotten worse with low back pain starting a year and a half ago. Within the last year, he noticed increased pain in his lower extremities with muscle spasms particularly affecting the left leg. He was referred to a neurologist and diagnosed with SPS. He received a referral for outpatient physiotherapy to perform a falls risk assessment, help with his balance and gait, manage muscle rigidity and spasms, and maintain his independence and quality of life.

Examination Findings

Subjective

• Patient Profile (PP): 75 y/o male, right hand dominant
• History of Present Illness (HPI): diagnosed with stiff person syndrome 1 year ago, left plantar flexor contracture (~2 months), muscle spasms, and progressive rigidity (~ 1 year) and lower extremity pain (~6 months), chronic low back pain (~1.5 years).
• Past medical history: Type 1 diabetes, hypertension, high cholesterol.
• Medications: Oral diazepam, gabapentin (1), and insulin
• Health Habits: Non-smoker, drinks occasionally (~2 drinks a week).
• Psychosocial: The patient describes feeling lonely due to COVID-19 as well as frustrated with the diagnosis. Over the past 6 months, he has avoided gardening and groceries due to feeling unsteady and having a fear of falling. He lives with his wife and his son lives 20 minutes away and visits weekly where his daughter lives 1 hour away and visits monthly.
• Home: Bungalow, 5 stairs into the home with railings on the right at both front and back door. The bathroom has a stand-up shower with railings.
• Previous Functional Status: Able to ambulate more than 200m with no gait aid, active in gardening and golfing, no issues performing activities of daily living (ADLs)  
• Current Functional Status: Less confident walking outdoors, feeling unsteady, rigidity and painful spasms in both lower extremities prevented him from engaging in his hobbies (gardening, watching golf, sailing)
• Imaging/Diagnostic testing: MRI and X-ray for chronic low back pain (all negative), Blood tests (Anti-GAD body 92.5 units/mL), nerve conduction studies (No abnormalities)
• Precautions/Contraindications: None
• Chief complaint: decreased balance control, increased rigidity and painful spasms in lower extremities particularly in the left leg limiting his ability to do ADLs.

Objective

General

• Vital signs
  • Pulse 80bpm, BP 145/95mm HG, RR 15 bpm
• Pain
  • Visual analogue scale
    • 3/10 constant pain in the lower back
    • 4/10 during painful spasms in the right lower extremity, due to hot weather and stress
    • 7/10 during painful spasms in left lower extremities, due to hot weather and stress

Posture

• Thoracic kyphosis
• Hyper-lordosis

Gait

• Slow and wide gait, appears to walk stiff legged
• Left ankle stiff and lack dorsiflexion, than right LE
• Max walking distance 200m  
• Typically ambulates with a 4WW outdoors and single cane indoors  
• Shuffling
• Decreased arm swing

Tone

• Increased tone in the lower extremities, more increased tone on the left
• Mild plantar flexion contracture, as well as lack of knee extension

AROM

• Knee flexion (right 135°, left 120°)
• Knee extension (right -10°, left -20°)  
• Ankle dorsiflexion (10° right, left -5°)
• Ankle eversion  (10° right, left 5°)
• All other ROM within normal limit (WNL)

PROM

• Knee extension (right -5°, left -10°)
• All other ROM within normal limit (WNL)

Clinical Impressions

The patient is a 75 year old male with mild SPS. His subjective interview indicates that initially in his diagnosis he was able to participate in his regular ADLs but for six months now has avoided doing his regular ADLs due to feeling unsteady, having progressive rigidity and having a fear of falling. Major clinical findings from the objective assessment revealed: hyper-lordosis, mild left plantar flexor contracture, lack of ROM in his ankles and knees. His left ankle dorsiflexion and knee extension are particularly affected, he also presents with chronic low back pain, rigidity, painful muscular spasms in lower extremities (left affected more than right), and decreased trunk and lower extremity strength.

His self reported ABC score was 50% indicating that he is moderately confident with his balance and has a fear of falling . This finding indicates that his lack of participation in his ADLs is mostly due to his lack of confidence and is at a fall risk .

The patient's lower extremity and trunk strength may also be a factor impending his balance. His trunk impairment score was 10/23, indicating that his siting static and dynamic balance is poor . His BBS score is 40 which indicates that he is at an increased risk for falls (<45 indicates increased risk of falls) . His TUG score is 30s indicating that he is at an increased risk of falling (normal for his age is 9s+/- 3s) . His RMI score is 7/15, a higher score would indicate he has better mobility performance . These findings indicate a need to include balance, gait intervention, and reassessment of appropriate gait aids in his treatment plan due to his overall increased risk of falls.

Mr. Reed's health prior to the diagnosis of SPS was fair as he had type 1 diabetes, hypertension and high cholesterol. However, he did partake in some activities and was relatively active for his age. His activities have been limited for six months now by his fear of falling, progressive rigidity and painful spasms in the lower extremities with his left side being worse than his right. It is believed that Mr. R would be a good candidate for physiotherapy to assist with improving his confidence, balance, and strength while reducing his muscle rigidity. Furthermore, Mr. R’s care should extend to a multidisciplinary approach which will be explored later in this case.