Cynthia Clare Ivy*, Susan M Smith and Miranda M Materi
Mayo Clinic Arizona, Scottsdale, Arizona, USA
Received date: November 15, 2014; Accepted date: March 20, 2015; Published date: March 23, 2015
Citation: Ivy CC, Smith SM, Materi MM (2015) Upper Extremity Orthoses Use in Amyotrophic Lateral Sclerosis/Motor Neuron Disease: A Systematic Review. Int J Phys Med Rehabil 3:264. doi:10.4172/2329-9096.1000264
Copyright: ©2015 Ivy CC, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Background: Orthoses decrease the effects of muscle imbalance, provide greater ease in performance of activities of daily living, prevent joint contracture, and relieve pain. There are no published systematic reviews on the use of upper extremity (UE) orthoses in amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND).
Objective: The aim of this systematic review is to determine common orthosis interventions for ALS/MND.
Methods: The authors performed a systematic review of the literature available on Medline, EMBASE, Google Scholar, PubMed, the Cochrane Database of Systematic Reviews, and CINAHL. Key words used: 1) ALS, amyotrophic lateral sclerosis, Lou Gehrig’s disease, MND, motor neuron disease; 2) OT, occupational therapy, hand therapy, PT, physical therapy; 3) splint, brace, orthosis, orthoses, orthotic, orthotic device. Three reviewers critically appraised 22 articles using a standard format.
Results: The reviewers identified no randomized controlled or controlled clinical trials, five level 4 (case reports) and seventeen level 5 (expert opinions) using Sackett’s original 5 level pyramid.
Conclusions: Patients with ALS demonstrated improved function, increased range of motion and decreased pain with orthoses. However, there were few studies that met the search criteria. Furthermore, the studies that were reviewed had limited subjects, making it difficult to draw definitive conclusions. Further research is needed to determine appropriate study designs. for the use of upper extremity orthoses in ALS/MND. Prospective studies would strengthen the results.
ALS; Motor neuron disease; Occupational therapy; Physical therapy; Splint; brace; Orthotic device
This article presents a review of published accounts on the use of orthoses that are helpful to persons with Amyotrophic Lateral Sclerosis (ALS) and other Motor Neuron Diseases (MNDs). The authors used the structured review technique of a systematic review . “There are five steps involved in conducting a systematic review:
•Framing questions for a review.
•Identifying relevant work.
•Assessing the quality of studies
•Summarizing the evidence and
•Interpreting the findings.
The question posed in this review is, “In what manner is upper extremity orthoses use beneficial to persons with ALS (PALS)?”
One manner in which Occupational Therapists (OTs) assist patients with activities of daily living (ADLs) and improving function is through orthotic fabrication, fitting, and training. The terminology of orthoses evolved from the term “splint”. Hand surgeons were adept at making orthoses from the 1880s continuing into the late 1950s . Due to the polio epidemic and greater need for orthotic devices, occupational and physical therapists became involved in their fabrication. During World War II, surgeons, nurses, therapists, and medical corpsmen made orthotic devices. By the 1960s, with the onset of the polio vaccine, the need for orthoses decreased. Occupational therapists continued making orthoses while the other professionals reduced their involvement . Orthoses used by PALS may be beneficial to support weak or ineffective muscles and joints, to reposition the hand in a functional position, prevent joint contracture and muscle shortening due to muscle imbalance, and prevent pain associated with resting position.
ALS, involving both the upper and lower motor neurons, is the most common adult motor neuron disease. Upper Motor Neuron (UMN) loss in the motor cortex may cause increased muscle tone. The lower motor neuron (LMN) loss in the brain stem and spinal cord results in muscle weakness with normal or decreased tendon reflexes. The average onset age is 55 to 60 years of age. The ratio of male to female is 1.5 to 1. After age 65-70 the ratio changes to 1 to 1 . The average disease length until death or mechanical ventilation is 3 years from onset of muscle weakness . It is important for OTs and other health care practitioners to be aware of interventions such as orthotic fitting and training that may enhance independence in activities of daily living and quality of life. Due to the rapid progression of the disease process, it is crucial to identify the individual’s needs and provide efficient interventions. The purpose of this study is to synthesize available information on upper extremity orthosis use in ALS.
The authors compiled articles gathered from CINAHL, Cochrane, EMBASE, Google Scholar, Medline and PubMed up to the acceptance date of this article. Key words used include amyotrophic lateral sclerosis, ALS, Lou Gehrig’s disease, MND, motor neuron disease, with hand therapy, occupational therapy, OT, physical therapy, physiotherapy, PT, with brace, orthoses, orthosis, orthotic, orthotic device, and splint. The authors included articles that met the following selection criteria: observational, qualitative, and quantitative studies that mention the use of upper extremity orthoses, English only, and a diagnosis of ALS/MND. The use of upper extremity orthoses was determined by the words brace, splint, orthotic device, orthosis, and orthoses concerning upper extremity rehabilitation. The authors eliminated abstracts, book chapters, posters, articles about individuals under the age of 18, and articles about powered orthotics. Fifty-five articles were initially selected and the bibliographies hand searched by two reviewers (MM and SS). If a consensus was not reached amongst the two reviewers (MM and SS), the third author (CI) would also read the article for inclusion and exclusion criteria and a discussion amongst the three authors would ensue until consensus was achieved. Twenty-two articles meeting the criteria are included in the synthesis. All three authors critically appraised each article using a standard format and provided a numerical score based on a 0-10 scale on their impression of the quality of the study based on the critical appraisal form. However, due to the lack of high-level studies, the authors chose to use all articles meeting the selection criteria regardless of the numerical value. The inter rater reliability on the forms was established.
The results of the study yielded 5 case reports and 17 expert opinions (Table 1). In accordance with the 5 level pyramid proposed by Sackett [4,5], this translates to 5 level 4 and 17 level 5 studies that were critically appraised by the reviewers. Randomized controlled trials or controlled clinical trials were not discovered. During critical appraisal discussion, the reviewers chose to eliminate one article  from the synthesis, as it was a review of other appraised articles and did not offer new information.
|Author (s)||Methodology||Level of Evidence||Type of Orthosis/Orthoses||Octcome/Conclusion|
|Campbell &Enderby||Expert Opinion||5||Wrist cock-up orthosis
First webspaceorthosis for adduction
|Improved grip, pinch and grasp strength|
|Dal Bello-Haas, Kloos&Mitsumoto||Case Report||4||Wrist thumb orthosis
Resting hand orthosis
|Assist in reducing pain due to contractures
Wrist thumb orthosis used for acivities
Resting hand orthosis for prevention of clawing
Support weak muscles
|DeLisa, Mikulic, Miller &Melnick||Expert Opinion||5||Wrist support orthosis
Resting static orthosis
|Provide improved wrist extension and improved hand function
Wrist cock-up may assist to grasp objects and maintain optimal joint positions
May reduce contractures which lead to pain and decreases functional use
Consideration should be given to the patient’s perceived value of the device
|Francis, Bach &Melnick||Expert Opinion||4||Wrist cock-up orthosis in 30-35° extenion||Improve grip efficiency|
|Goldberg||Case report||4||Small thumb orthosis to hold the IP in extension||Orthosis deemed unsuccessful; unable to grasp the remote control while wearing the orthosis|
|Goldblatt||Expert Opinion||5||Wrist cock-up osthosis||Brings fingers into position to exert maximum grip strength|
|Ivy, Smith &Materi||Case report||4||Prefabricated including figure of eight and dorsal wrist orthoses
Custom orthoses including finger orthosis, hand based thumb spica, resting hand, and sttachable hand component for extension
|Can improve meaning and quality of life
Therapists play a crucial role on the team and can recommend and fabricate orthoses
Prevent PIP buckling during use of mouse
Dorsal wrist orthosis maximizes use of sensory surface
Dorsal wrist orthosis lighter
Circumferential finger orthoses prevent contractures, decrease pain and add leverage for activities requiring pressure force
Attachable digit night extension piece may be used with day wrist orthoses
|Janiszewski, Caroscio&Wisham||Expert Opinion||5||Opponensorthosis
|Reports orthoses helpful
Supprots weak muscles
Recommend prompt referrals
|Johnson, Florence &Abresch||Expert Opinion||5||Neutral resting position during sleeping hours||Goal of limiting contracture
Suggests early intervention
|Lenox||Expert Opinion||5||Palmar and dorsal cock-up orthoses
Static and dynamic orthoses
|Dorsal cock-up improves mobility in hand and fingures
Static orthoses support weak and immobile fingers and reduce painful contractures
Dynamic orthoses are costly and may be difficult to obtain
|Lewis &Rushanan||Expert Opinion||5||Wrist cock-up orthosis in approximately 30° extension
Resting hand orthosis
|Wrist cock-up can improve grip function, reach and grasp
Resting hand orthoses may be used to prevent contractures
|McDonald||Expert Opinion||5||Nighttime resting orthosis in wrist extension and MCP extension, PIP flexion
Daytime position emphasis on wrist and finger extension
|Early diagnosis and initiation of orthoses contractures are still mild
Should not compromise function
|Majmudar, Wu &Paganoni||Expert Opinion||5||Resting hand (light weight; wrist in neutral)
Anti-claw (intrinsic plus)
Volar cock-up in 20-30° of wrist extension
Short opponens with thumb in abduction and opposition
|Reduce hand pain from finger contractures
Allow for increased MP flexion for functional positioning during activitie
Improved grasp when wrist extensors weak
Improved grasp with weak intrinsic muscles
Useful day and night
Recommends controlled studies for impact and safety
|Morris||Expert Opinion||5||No specific orthoses were described||Can help to prevent and decrease impairment such as muscle strain, or disuse atrophy
Prompt referral are needed to optimize function
Publication of therapy outcomes needed for reimbursement and accountability
|Rocha, Reis, Simoes, Fonsea& Medes Ribeiro||Expert Opinion||5||Wrist extension orthosis in 30° of extension||Improved grip function|
|Sinaki||Expert Opinion||5||Wrist support
Thumb orthosis lacing the thumb in apposition
|Wrist supports improve extension and allow for a better grip
Thumb orthosis in which the thumb is placed in apposition allows for more effective use of hand for fine motor movements
|Sinaki& Mulder||Expert Opinion||5||Thumb orthosis
Wrist immobilization orthosis
Dynamic MCP extension orthosis
|Thumb shell extends to the IP joint of the thumb allowing for thumb opposition, enabling a 3-point pinch; function|
|Smith & Norris||Expert Opinion||5||Wrist orthosis||Orthoss may help with grasp|
|Skalsky& McDonalds||Expert Opinion||5||Nighttime resting with MP extension and IP flexion
Daytime wrist and finger extension
|Use orthoses before contractures present and while contractures mild
Orthosis use should not compromise function or sensation
|Tanaka, Houaiya, Akagi&Kihoin||Expert Opinion||5||Short thumb spica
Long thumb Spica
|Simple webspacer improved tip to tip pinch strength, improved FIM score, and improved ability to fasten buttons and write
Cock-up for inability to extend wrist
Hand orthoses useful for increasing independence
Algorithm can help providers choose what orthosis is best for particular patients
|Tanaka, Saura, Houraiya&Tanimura||Expert Opinion||5||Neoprene orthosis as simple webspacer||Orthoses improve function
Orthoses must be fabricated in a timely fashion
Ease of application important
Usefulness diminishes as disease progresses
Soft material suggested with intact sensation
Table 1: Case reports & experts opinion.
Thirteen articles [7-19] advocate the use of volar wrist supports, with 5 articles specifying that the wrist be placed in 30-35 degrees of extension [9,13-16]. Twelve articles state wrist extension orthoses improve grip strength and/or efficiency therefore improving function [7,8,10,11,13-16,18,20] (Figures 1 and 2).
Eight of the reports advocate the use of resting hand orthoses that include the forearm and the hand [8,11,13,14,21-24] (Figure 3). Two case reports state that a resting hand orthosis may prevent intrinsic minus positioning, also referred to as clawing whereby the metacarpal phalangeal (MCP) joints fall into hyperextension and the Interphalangeal (IP) joints become fixed in flexion [11,21]. Conversely, McDonald and Skalsky and McDonald, recommend the MCPs be placed in neutral and the IPs be placed in flexion within the orthosis for night. The same authors promote full wrist and finger extension positioning for day use. They also emphasize that orthoses should not compromise function or sensation.
Ten of the reports recommend a thumb positioning orthosis to assist with functional pinch [7,11,12,14,16,17,19,21,25,26] (Figure 4). Campbell and Enderby recommend thumb adduction. Goldberg discusses a small thumb orthosis that holds the IP joint in extension. Tanaka, Saura, Houraiya, and Tanimura Sinaki and Sinaki and Mulder state that the thumb orthosis may increase independence in multiple tasks such as holding a cup, fastening buttons, writing, and pouring water. Tanaka, Saura, Houraiya and Tanimura published an algorithm for clinical decision-making. They recommend early use of a wrist cock-up splint, long thumb spica (Figure 5), short thumb spica, and simple web spacer. Other authors advocate the use of a thumb spica orthosis for opposition  and improved grasp in those with thumb abduction and extension weakness . Combining the wrist and the thumb in a single orthotic to address both weak wrist extension as well as weak abduction may be useful in some cases [12,16,19,21] (Figure 5). Additionally, Lenox and Sinak discuss use of dynamic finger extension assist orthoses. Lenox also notes that a dorsal placement of the wrist orthosis may allow for increased finger motion.
In addition to suggestions on positioning for prevention of contractures and efficiency in grip and pinch strength, many of the reports address specific functional improvements with orthotic use. Seven of the articles mention functional gains with orthosis use [8,11,13,16,17,21,26]. Described functional improvements include using technology, holding objects including a hairbrush, walker, cup, and utensils and the ability to complete personal hygiene. In addition, case reports discuss improvement in sociability and participation in leisure activities such as crocheting .
Case reports also show relief of pain with orthoses [11,21]. In one of the reports, two case studies explicitly mention circumferential finger and figure of style orthoses, prefabricated dorsal wrist supports, and nighttime forearm resting hand orthoses for pain management and/or comfort  (Figures 3, 6-8).
This systematic review reveals that the published literature on the use of upper extremity orthosis with ALS/MND is anecdotal and based on expert opinion and case report. Most of the level 4 studies are single patient case studies. Furthermore, the outcomes that were used to measure success in the studies are not consistent or comparable. Based on these facts, there is not enough information to assess bias between studies. There is not enough data to compare the recommendations between studies mathematically to look for bias. To compare the studies mathematically, it would be necessary to compare studies with more than one patient that measure the same outcome.
Despite not finding higher level of evidence studies, there were a number of case reports and expert opinions on the topic of orthoses with motor neuron disease documented in the literature. No prospective studies and no consistent methodology amongst the authors in reaching the conclusions that they reported in the papers are the biggest limitations of this systematic review. Because of this, it is impossible to completely synthesize the information. It is clear however, that there is a trend toward the usefulness of wrist extension orthoses, night orthoses, and thumb positioning orthoses. Persons with ALS/MND could benefit from further published accounts on the use of upper extremity orthoses to decrease the effects of muscle imbalance, provide greater ease in performance of ADLs, prevent joint contracture, and relieve pain.
Published accounts could come in the form of various prospective methods. A case series may be one useful method. This is a descriptive study that could be arranged prospectively to monitor change in function, pain, and range of motion over time using consistent outcome measures. Planning to report cases ahead of time would allow for increased detail and uniformity in the data that is collected. This in turn would allow increased ability to analyze the usefulness and best timing of orthotic intervention. Completing a randomized controlled trial on the effectiveness of orthotic use could occur by comparing various orthosis designs. A comparison might be made between volar and dorsal wrist orthoses, full extension of the wrist versus partial extension and /or prefabricated versus custom orthoses. Outcome measures for these types of studies could include functional outcome measures, pain assessments, and patient satisfaction surveys. Existing valid and reliable measurement tools are pain scales such as a Likert scale  range of motion with goniometry [28,29], grip and pinch strength [30,31]. The Disability of the Arm, Shoulder and Hand (DASH) questionnaire or the shorter version of this (QuickDASH) , the Upper Limb Functional Index [21,33], the Canadian Occupational Performance Measure  and quality of life scales developed specifically for ALS  or others with advanced disease [36-38]. Pre- and post-photographs could document success in positioning and reduction of resting contracture Biomechanical studies may be useful to further evaluate the impact of imbalance between the intrinsic and extrinsic musculature and how best to accommodate to the imbalance with external support and positioning.
There was some conflicting information in the reports on the positioning for resting orthoses. Two papers described placing the hand in the intrinsic minus position, which is with the MCPs extended, and the IPs flexed [23,24]. This is a common position of deformity with intrinsic weakness, frequently observed in ALS/MND, therefore one would typically place the hand in an alternate position with the MCPs flexed and IPs extended in order to place the presumably shortened soft tissue structures on stretch. If however, spasticity or a comorbid condition such as rheumatoid arthritis leads to laxity of the MCPs or subluxation of the extensor digitorum communis over the metacarpal heads, this would be an optimal position for the hands. Another conflicting recommendation was placing the thumb in adduction, also a common deformity in ALS/MND due to muscle atrophy . Typically, most grasp patterns favor the thumb in opposition. If however, the provider wishes to gain a strong lateral or appositional pinch, also known as “key pinch” as in grasping a car key, adduction of the thumb may be desirable. Tanaka et al. recommend soft material if the patient has intact sensation. It is important to try to make all orthoses as comfortable and lightweight as possible and sometimes a softer material achieves these goals. If there is not intact sensation, which would typically be from a co-morbid diagnosis such as an ulnar nerve compression, soft material may also be important to lessen the chance of pressure related ulcers that may go unnoticed due to the lack of sensation.
There were several themes apparent in the appraisal of the papers. One overriding theme noticed in the 21 papers is emphasis on early intervention and a multidisciplinary approach. The importance of frequent evaluations in order to have the most beneficial timing of orthotic intervention is another theme. Authors also regularly suggested individualizing the treatment with particular attention to the unique functional goals and needs. Figure 9 provides a quick reference for common ALS/MND upper extremity problems and some possible orthotic solutions. Wrist hand finger and thumb orthoses in a resting position, worn at night or at rest during the day, may help to maintain tissue length. A wrist orthosis may increase grip strength and provide support for functional use and grasp during activities of daily living. Finger extension orthoses may assist with preventing and decreasing contractures at the proximal interphalangeal joints, thus preventing or diminishing pain. An opposition style thumb orthosis may be helpful in positioning the hand in a functional posture. It is essential to be aware of problems arising with sensation or function when using an orthotic intervention. One may achieve this through periodic assessments by the occupational therapist for proper fit as well as training and discussion of the purpose and use of the orthosis. It is important to assess the patient’s function throughout the course of the disease process due to the progressive nature of the disease and thus the changing needs of the patient. Future published accounts of the benefits of upper extremity orthotic intervention for ALS/MND will help guide timing of fitting and fabrication as well as decisions on the most appropriate upper extremity orthoses to consider.