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Amyotrophic Lateral Sclerosis: A Spinal-Onset Case Study

Original Editor - Hannah Perreault, Katie Wolters, Jessie Fleming, Jill Hubbard, Rochelle McCoy, Aamira Lokhandwala

Top Contributors - Hannah Perreault, Jessica Fleming, Katie Wolters, Jillian Hubbard, Rucha Gadgil, Aamira Lokhandwala and Rochelle Ann-Marie McCoy  

Introduction

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurological disorder that affects voluntary muscle movement[1]. Specifically, upper and lower motor neurons are destroyed which results in various devastating symptoms such as muscle weakness, speech impairments, and swallowing difficulties[1]. Patients die on average 3-5 years after symptoms onset due to respiratory failure[1]. The current cause and cure remain unknown.

In this fictional case study, a 70-year-old male has been diagnosed with the limb/spinal-onset subtype of ALS six months prior. Client characteristics, subjective and objective examination findings, and a clinical hypothesis are documented throughout the case study. Additionally, a potential physiotherapy intervention is outlined in detail using the latest evidence based research, including verified outcome measures for this population. Primary patient-centred goals include the maintenance of mobility, strength, and participation in meaningful activities despite living with a progressive disease. Throughout this case study, the role of physiotherapists and the importance of a strong inter-disciplinary team is highlighted in the management of ALS.

Client Characteristics

Figure 1

The patient is a 70 year old, right hand dominant, caucasian male named Lou Gherig. He retired twenty years ago after a successful career in professional baseball and now lives with his wife and adult children in a two storey home on the west side of Kingston.

Lou was diagnosed six months ago with ALS by a neurologist at KGH and presented with both upper and lower motor neuron deficits. Specifically, Lou suffers from the limb / spinal onset phenotype of ALS. He was prescribed Riluzole medication which has the potential to increase his lifespan by several months[2]. He does not present with any comorbidities other than hypertension. During the initial physiotherapy assessment, it was noted that Lou has progressively worsening weakness in his upper and lower extremities.

Six main problems that have been identified include: muscle cramping, mild spasticity in the upper extremities, decreased balance, inability to manage stairs independently, struggling to walk his large dog and not being able to partake in painting like he used to, due to fatigue and dyspnea when engaging in activity.

Examination Findings

While there were several problems identified through assessment, the three primary concerns identified by the patient are as follows:

  • Muscle weakness in the musculature of bilateral distal upper and lower extremities
  • Inability to ambulate independently
  • Restriction from painting due to loss of manual dexterity

A common comorbidity that should be screened in all ALS patients is hypertension. Literature shows a strong association between hypertension and ALS, with and increased risk of death occurring if both are present[3]. Therefore, Lou was screened for hypertension, and it was revealed in his past medical history that he does have this co-morbidity.

Clinical Hypothesis

A physiotherapy diagnosis notes decreased overall function due to generalized muscle weakness in the distal extremities, and impaired balance. Body structure, activities, and participation have all been impaired. Given that Lou was previously active, has a supportive family at home, and is financially stable, it is expected that physiotherapy will be able to minimize the impairment from ALS and help to maintain as much function as possible. However, Lou has been experiencing some depressive feelings after his diagnosis, is not motivated to complete rehabilitation, and has a fear of falling. These factors will be barriers in terms of prognosis.

Outcome Measures

Body Structure and Function:

Modified Ashworth Scale (MAS)

The MAS is an assessment tool used to measure increases in muscle tone or spasticity, where the examiner applies a high velocity stretch to a joint or muscle and then grades the spasticity on an ordinal scale of 0-4[4].

Overall, measurements obtained with the Modified Ashworth Scale (MAS) have varying inter- and intra-rater reliability depending upon which limbs is being tested[5]. As a result of this varying reliability, the validity of the MAS must be questioned[5][6]. There is also a lack of research surrounding the validity and reliability of the MAS in the ALS population specifically[7]. Nonetheless, it is still the standard for clinical assessment of extreme spasticity[6], and can be considered a supplemental outcome measure for ALS[4].  

Muscle Strength Testing (MMT and MVIC)

Manual muscle testing (MMT) is a technique used to assess the strength of a given muscle that is being testing, where the assessor manually resists the action of that muscle and then scores the strength on a scale ranging from 0-5 (no strength being 0/5 and normal strength being 5/5). Maximal voluntary isometric contraction (MVIC) is another method to assess strength, however it requires specialized equipment and training[8]. In neuromuscular diseases, it is considered a standardized method to assess muscle strength and has been used extensively in individuals with ALS[9].

Compared to MMT, MVIC eliminates muscle length and velocity as factors in testing, allowing for the production of more reliable and valid data[8]. Much of the research surrounding MMT is varied, with reliability and validity dependent on which muscle is being tested[10]. MMT is also said to lack sensitivity[11]. However, a study examining MMT in comparison to MVIC stated that MMT was better able to detect progressive weakness in ALS, making it the favoured technique for this population[10]. It is important to note however, that in this study there were significantly more muscles tested using MMT, and therefore difference in detecting change was likely accounted for by the number of muscles sampled[10]. Nonetheless, MMT is still considered a core assessment tool[12]. Either technique can be used for strength testing, however MVIC requires specialized equipment that not all physiotherapists have access to.

Activity:

Berg Balance Scale (BBS)    

The BBS is used to assess balance and falls risk in older adults through a series of 14 items that include varying static and dynamic balance activities[13]. Each item is scored on a scale of 0-4, determined by the ability to perform a given activity[13].

The use of the BBS in the ALS population is not well cited in the literature, however its use in other neurological populations is better documented[14]. One study shares that the BBS demonstrates excellent internal consistency and reliability when used with individuals with chronic progressive neurological conditions, and is strongly recommended for that general group[15]. Its use in many older adult populations also demonstrate excellent inter and intra-rater reliability, and high specificity[13].  

6 Minute Walk Test (6MWT)

The 6MWT evaluates sub-maximal aerobic capacity or endurance by assessing the distance walked over the course of 6 minutes[16]. One study determined that the 6MWT is a valid measure of aerobic capacity in ambulatory patients with ALS, while another shares that the test demonstrates good responsiveness and reliability in a longitudinal setting with ALS[17][18]. Its use in chronic progressive neurological conditions as a whole is also strongly recommended, with evidence of excellent reliability[15].  

Nine Hole Peg Test (NHPT)

The NHPT is scored based on the time it takes to remove the nine pegs, one by one, from the holes on the board, place them into a container, and then one by one replace them into the holes on the board[19]. This test is used to examine hand dexterity and can be a good tool to follow dexterity progression in ALS[19].

Example equipment for the NHPT

While research is limited, this test has demonstrated high inter-rater reliability and is sensitive to patients with neuromuscular or musculoskeletal conditions[20]. In other populations such as multiple sclerosis, stroke and Parkinson's disease, this test has demonstrated excellent test-retest reliability, excellent inter-rater reliability, and validity varying between the different neurological conditions[21].

Participation:

Amyotrophic Lateral Sclerosis Functional Rating Scale revised (ALSFRS-R)

The ALSFRS-R is a 12-item scale addressing activities of daily living and global function in patients with ALS[22]. There are four specific domains that it addresses, including fine motor, gross motor, bulbar, and respiratory function[23]. For each item on the scale, patients can rate their abilities from 0 = unable to perform the task, to 4 = normal functioning[23]. This scale helps estimate a patient’s degree of functional impairment[22].

This test has excellent test-retest reliability, excellent inter and intra-rater reliability, excellent internal consistency, good construct validity, and is sensitive to change[23][22]. However, use of a total score from the scale (sum of each domain) shows weak factorial validity and poor unidimensionality, meaning the focus should be on reporting subscores from each domain instead[23]. Overall, the ALSFRS-R has also been shown to be an independent predictor of survival and a sensitive prognostic biomarker (specifically when examining the rate of change in the ALSFRS-R score over time)[23].

Physical Therapy Management

Aerobic Exercise

Aerobic exercise under the AMSC guidelines for neurological disorders, recommends 2 to 3 days a week of moderate intensity exercise be performed for 20-30 minutes to improve quality of life, balance, and gait[24]. Prior research in this area also supports treadmill training with bodyweight support for those with ALS. Findings from a systematic review showed an improvement in gait kinematics, velocity, symmetry, and overall endurance[25]. In addition to these findings, treadmill training with sensory feedback (such as sensory cues, visual cures, rhythmic cues) was also looked at with ALS participants[25]. It was found that both verbal and musical auditory cues had the greatest effect on improving gait, specifically step length and width[25].

When making adjustments to Lou’s intervention plan, a good modification would be to add in verbal or rhythmic cueing to help him achieve his short term goal. At the end of the intervention, outcome measures were re-taken. Lou was able to perform 10 sit to stands (below the average for his age, 14) in 30 seconds without the use of his upper extremities for support, and was able to achieve a score of 51 on the Berg Balance Scale[26][27]. These outcome measures, although maintained, indicate that he is still at risk of a fall. Therefore, a rollator would be best suited for Lou when ambulating for 15 minutes or longer[27]. By the end of the 4 weeks, although there was minimal improvement with Lou’s gait, he was still able to complete his short term goal. He is also showing improvements with his posture, as he is now able to maintain an upright position while walking.

Strengthening with Moderate Exercise

Moderate exercise is commonly recommended to help manage many neurological diseases to help enhance muscle strength and avoid further complications[28]. However, those with ALS do not get these same benefits due to the rate of muscle deterioration that occurs over time[28]. There is a lack of evidence to support the efficacy of exercise in maintaining physical function, quality of life, and increasing life expectancy of those with ALS[29]. This has caused much controversy over the years, suggesting that regular exercise should be avoided to preserve muscle strength[29]. Recently, growing numbers of data have started to arise, showing that resistance exercise is safe and does have some benefit for those with ALS[30]. A recent randomized, controlled study looked at ALS participants and the combined effects of moderate aerobic and isometric exercises programs[31]. Positive effects associated with exercise were found in terms of overall independence, oxygen consumption, and fatigue[31]. This study then concluded that moderate intensity exercise can be beneficial in preventing muscle atrophy and deconditioning due to inactivity, especially at earlier stages of ALS[31].

Using this research and the AMSC guidelines, a resistance exercise program that was individualized to Lou was developed to help him achieve his short term goals. At the end of the 4 weeks, using the RPE scale and MMT’s outcome measures, Lou’s strength was very similar to his initial assessment. Lou achieved: -4/5 - quads, 4/5 - hamstrings, -4/5 - gastrocnemius, -4/5 - biceps, -4/5 - triceps, 4/5 - wrist flexors/extensors with an RPE of 4-6. Lou was able to complete his short term goal and was feeling very motivated to continue his intervention program to further achieve his long-term goals.

Fine Motor Activities

The research provides little evidence on physical therapy and the outcome of dexterity exercises for those with ALS. However, a recent systematic review showed the importance of taking a interdisciplinary approach when caring for these patients[32]. The involvement of both occupational therapy, physical therapy and many other health care providers can maximize independence, quality of life and function for those diagnosed with ALS[32]. Evidence shows that occupational therapy (OT) can help maintain hand dexterity through assistive devices and robotic assisted gloves[33]. Thus, if Lou’s dexterity training was ineffective in maintaining his griping and grasping abilities, it would be beneficial to refer him to an OT. Dexterity training commonly had participations perform reaching and grasping tasks for 15 minutes daily, which supports the intervention parameters that were provided for Lou[33]. At the end of the 4 weeks, Lou was able to maintain a similar ALSFRS score of 40 and showed more control and confidence in himself when engaged in his painting activities using compensatory strategies. These compensatory strategies, such as using foam tubing to increase the size of the paint brush handle, allowed him to accomplish his last short-term goal.

The Cochrane review of therapeutic exercise for individuals with ALS suggests that the ALSFRS is a better indicator of maintained function over long term periods of at least 3 months as opposed to manual muscle testing, which will likely decrease despite exercise efforts due to the progressive nature of this disease[29]. The goal specifies the maintenance of score within 3 points because the Minimal Important Difference of the scale is 3.24[34], meaning that a change of less than 3.24 points does not make a significant difference in the outcome. Functional exercises were chosen and prescribed to Lou in reflection of the ALSFRS by which his goal will be measured. The same Cochrane review also suggests that moderate intensity is appropriate for the ALS population to maintain muscle activity without overworking the muscles and further degrading them[29]. Therefore, American College of Sports Medicine (ACSM) guidelines[24] were followed for moderate intensity resistance exercises, which can also be applied to functional exercises that consist of strengthening activities (like those prescribed). Intervention modifications were found throughout several reviews and meta-analyses which discuss solutions for several of the impairments which complicate ADLs that are common in the ALS population. Again, these interventions will be used depending on how Lou progresses over the 3 months following his initial visit.

Balance and Gait

The Berg Balance Scale was chosen to measure this goal because it is a scale that has significant cut offs which indicate risk of falls[35]. The goal was set to maintain a score of 45 or greater because scores less than 45 indicate a higher risk of falls[35]. The intervention for this goal includes a mix of ambulation practice as well as balance exercises. Though there is not very much evidence for aerobic training in the specific population with ALS, there is previous research which supports the use of body-weight supported treadmill training for other neurologically impaired populations such as multiple sclerosis[25]. In these trials, it was found that auditory cues also had a significant impact on gait improvement, specifically in the form of verbal and musical cues[25]. As for the balance exercises, there is also a limited amount of research on the ALS population specifically. A review of intervention measures for this population done in 2018 recommends that balance training is effective[36], but does not provide parameters. The Cochrane review on therapeutic exercise for individuals with ALS, however, does offer a recommendation of moderate load / moderate intensity exercise to promote strengthening without overworking the muscles[29]. Modifications for these interventions were found in various reviews of both medical and exercise interventions for the ALS population, and will depend on the severity of Lou’s symptoms as he progresses.

Goals and Interventions

There is no cure or form of treatment that can reverse the progressive effects of ALS, so the focus of this intervention program is on supportive treatments that maintains Lou’s current function and quality of life[8]. Lou expressed prior to starting his rehabilitation program that he was unmotivated and has a fear of falling. Research has shown that when health care professionals are able to understand a patient’s educational needs, behaviours such as motivation and fearfulness can be addressed leading to a more positive experience[37]. To address Lou's needs, he was provided education on ALS, possible symptoms, reduced function, and the benefits of exercise before beginning his rehabilitation program.

Short Term Goals Intervention: Type Intervention: Frequency, Intensity, Timing[24] Modifications for Intervention
  • By week 4, patient will ambulate independently using a rollator for 15 minutes with minimal breaks. 30 Second Sit to Stand and Berg Balance Test will be used to assess lower extremity endurance
 Treadmill training with body weight supported Low to Moderate intensity (RPE: 4-6)



2-3x per week (alternating days)

20-30-minute duration

Balance: patient can use a recumbent bike or go swimming

Fear of falling/balance: educate and auditory cueing

Muscle weakness: pacing or breaking activity up into 10-minute intervals

Fatigue: decrease intensity, have patient use AFO to conserve energy

  • Patient will perform all exercises in their individualized exercise program at a moderate intensity to maintain muscle strength by week 4. RPE and manual muscle tests will be used to measure patient intensity and strength
 Isometric exercises:

1. Quad over rolls

2. Glute squeezes

4. Dorsiflexion against gravity

5. Shoulder raises

6. Bicep curls

7. Ball sqeezes

15 reps


2 sets


2-3x a week


5-10 second holds

Using a (light/medium) resistance band

Muscle cramping during exercise: add in rest periods between sets and apply a heat pack to muscles at the end of the session

Spasticity: have the patient practice movement compositions/synergies to help smooth/coordinate movements.

Motivation: Educate patient

  • In 4 weeks, Patient will independently engage in painting activities using compensatory strategies. ALS Functional Rating Scale (ALSFRS) will be used to measure patients function
 Dexterity Training:

- Reaching and grasping different sized paint brushes and putting them into a jar

15 minutes (or till mental/physical fatigue)



2-3 sets

3 days minimum but recommended daily

Coordination: using imagery to help visualize themselves performing the task.

Increased

Irritability/difficulty: breaking up and practicing different aspects of the task

Motivation: Educate patient

Long Term Goals Intervention: Type Intervention: Frequency, Intensity, Timing Modifications for Intervention
  • At 3 months post-diagnosis, the patient will be able to maintain independent ambulation in a safe manner, measured as a score of 45 or greater on the Berg Balance Scale[35]
 Treadmill training with Body Weight Support[25]


Balance exercises (to be completed while holding on to a support such as a countertop)[36]:

1. Sit-to-stand

2. Standing Marches

3. Lateral walking

4. Standing Leg abductions

2-3x per week for each type of exercise[24]


Light to Moderate intensity for aerobic exercise[29] (RPE: 2-4 on the Borg CR10 Scale)[38]

20-30 minute duration for aerobic exercise[24]

5 reps, 1-2 sets for balance exercises[36]

Balance / Fear of Falling: patient can use a recumbent bike instead or place hands on the guide rails of the treadmill during walking. Balance exercises can be traded for seated balance exercises[36]

Muscle Weakness: have patient use AFO or implement non-invasive brain stimulation before exercise[39]

Fatigue: decrease intensity or implement pacing by breaking exercise up into 5 or 10 minute segments[40]

Motivation: play the patient’s favourite type of music during exercise[25]

Dysarthria: provide signage for the patient to point to in order to indicate their RPE so that it does not need to be vocalized[40]

  • At 3 months post-diagnosis, the patient will have maintained function of muscles involved in hand and foot movement as measured by a maintained score on the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)[29] within 3 points[34]
 Functional exercises:

1. Fine motor exercise - practicing brush strokes with paintbrush, towel scrunches with toes

2. Gross motor exercise - pushing/pulling door closed/open, standing heel and toe raises

3. Gripping exercise - paintbrush squeezes, holding cup with water

4. Walking exercises - stepping over a pylon forward, backward, and sideways, stopping and turning drills

Moderate intensity resistance exercise parameters[29]


2-3x a week[24]

15 reps[24]

1-2 sets[24]


5-10 second holds[24]

RPE: 2-4 on the Borg CR10 Scale)[38]

Balance / Fear of Falling: patient can perform exercises in a seated, supine, or prone position instead of standing[36]

Muscle Weakness:  implement non-invasive brain stimulation before exercise[39]. Note that functional electrical muscle stimulation is NOT indicated for this population[41]

Fatigue: decrease intensity or implement pacing by breaking exercise up into 5 or 10 minute segments[40]

Motivation: play the patient’s favourite type of music during exercise[25]

Dysarthria: provide signage for the patient to point to in order to indicate their RPE so that it does not need to be vocalized[40]

  • At 3 months post-diagnosis, the patient will be able to maintain the ability to paint using a robotic-assistive glove. Manual dexterity of the hand will be measured by performing a 9 Hole Peg Test[42], on which the patient will score under 1 minute with use of the adaptive device[19]
 Painting with a robotic-assisted glove[33] 2-3 days/week[24]

Light to Moderate intensity[29] (RPE: 2-4 on the Borg CR10 Scale)[38]

15 minutes[33]

Fatigue: decrease intensity or implement pacing by breaking exercise up into 5 or 10 minute segments[40]

Motivation: play the patient’s favourite type of music during exercise[25]

Dysarthria: provide signage for the patient to point to in order to indicate their RPE so that it does not need to be vocalized[40]

Technology - Mediated Tools

There are several different technology-mediated tools available for individuals with ALS that are intended to aid in treatment and/or assessment of functioning[43]. These tools include but are not limited to multidisciplinary telehealth consults, communication devices through eye tracking, brain-computer-interfaces, and different types of robotics aimed at improving mobility[43]. The use of certain devices by individuals depends on where they are in their disease progression. For example, the majority of the brain computer interface devices available are used by individuals who have lost the majority of their voluntary motor control.

The Hybrid Assistive Limb is a wearable robot device that provides gait assistance based on voluntary intention by detecting weak bioelectrical signals of neuromuscular activity on skin surface[44].

For Lou, he has not progressed to advanced stages of ALS yet and has goals surrounding functioning and mobility, therefore the use of a robotic exoskeleton may be beneficial. Robotic exoskeletons are wearable units that are controlled by computer boards to power the system in order to maintain mobility and cognition[45]. There are various robotic exoskeletons available on the market for both upper and lower extremities, each providing different levels of assistance for the individual. Some of these products include the Hybrid Assistive Limb, KINARM, and ArmeoPower.

The Armeo Power is a robotic exoskeleton that has been used to help enhance motor functioning in the upper extremity by assisting active movement across a large 3D workspace[46].
KINARM is a robotic exoskeleton that permits flexor and extensor motion of the shoulder and elbow in the horizontal workspace. A virtual reality system provides users of visual feedback of movement of screen as participants hand is obscured via a barrier[47].

With these types of devices, patients are able to perform intensive, repetitive, goal-oriented exercises to facilitate improvement in strength and sensorimotor output[46]. A case study showed that using Armeo Power, which assists active movement across a large 3D workspace, showed significant improvement in strength related to shoulder abduction, elbow flexion and elbow extension in an individual suffering with flail arm ALS[43]. For Lou, the combination of the traditional rehabilitation approach for dexterity or locomotion training in conjunction with training with robotics can help him reach his goal of painting and being able to maintain his level of functioning. The use of robotics is also beneficial in a clinical setting as it can be used as an assessment tool as well as a treatment method, thus being able to quantify the sensory, motor, and cognitive impairments in patients which can be useful for therapeutic decision making[43].

There are various challenges associated with using robotic devices, including fatigue related to usage and the lack of global muscle activation. One study that investigated the feasibility of robotic exoskeletons (specifically the KINARM) use in the ALS population found that the most common reason that individuals were unable to complete the task was because the task was not attempted due to fatigue and/or motivation. In order to overcome this barrier, positioning of the machine should be considered[47]. With a modification of positioning, the machine was able to support more of the weight of the upper extremity and torso. Patients were also strapped into harnesses to aid in posture[47]. Another strategy that could be used is implementing a task hierarchy to reduce the number of tasks performed. Within the study there were a total of nine tasks that the participants were asked to perform, including a combination of sensorimotor and behavioural tasks[47]. If this type of treatment was to be used in a clinic setting, I think it would be important to consider tasks most relevant to patient goals and implement rest periods to avoid overexertion and keep motivation high.

Secondly, when using the device, only one body part can be trained at the same time which is not conducive to overall improvement[46]. ALS is usually a condition that affects the body bilaterally, therefore with the consideration of fatigability, it is likely that in a session only one body part would be able to be trained during a session. In order to manage this challenge, clinicians should consider the goals and needs of the patient. The priority in which areas are treated through the robotic exoskeleton should be based on function as well as patient goals.

References

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  13. ↑ 13.0 13.1 13.2 Berg balance scale [Internet]. 2020 [cited 2023 May 12]. Available from: https://www.sralab.org/rehabilitation-measures/berg-balance-scale
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  21. ↑ Nine-hole peg test [Internet]. 2021 [cited 2023 May 12]. Available from: https://www.sralab.org/rehabilitation-measures/nine-hole-peg-test#multiple-sclerosis
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  28. ↑ 28.0 28.1 Drory, V. E., Goltsman, E., Goldman Reznik, J., Mosek, A., & Korczyn, A. D. The value of muscle exercise in patients with amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 2001, 191(1), 133–137.
  29. ↑ 29.0 29.1 29.2 29.3 29.4 29.5 29.6 29.7 29.8 Dal Bello-Haas, V., & Florence, J. M. Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database of Systematic Reviews, 2013, 2013(5), CD005229–CD005229.
  30. ↑ Lui AJ, PT Byl NN. A systematic review of the effect of moderate intensity exercise on function and disease progression in amyotrophic lateral sclerosis. J Neurol Phys Ther 2009, 33, 68-87.
  31. ↑ 31.0 31.1 31.2 Merico, A., Cavinato, M., Gregorio, C., Lacatena, A., Gioia, E., Piccione, F., & Angelini, C. Effects of combined endurance and resistance training in Amyotrophic Lateral Sclerosis: A pilot, randomized, controlled study. European Journal of Translational Myology, 2018, 28(1).
  32. ↑ 32.0 32.1 Majmudar, S., Wu, J., & Paganoni, S. Rehabilitation in amyotrophic lateral sclerosis: Why it matters: ALS Rehabilitation. Muscle & Nerve, 2014, 50(1), 4–13.
  33. ↑ 33.0 33.1 33.2 33.3 Yamakawa, I., Yamada, A., Sonoda, Y., Wakita, K., Nishioka, T., Harada, Y., Ogawa, N., Kitamura, A., Sanada, M., Tani, T., Imai, S., & Urushitani, M. Occupational therapy using a robotic-assisted glove ameliorates finger dexterity and modulates functional connectivity in amyotrophic lateral sclerosis. Journal of Clinical Neuroscience, 2023, 107, 144–149.
  34. ↑ 34.0 34.1 Fournier CN, James V, Glass JD. Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R. Amyotroph Lateral Scler Frontotemporal Degener. 2023 May;24(3-4):311-316. doi: 10.1080/21678421.2022.2153607. Epub 2022 Dec 7. PMID: 36476010.
  35. ↑ 35.0 35.1 35.2 Lowe R, Upadhyay A, Jackson K, Walker W, Khadir SA, Coh C, et al. Berg Balance Scale [Internet]. Physiopedia. [cited 2023 May 10]. Available from: https://www.physio-pedia.com/Berg_Balance_Scale  
  36. ↑ 36.0 36.1 36.2 36.3 36.4 Bello-Haas VD. Physical therapy for individuals with amyotrophic lateral sclerosis: current insights. Degenerative Neurology. 2018 Jul 16;8:45-54. doi: 10.2147/DNND.S146949. PMID: 30890895; PMCID: PMC6065609.
  37. ↑ Madsen, L. S., Jeppesen, J., & Handberg, C. “Understanding my ALS”. Experiences and reflections of persons with amyotrophic lateral sclerosis and relatives on participation in peer group rehabilitation. Disability and Rehabilitation, 2019, 41(12), 1410–1418.
  38. ↑ 38.0 38.1 38.2 Chukwuemeka U. Borg Rating of Perceived Exertion [Internet]. Physiopedia. 2023 [cited 2023 May 11]. Available from: https://www.physio-pedia.com/Borg_Rating_Of_Perceived_Exertion
  39. ↑ 39.0 39.1 Liscic RM, Alberici A, Cairns NJ, Romano M, Buratti E. From basic research to the clinic: innovative therapies for ALS and FTD in the pipeline. Mol Neurodegener. 2020 Jun 1;15(1):31. doi: 10.1186/s13024-020-00373-9. PMID: 32487123; PMCID: PMC7268618.
  40. ↑ 40.0 40.1 40.2 40.3 40.4 40.5 Shoesmith C, Abrahao A, Benstead T, Chum M, Dupre N, Izenberg A, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. Canadian Medical Association Journal. 2020 Nov 16;192(46):1453–68.
  41. ↑ Sun XS, Liu WX, Chen ZH, Ling L, Yang F, Wang HF, Cui F, Huang XS. Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis. Chin Med J (Engl). 2018 Sep 20;131(18):2146-2151. doi: 10.4103/0366-6999.240798. PMID: 30203787; PMCID: PMC6144859.
  42. ↑ Wang YC, Bohannon RW, Kapellusch J, Garg A, Gershon RC. Dexterity as measured with the 9-Hole Peg Test (9-HPT) across the age span. Journal of Hand Therapy. 2015; 28(1):53-60
  43. ↑ 43.0 43.1 43.2 43.3 Pugliese R, Sala R, Regondi S, Beltrami B, & Lunetta C. Emerging technologies for management of patients with amyotrophic lateral sclerosis: from telehealth to assistive robotics and neural interfaces. Journal of neurology. 2022; 269(6):2910–2921. https://doi.org/10.1007/s00415-022-10971-w
  44. ↑ Morioka H, Hirayama T, Sugisawa T, et al. Robot-assisted training using hybrid assistive limb ameliorates gait ability in patients with amyotrophic lateral sclerosis. Journal of clinical neuroscience. 2022;99:158-163. doi:10.1016/j.jocn.2022.02.032
  45. ↑ Gorgey A S. Robotic exoskeletons: The current pros and cons. World journal of orthopedics. 2018; 9(9): 112–119. https://doi.org/10.5312/wjo.v9.i9.112
  46. ↑ 46.0 46.1 46.2 Calabrò R, Russo M, Naro A, Milardi D, Balletta T, Leo A, et al. Who May Benefit From Armeo Power Treatment? A Neurophysiological Approach to Predict Neurorehabilitation Outcomes. PM & R. 2016; 8(10): 971–978. https://doi.org/10.1016/j.pmrj.2016.02.004
  47. ↑ 47.0 47.1 47.2 47.3 Simmatis L, Atallah G, Scott S H, & Taylor S. The feasibility of using robotic technology to quantify sensory, motor, and cognitive impairments associated with ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2019; 20(1-2): 43–52. https://doi.org/10.1080/21678421.2018.1550515
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