Physiotherapy Management of Middle Stage Amyotrophic Lateral Sclerosis (ALS): A Case Study

Original Editor - Tevin Truong Top Contributors - Tevin Truong, Kevin Duong and Emma Bonafiglia

Abstract

This fictional case study involves a middle-aged male who was diagnosed with Amyotrophic Lateral Sclerosis (ALS) that is classified as middle stage [1]. The case study documents the rehabilitation process from initial assessment to current functional status, while increasing awareness of the clinical presentation and progressive nature of the disease. The case study discusses the therapeutic interventions used to aid in achieving the goals and overcoming challenges faced by the patient. Overall, the patient was able to increase his confidence, autonomy, and prognosis acceptance through the use of therapeutic interventions.

Introduction

Case Background

John East, a 52-year-old Caucasian male, was referred to physiotherapy for evaluation of weakness and difficulty with walking. John was diagnosed with ALS by his neurologist 7 months prior to the initial assessment appointment. He has experienced progressive weakness and atrophy of his arms and legs, making it difficult for him to ambulate stairs and making his job as a mail courier increasingly difficult. John also noticed the development of involuntary twitching of his arm and leg muscles and also experiences painful cramping, specifically in his legs. 

Similar Literature

This article, published in the Muscle and Nerve Invited Review, describes what ALS is and the different rehabilitation approaches using multidisciplinary care. It describes three case studies that represent the early, middle, and late stages of ALS and how rehabilitation changes throughout the progression of the disease. While affirming that a multidisciplinary rehabilitation team is well positioned to make a positive impact on ALS patients, it also acknowledges that ALS is a complex, evolving disease that requires the conduction of more research. [2]

Purpose of the Case Study

To highlight the role of physiotherapy in middle stage ALS. Use of physiotherapy interventions can help maximize patient function, mobility, independence, and quality of life [3]. Needs and problems to be addressed:

  • Weakness and fatigue
  • Pain
  • Respiratory dysfunction (dyspnea due to weakness in ventilator muscles)
  • Decreased range of motion

Challenges with Diagnosing and Managing ALS

  1. ALS is a progressive disease that presents with a constellation of symptoms that vary drastically between individuals [4]. It is important to be educated on the trajectory of ALS and apply the appropriate interventions at each stage [4].
  2. ALS has a high degree of patient and caregiver burnout and needs to be addressed using a multidisciplinary team approach [3].
  3. There is no definitive test to diagnose ALS [5]. ALS is diagnosed using a combination of subjective measures (ex. symptoms, family history), objective measures (ex. physical and neurological examination), and laboratory tests (ex. EMG, MRI, blood samples) [5].

Client Characteristics

Personal Information

A 52-year-old caucasian male named John East. John lives with his wife, Sherry, in a bungalow in Kingston, ON. John has two adult children, who are both away from home attending university. They are only able to visit during summer and winter holidays. John was able to continue working as a mail courier during the early stages of his ALS diagnosis, but has since taken a leave of absence from his job due to painful muscle spasms and difficulty walking. John enjoys socializing with family, golfing and fishing with his friends, and walking his dog Hank down by the creek behind his property.

Condition

ALS is a progressive neurodegenerative disease that affects the upper and lower motor neurons of the brain and spinal cord, resulting in a loss of muscle control. Currently, there is no known cure for ALS [4][6].

Reason for Referral

To evaluate John's muscular weakness and gait difficulties.

Examination Findings

Subjective

During the initial assessment, John’s affect is friendly and cheerful. He states that he’s had a good experience with the healthcare system since his diagnosis 7 months ago, despite the dreary prognosis for most ALS patients. John hopes to recover some of his balance and walking so that he can play golf with his friends again. He states that they usually walk the course but since his diagnosis he had to use a cart and eventually stop playing, which has been frustrating. John says his clothes don’t fit the same anymore and some days he’s too weak to dress himself. John also states he’s been feeling sad because he has to rely on his wife Sherry, to do most activities of daily living. He feels the pressure to “get better” and contribute to household chores and wants to be able to walk his dog independently again. He states that he hopes physiotherapy can help him return to some of his favourite activities and slow down the progression of ALS [3][7].

Past medical history: Type II diabetes that is well-managed with Metformin, no other significant past medical history.

Social history: lives in a bungalow with spouse and dog. Has two adult children who are unable to visit frequently, no other family in town. Enjoys spending time participating in outdoor activities with friends and dog.

Occupation: currently on leave of absence from job as mail courier due to painful muscle spasms and difficulty with walking.

Subjective outcome measures administered:

Objective

General observation:

  • John has visible fasciculations of quadriceps bilaterally [1][7].
  • There is suspected global muscle wasting because John says his clothes don’t fit the same [1].
  • There is moderate muscle atrophy in the interossei regions of John’s hands and feet, more so in his feet than hands [1].

Speech: no significant findings.

Active range of motion (AROM) measured using goniometry. Findings include:

  • Moderate decrease in all major joints.Most notable decreases in:
    • Shoulder flexion = 120°
    • Shoulder abduction = 90°
    • Hip flexion = 85°
    • Knee flexion = 100°
    • Bilaterally ankle dorsiflexion = 5°

Global strength measured using manual muscle testing (MMT). Findings include:

  • Moderate muscle weakness globally, most notably in:
    • Shoulder abduction = 3/5
    • Hip flexion = 3/5
    • Knee flexion = 3/5
    • Bilateral ankle dorsiflexion = 2+/5

Hand strength measured using hand-held dynamometer. Findings include:

  • Lower than average grip strength [10].
    • R = 25 kg; L = 27 kg

Tone measured using passive range of motion (PROM) and the Modified Ashworth Scale [11]. Findings include:

  • Increased tone in global muscles [11].
    • UE = 1+; LE = 2

Sensation measured using sharp/dull and hot cold testing. No significant findings.

Reflexes:

  • Positive Babinski’s sign bilaterally.
  • Positive Clonus Test bilaterally.
  • Hyporeflexia in LEs and UEs.
  • Negative Hoffman’s sign.

Gait measured using Timed Up and Go Test (TUG) [12].

  • John performed the TUG independent of any gait aids.
  • Observation of John's gait reveals short, shuffling steps, decreased single leg support time, and increased difficulty/slowness turning around the cone. John may benefit from using a gait aid. There was also noticeable foot dropping/slapping bilaterally.
    • TUG score = 21 seconds

Balance measured using the Romberg Test [13].

  • John was able to complete the first task with feet together and eyes open = 30 seconds reached
  • John was unable to maintain balance with feet together and eyes closed = 9 seconds reached before needing therapist support

ALS Functional Rating Scale Revised (ALS-FRSR) which is a test used to measure the activities of daily living specific for ALS patients [1]. The ALS-FRSR is scored using 12 activities of daily living measured with a 5-point scale (0 = unable, 4 = little to no difficulty). The total score is 48 and a higher score indicates more independent functioning [1].

John scored 33/48 indicating moderate difficulties with activities of daily living.

Clinical Hypothesis

PT Diagnosis

  • The patient presented with moderate global muscle weakness, a moderate decrease in range of motion in all major joints, and a lower than average measure of grip strength, which acts as a valid indication of overall strength [14]. This was affecting his functional independence and ability to perform activities of daily living. The patient tested positively for hyporeflexia, hypertonia, and complained of fasciculations and painful muscle cramping, all of which are consistent with the progression of ALS [1]. He found his gait and balance deficits unsettling and disabling, as they are essential components to independent functioning.

Problem List

Progressive muscular weakness + atrophy in extremities.

  • Unable to dress himself independently.
  • Clothes are too big.

Painful cramping in L/E + general pain. NRPS score = 4/10.

Difficulty with ambulation and climbing stairs.

  • Inability to walk dog independently or ambulate safely around the home.
  • TUG score = 21 seconds

Unable to work.

Difficulty balancing in the shower.

Respiratory dysfunction (dyspnea due to weakness in ventilatory muscles).

Decreased ROM.

Feels pressure to “get better” quickly.

Moderate depression.

  • PHQ-9 score = 13/27.

Hypertonia.

Hyporeflexia.

ALS-FRSR score = 33/48 moderate difficulty with activities of daily living.

Intervention

Patient Centred Treatment Goals

Reduce dyspnea with respiratory training techniques.

Ambulate independently and safely.

Maintain balance in shower.

Improve muscle weakness.

Improve range of motion.

Management Program

Below is a copy of John's prescribed exercise program targeted towards his physiotherapy goals [15][16][17][18][19][2].

John East's exercise program

Outcomes

Degree of Improvement/Change Post-Treatment

The diaphragmatic breathing and inspiratory muscle training slightly reduced the patient’s symptoms of dyspnea by preventing further weakening of ventilatory muscles. The patient felt more in control, aware, and confident in his balance abilities after performing internal and external perturbation training. It is possible that the kitchen counter exercises were able to slow further muscle wasting and atrophy as the patient did not present with any increase nor decrease in muscle strength. Lastly, when including the use of a gait aid, he was able to improve his TUG score by two seconds. Although the interventions did not show a huge improvement in pain or strength scores, the patient reported to feel more confident in his abilities and enjoyed the social interaction associated with the therapy.

Discharge plan/Referral to Other Healthcare Practitioners

Maintaining current functional abilities is an important component in slowing the progression of ALS, therefore the patient will not be discharged from the physiotherapy program at this time. Currently, the patient has been referred to an occupational therapist, a dietician, and a psychologist. The occupational therapist will ensure that the patient’s current living situation is appropriate for his condition, the dietician will work on nutrition education to prevent further muscle wasting and promote overall health, and the psychologist will help manage the patient’s moderate depression and provide support to the patient and the family during this time. In the future, it is likely that the patient will need to expand his health care team to include the roles of a speech language pathologist, a respiratory therapist, and a nurse and/or a palliative care team.

Discussion

ALS is a progressive neurodegenerative disease with no known cure. Physical therapy interventions for treating patients with ALS should aim to delay the progression of the disease [2]. Physical therapists should have shared, patient centred goals when developing treatment plans and help them maintain as much independence as possible [1]. As the disease progresses, an interdisciplinary approach should be taken to address the multifactorial nature of ALS [1]. For example, occupational therapists and speech language pathologists can both be referred to in order to maintain independence and quality of life. Furthermore, utilizing a biopsychosocial approach can help to tackle the multidimensional effects of ALS. Using validated outcome measures is an integral step for clinicians to assess disease progression and treatment effects. Throughout the rehabilitation process, it is critical to have clear communication, maintain a patient centred approach and to educate the patient.

Self-Study Questions

ALS affects which type of nerve(s)?

  1. Sensory
  2. Motor
  3. 1 and 2
  4. None of the above

What health care professional does not play an important role in the care of someone with ALS?

  1. Occupational Therapist
  2. Dentist
  3. Personal Support Worker
  4. Speech Language Pathologist
  5. All of the above

How is ALS diagnosed?

  1. A combination of subjective measures, objective measures, and laboratory tests
  2. The ALS diagnostic tool kit
  3. EMGs and muscle biopsies exclusively
  4. Presence of unilateral fasciculations only
  5. None of the above

References

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  2. 2.0 2.1 2.2 Majmudar, S., Wu, J. & Paganoni, S. Rehabilitation in Amyotrophic Lateral Sclerosis: Why it Matters. Muscle Nerve 2014. 50: 4-13.
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  6. Mayo Clinic. Amyotrophic Lateral Sclerosis (ALS). Available from: https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022 (accessed 6 May 2020).
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