Stiff Person Syndrome: A Case Study

Abstract

The following is a fictitious case for educational purposes involving Mr. Reed, a 75 year old male with Stiff Person Syndrome (SPS) who was referred for physical therapy. The patient exhibits balance and mobility problems, lower extremity stiffness and painful cramps, and Mild plantar flexor contracture. Physical therapy includes balance and gait training strengthening and flexibility exercises soft tissue release and heat patterns. Following the therapeutic intervention, Mr. Reed’s range of motion (ROM) and strength improved, as did his score On the Activity Specific Balance Confidence Scale (ABC Scale), Rivermead Mobility Index (RMI), Berg Balance Scale (TUG) and Trunk Impairment Scale.

Introduction

Stiff person syndrome (SPS) is a rare neurological disorder diagnosed in approximately one in a million individuals [1]. It is strongly associated with autoimmune diseases such as type 1 diabetes [2], affects the central nervous system, and currently has no satisfactory treatment. [3].Low This occurrence may lead to limited available research on the effectiveness of physical therapy interventions. It is diagnosed as a disease that reduces the patient’s quality of life and general mobility. [2]

Clinical Features

In individuals with SPS, postural stiffness and fluctuating muscle spasms with excessive lordosis of the trunk muscles are commonly present [4] [5]. These symptoms are usually insidious in onset, with increased tension in the proximal lower extremities and paraspinal muscles of the abdominal muscles [4] [5] The hallmark feature of this disorder is co-contraction of agonist and antagonist muscles and persistent involuntary discharge of motor units [2]. Anticipatory anxiety and task-specific phobias due to sudden startle responses can also be seen. Patients with SPS may also experience intermittent cramps Especially in the lower extremities where there may be associated triggers [4] [5]. Spasticity may cause the patient to fall in a tin man-like position [5]. Other neurologic symptoms include gaze palsy, nystagmus, increased reflexes, and paroxysmal dysautonomic crises. [3]

Differential Diagnosis

Multiple sclerosis

Parkinson’s disease

Mental disorders secondary to phobias and emotional triggers [4].

Here, we highlight the posture of individuals with SPS. Note plantar flexor contracture and varus. Unfortunately, the hyperlordotic posture is not described. [5]

Review Of literature

In 2011, Hegyi reported the case of a 24-year-old woman who had been diagnosed with SPS for about a year. In Hegyi’s report, patients attended 17 sessions during 15 weeks of outpatient physiotherapy. The main concerns of patients are pain, muscle spasms, abnormal gait and range Motor (ROM) deficits, especially in the left lower extremity. In this case, the patient’s physical therapy intervention is aimed at treating these problems. Treatment includes: Therapeutic Ultrasound Soft Tissue Mobilization Manual Stretching Therapeutic Exercises and Adaptations Ankle Foot Orthosis. These interventions were successful and improved the symptoms that emerged.

In another case reported by Potter in 2006, a 33-year-old man was receiving 10 days of inpatient physical therapy. He had been diagnosed with SPS for three years prior to the documented physical therapy intervention. The man showed decreased static and dynamic balance, unable to maintain Standing in a standing position for more than five seconds without handheld support and requiring the use of a walker to walk short distances [5]. The man usually uses a wheelchair for any attempts to get around. Patient presents with axial rigidity, hyperlordosis and bilateral lower extremity Stiffness [5]. When examining his past medical history, it became apparent that he suffered from anxiety, depression, substance abuse, and chronic bronchitis. He is also in pain, 4 out of 10 when sitting quietly, sometimes 10 out of 10 depending on the situation.

Patients received an average of 45 minutes of physical therapy per day for 10 days, with treatment focused on improving functional independence and basic motor skills. Physical therapy includes therapeutic exercise and functional retraining. Patients received a home exercise program that focused on stretching His lower body and flexion-based torso workout. Despite the longer physiotherapy session, the patient requested early discharge, and his function improved even with the shorter session. In this case, the patient appears to have more advanced instances of The SPS is compared to the fictional patient case we will explore, but the goals and physical therapy management remain similar. We can use the case of Mr. Reed to support the efficacy of physical therapy in patients with SPS, which has been questioned in the past [4].

In the fictional example below, our patient is named Mr. “Reed”. Mr. Reed is a 75-year-old male who was diagnosed with classic SPS about a year ago. Mr. Reed had increased axial stiffness and bilateral lower extremity stiffness, with his right side more affected than to his left lower extremity. Mr. Reed presented with hyperlordotic low back pain, hypertonic trunk musculature, and hypertonic lower extremity musculature. Although SPS is a rare neurologic disorder and there is limited evidence regarding the efficacy of physical therapy as a treatment for these two cases The above supports the indication for physical therapy in Mr. Reed’s case. The purpose of this case study was to provide more information and literature on the efficacy of physical therapy applied to patients with SPS in an outpatient setting.

Client Characteristics

Mr. Reed, also known as Mr. R, is a 75-year-old male. He is a retired data analyst who lives with his wife at their home in Kingston, Ontario. Mr. R noticed an increase in falls starting two and a half years ago due to decreased balance (his last fall was around two weeks Before) Gradually worsening and low back pain from a year and a half ago. Over the past year, he has noticed increased pain in his lower extremities with muscle cramps, especially in his left leg. He was referred to a neurologist and diagnosed with SPS. he received Referral to outpatient physical therapy for a fall risk assessment helped his balance and gait control muscle stiffness and spasticity and maintain his independence and quality of life.

Examination Findings

Subjective

• Patient Profile (PP): 75-year-old male with dominant right hand
• History of Present Illness (HPI): Stiff Man Syndrome diagnosed 1 year ago Left plantar flexor contracture (~2 months) Muscle spasticity and progressive rigidity (~1 year) and lower extremity pain (~6 months) Chronic low back pain (~ 1.5 years).
• Past history: type 1 diabetes, hypertension, hyperlipidemia.
• Drugs: Oral diazepam gabapentin [6] and insulin
• Healthy Habits: Occasional alcohol (approximately 2 times per week) for non-smokers.
• Psychosocial: Patient described feeling lonely due to COVID-19 and depressed about diagnosis. For the past 6 months, he has avoided gardening and groceries due to feeling unsteady and fear of falling. He lives with his wife; his son lives 20 minutes away and visits him weekly; his Daughter lives 1 hour away and visits once a month.
• Residential: Bungalow 5 steps of stairs to enter the residence with railings on the right side of the front and rear doors. The bathroom has a stand up shower with railings.
• Previous Functional Status: Able to walk more than 200 m without gait assistance, actively gardening and golfing, performing Activities of Daily Living (ADL) without issue.
• Current functional status: low confidence when walking outdoors, stiffness, instability and cramping pain in both lower extremities, which prevents him from pursuing his hobbies (gardening, watching golf sailing).
• Imaging/Diagnostic Tests: MRI and x-rays (all negative) blood tests (anti-GAD 92.5 units/mL) nerve conduction studies (no abnormalities) for chronic low back pain.
• Precautions/contraindications: None
• Chief Complaint: Impaired balance control resulting in stiffness and painful cramps in the lower extremities, especially the left leg, limiting his ability to perform daily activities.

Objective

General

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

Posture

• Thoracic kyphosis
• Hyperlordosis

Gait

• The slow, wide gait appearance of walking with stiff legs
• Left ankle stiffness, lack of dorsiflexion with gait
• Max walking distance 200m  
• Usually walks with 4WW outdoors and single crutch indoors
• Shuffling
• Decreased arm swing

Tone

• Increased tension in the lower extremities Increased tension in the left side more
• Mild plantarflexion contracture and insufficient knee extension

AROM

• Knee Flexion (Right 135° Left 120°)
• Knee Extension (Right -10° Left -20°)
• Ankle dorsiflexion (10° right left -5°)
• Ankle valgus (10° right 5° left)
• All other ROM within normal limit (WNL)

PROM

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

Strength

• Right lower extremity (4/5)
• Left lower extremity (3+/5)

Sensation

• Intact upper and lower extremities

Neurological testing[7]

• Myotomes: All lower extremities 4+ Upper extremities normal
• Dermatomes:(normal)
• Reflexes:Hyperreflexia

Self-Reported Outcome Measures[8]

• Activity-Specific Balance Confidence Scale (ABC)
  • Score: 50% (moderate level of physical performance)
• Rivermead Mobility Index (RMI)
  • 7/15

Outcome Measures [5][8]

• Berg Balance Scale (BBS)
  • 40/56
• Timed Up and Go (TUG)
  • 30 seconds
  • 0.5m/s gait speed
• Trunk Impairment Scale
  • 10/23

Clinical Impressions

The patient is a 75-year-old man with mild SPS. His subjective interview indicated that at his initial diagnosis he was still able to participate in his regular ADLs, but over the past six months due to feelings of instability, progressive stiffness, and fear decline. The main clinical findings on objective assessment showed left foot lordosis with mild plantar flexor contracture, lack of ankle and knee mobility, especially left ankle dorsiflexion and knee extension were affected. He also presented with chronic low back pain, stiff pain Muscle spasms in the lower extremities (the left side is more affected than the right), and strength in the trunk and lower extremities is decreased.

His self-reported ABC score was 50%, indicating some confidence in his balance and fear of falling [9] [10]. This suggests that the main reason for any lack of involvement in his ADLs is his lack of confidence and risk of falls [9][10].

The patient’s lower body and trunk strength may affect their balance. His trunk impairment score was 10/23, indicating poor static and dynamic balance in the sitting position [8][11]. His BBS score is 40, indicating that he is at increased risk of falling (<45 indicates increased risk of falls) [12][13]. His TUG score was in the mid-30s, indicating that he was at increased risk for falls (normal for his age was 9s +/- 3s) [14][15][16]. His RMI score is 7/15; higher scores will demonstrate better mobile performance [17][18]. These findings emphasize the need Balance gait intervention and reassessment of appropriate gait aids were included in his treatment plan to address his overall increased risk of falls.

Mr. R was in fair health prior to his SPS diagnosis; he had type 1 diabetes, high blood pressure, and high cholesterol. However, he does participate in some activities and is relatively active for his age. For six months, his activities were progressively rigid and Lower extremity spasm pain, more severe on the left side than the right side. It is believed that Mr. R would be an ideal candidate for physical therapy to help improve his confidence balance and strength while reducing his muscle stiffness. In addition, Mr. R’s care should extend to to a multidisciplinary approach, which will be explored later in this case.

Problem List

1. Decreased balance
2. Decreased confidence and increased fear of falling
3. Hypertension
4. High cholesterol
5. Type 1 diabetes
6. Lower back pain
7. Lower extremity pain (due to muscle spasms)
8. Decreased left ankle dorsiflexion and eversion ROM
9. Mild ankle plantar flexor contracture
10. Reduced left knee flexion and knee extension ROM
11. thoracic kyphotic posture
12. Hyperlordosis in lumbar spine
13. Decreased lower extremity strength
14. Rigidity
15. Hypertonia
16. High Anti GAD levels
17. Abnormal gait

Short term goals

1. Increased his ABC score from 50% to 64% in 6 weeks
2. Reduced his low back pain from 3/10 at rest to 1/10 in 5 weeks
3. Reduced pain from muscle spasms in left leg from 7/10 to 4/10 and right leg from 4/10 to 2/10 in 5 weeks
4. Increased his left ankle dorsiflexion ROM from -5° to 0° in 6 weeks
5. Increased his left ankle eversion ROM from 5° to 8° in 6 weeks
6. Increased his knee extension AROM from -10° to -5° on the right side and from -20° to -15° on the left side in 5 weeks
7. Increased his left knee flexion ROM from 120° to 127° in 6 weeks
8. Improved his lower body strength from 4/5 to 5/5 on the right and 3+/5 to 4/5 on the left in 6 weeks
9. Increased his TUG score from 30 to 18 in 6 weeks
10. Improve his Trunk Impairment Score from 10 to 15 in 6 weeks

Long term goals

1. Increased his BBS from 40 to 47 in 16 weeks
2. Increased his RMI score from 7 to 12 in 16 weeks
3. Walked around the block with his wife at 16 weeks and had improved gait mechanics
4. Garden for 30 minutes a day in 16 weeks

Treatment Plan

Frequency Intensity Time Fundamentals Education Role of PTP Pain Management Environment Modification Appropriate use of gait aids At his first appointment and subsequent check-ups N/A Ongoing list of questions 6 7 17 to ensure the patient understands how the physical therapist’s role is manage his Symptoms and what needs to be done to see improvement Balance training [19] Stand shoulder-width apart on a foam surface with eyes open seconds) at Clinic: 2 times per week for first 8 weeks, then progress to 1 time per week Home: 2 times per week at home for first 8 weeks, then progress to 3 times per week at home as tolerable, but aggressively try to break limit Initially daily 15 minutes, then gradually increase to 30 minutes Question list 1 2 Add Mr. R’s Have confidence in his balance and reduce the risk of him falling. Gait[5][20] Nordic Pole Walking Parallel Bars Walking Forward Backward Lateral Over Barriers At Clinic: 2x/week, then progressing to 1x/week At Home: Nordic Pole Walking 3x/weekly, then progressing to 4 times a week at home light intensity 10 Minutes initially progressed to 15 minutes Question List 1 2 17 Improve Mr. R’s gait mechanics walking endurance as well as increase his confidence and reduce the risk of falls. Strengthening [5][4][21] Kitchen Sink Exercise Isometric Quadriceps Calf Hamstring Posture Strengthening/Exercise Control Sit Stand Parallel Bar Steps Kneel Stand Versa At Home: 3x/week At Clinic: 2x/week Then Progress To 1x/week (Sit Stand Steps & Kneel Stand Stand) 65% of 1RM/lightintensityLight intensity3 sets of 12 reps2 Set of 15 Repetitions Question List 11 12 13 15 Postures Strengthening and motor control are key parts of gait and balance, so taking steps to improve Mr. R’s posture/motor control will help improve his gait and balance. Further strengthening of the lower extremities will improve balance and gait, which will allow Mr. R recovered his ADLs at a faster rate. ROM [5][4]TrunkHipKneeExtensionFlexionAnkleDorsiflexionGeneral ROM with fixed bikeAt home:3x/week progressed to 6x/weekIn clinic:2x/week then moved to 1x/week (fixed bike) Start with light pressure then progress moderately. stretch to Slight discomfort until you feel a stretch. Light intensity 30 seconds, static stretch repeated twice daily, 5-10 minutes before ROM practice in his ADLs.Additional treatments [4][22] soft tissue mobilization of lower back and lower limb muscles joint mobilization heat therapy of lower limb joints clinically: 2x/week moved to 1x/weekheat therapy as needed at home5-10 PassesAs tolerableOngoing10 minutes 3 times a week Problem checklist 8 9 10 14 15 Improves Mr. R’s overall mobility by improving his muscle extension and joint mobility.

NOTE:

• The following exercises were not done in one session; they were used throughout the 16-week treatment as the patient’s condition progressed.
• These exercises should be performed with caution to prevent muscle cramps.

[23][24]

Interprofessional Health Team

[25]

It is important to include a variety of healthcare professionals in Mr. R’s plan of care. For example, his family physician played a crucial role in managing his acute and chronic illnesses and identifying other underlying conditions he may have [26]. family doctor is one of them First point of contact within the healthcare system and can provide referrals to other specialists as needed. Neurologists are also important in Mr. R’s care because they specialize in diagnosing, treating or managing Stiff Person Syndrome. They can prescribe appropriate medication to manage his symptoms Tests, such as nerve conduction studies and electromyography, to assess nervous system integrity as the disease progresses and provide appropriate interventions to manage the disease when needed [27].

In addition to his family physician and neurologist, there are other professionals that need to be involved. Given Mr. R’s age, high cholesterol and type 1 diabetes, his referral to a nutritionist was deemed necessary. Considering Mr. R’s comorbidities and complexities Cases require professional advice. A nutritionist can provide important advice to help Mr. R self-manage his diabetes and high cholesterol, which can help reduce common long-term complications of diabetes and improve his quality of life [28].

Social workers can also be included in his care plan because they can provide resources and services to overcome barriers to psychosocial physical and emotional health [29][30]. In Mr. R’s case, a social worker can direct him to appropriate resources because he Reported feeling socially isolated due to COVID and frustrated with his diagnosis. Social workers can also help implement interventions to prevent the caregiver burden that Mr. R’s wife may experience if his condition worsens.

An occupational therapist could also be included to help improve Mr. R’s quality of life and allow his ADLs to become more independent. They can provide an assessment to determine the need for adaptive equipment or home modifications as his physical abilities change [31]. In addition they can Determine if appropriate adjustments should be made, or if new skills are to be learned, allowing Mr. R to pursue his gardening hobby, go grocery shopping, or perform his ADLs with ease [31]. Occupational therapists can also provide education and guidance to Mr. R’s spouse on how to Provide accommodation and care when needed [31].

Outcome

Based on the initial evaluation, it was determined that Mr. R should undergo four months of physical therapy to address his deficits and implement a home exercise program. Twice a week for the first eight weeks and once a week for the remaining eight weeks. he was reassessed at the end of the year His six-month treatment to determine how to continue ongoing treatment.

Key results include: Increased ROM Improves overall balance and confidence Balance controls tone gait mechanics and pay reduction/management. Although he is now more confident gardening and walking around the block, he still has mild balance impairment, thoracic kyphosis, and mild Liquidity issues. His current grades are:

• ABC: 70%
• RMI: 10/15
• BBS: 47
• TUG: 13s
• Trunk Impairment Score: 18/23

After 16 weeks of prescribed treatment, Mr. R’s BBS score improved to 47/56, indicating minimal detectable changes, and a reduction in the risk of falls [12]. His ABS score went from 50% to 70%, which still puts him in the middle of the pack category. However, this change indicated a minimally important difference (range 11-13%), and he was no longer at risk of falling as his score was above 67% [9][10]. His TUG score improved to 13s, further emphasizing the lower risk of falls and greater functional independence [14][15][16]. and his RMI The score improves to 10/15, indicating better mobile performance [17][18].

Overall, Mr. R achieved all of his short-term goals and most of his long-term goals during the 16-week treatment period. Mr. R’s RMI score improved significantly, but fell short of his second long-term goal. decided that at the end of the 16 weeks, his frequency of visits would be reduced to one month, and set additional long-term and short-term goals. A one-month follow-up visit is planned to reassess and provide progress on his home exercise treatment plan; this is a progressive disease and the focus remains on maintaining and continuing communication with Mr. R’s interprofessional health team.

Discussion

Mr. R (75 years old) presented with mild stiff-man syndrome. His initial evaluation revealed that he had lordosis, mild left plantar flexor contracture, decreased mobility in the ankle and knee (particularly on the left side), lower extremity stiffness, and painful muscle spasms (the left side was more affected). Our evaluation also indicated that he had chronic low back pain and decreased trunk and lower extremity strength. Mr. R was independent in his activities of daily living, but became more dependent due to his rigid muscle spasms and avoidance of physical activity due to his fear of falling. Therefore his treatment plan is Designed to reduce his stiffness first, while increasing his balance control, lower body ROM strength, gait mechanics and balance confidence. Treatment will then progress to maintaining function, and as SPS is a progressive disease, the ultimate goal is to ensure that Mr. R can continue to Carry out his daily activities and hobbies independently.

Mr. R’s treatment plan was particularly relevant to his goals and his SPS diagnosis, but due to the rarity of SPS, little is known about the optimal approach to treatment, especially since individual presentations vary. Therefore our treatment plan is based on previous case studies [4][5] His targets were mainly complaint assessment results as well as personal experiences of treating SPS patients. Mr. R’s main concerns were his increased tendency to fall, lower extremity stiffness, painful cramps, and lack of confidence in his ability to balance. Therefore, most of our The treatment plan focused directly on improving his balance and gait, increasing his lower extremity strength and ROM to help prevent future falls. Aspects of the treatment plan mimicked the BBS (such as 360-degree rotations and picking up items from the floor) as Mr. R had programmed Aims to improve scores on various outcome measures. Additionally, the focus was on strengthening and stretching Mr. R’s trunk and lower extremities bilaterally to reduce tonic spasticity and help him achieve his goal of walking with his wife.

One aspect of healing related to his goal is to have him go from kneeling to standing and vice versa. Going from kneeling to standing not only develops strength and balance, but also allows Mr. R to be more effective in many aspects of gardening (one of his hobbies) [32]. also The ability to go from kneeling to standing helped Mr. R feel more confident getting back up when he fell, which gave him more confidence to participate in his hobbies and other physical activities [33] [32].

The outcome measure chosen for Mr. R was based on previous case studies [5] [4] [8] as well as personal experience. None of the outcome measures selected for this case study were specific to SPS, but most of them were valid in the older population. Exceptions are trunk injuries A score created and validated for stroke patients. Based on other case studies reported by SPS [8], it was chosen as the measure of Mr R and allowed monitoring and comparison of changes in his trunk control [8] [11] from pre- and post-treatment. Monitoring trunk control changes is a An important aspect of treatment, as trunk control plays an important role in balance and postural control [34][35] – this is an area where Mr. Reed has had problems. RMI was another exception chosen for Mr R because it was based on other reported SPS case studies [8] but was created and validated for stroke patient. The RMI allowed self-reported measures of Mr. R’s mobility, including tasks related to balance and gait, as well as other more functional activities [17][18].

Throughout the case study, it was determined that Mr. R’s balanced gait posture and physical therapy management of pain were effective. It must be noted that patients’ positive attitude towards physical therapy may have contributed to this result. another contributing factor The success of the patient’s physical therapy was that his condition was considered mild at the time of diagnosis. Considering the results, physiotherapy should obviously be applied to SPS patients as it can help manage and improve their symptoms [4] [5].

Overall, this case attempts to demonstrate therapeutic avenues for patients with mild SPS. Each SPS patient may present with different symptoms [4] [5], thus requiring a different individualized treatment approach to effectively manage their condition. This case study is unique in that The average age at diagnosis of SPS usually occurs around the age of 40 [4]. At age 75, Mr. R had only been officially diagnosed for one year, making his case uncommon compared to other case studies of younger individuals with similar diagnoses. At Mr. R’s age More consideration must be given during the physiotherapy management of his treatment. Given his age diagnosis and items, if Mr. R had no symptoms other than a mild case of SPS, the prognosis might not be as positive. It should be noted that as the disease progresses Physiotherapy in this entire case is not a cure, but is intended to improve and maintain Mr. R’s function and independence. As research in this area advances, other treatments and outcome measures may be more appropriate and applicable to the described patients.