All hands on deck: The multidisciplinary rehabilitation assessment and management of hand function in persons with neuromuscular disorders

Abstract

Hand function is important in every aspect of our lives. Across a wide range of neuromuscular disorders—inherited ataxias, motor neuron diseases, polyneuropathies, and myopathies—people can experience losses in hand strength, tone, movement, dexterity, joint range, and sensation. Such changes can adversely affect function and independence in daily activities, reducing participation and quality of life. People with neuromuscular disorders (pwNMD) known to involve the hand should be assessed at regular intervals for changes both clinically and using impairment, performance, function, and patient‐reported outcome measures as appropriate. A patient‐centered approach to management is recommended, with clinicians partnering with the individual, their caregivers and the interprofessional teams to create personalized solutions that can overcome barriers to participation and best meet the goals of individuals affected by neuromuscular disorders. Management strategies should be multifaceted, and may include exercise, orthoses, assistive devices, technological solutions, environmental or task adaptations, medications, and/or surgery. Exercise recommendations and orthoses should be individualized and evolve based on disease progression, impairments, and functional limitations. While medications and surgery have a small role for specific clinical situations, there is a plethora of assistive and technological solutions to assist with basic and instrumental activities of daily living, work/education, and leisure for pwNMD with reduced hand function. In addition, clinicians should advocate for appropriate accommodations for reduced hand function at work/school, and the development of and adherence to legislation supporting accessibility and inclusion.

Abbreviations

ADLs

activities of daily living

AFO

ankle foot orthosis

ALS

amyotrophic lateral sclerosis

BMD

Becker muscular dystrophy

CMT

Charcot–Marie–Tooth disease

DASH

Disability of Arm, Shoulder and Hand scale

DASH‐W

Disability of Arm, Shoulder and Hand scale‐work

DASH‐SM

Disability of Arm, Shoulder and Hand scale‐sport, music

DM1

myotonic dystrophy type 1

DMD

Duchenne muscular dystrophy

FA

friedreich ataxia

FRS

Functional rating scale

HMSN

hereditary motor sensory neuropathy

IADLs

instrumental activities of daily living

IBM

inclusion body myositis

IP

interphalangeal

LGMD

limb girdle muscular dystrophy

MCP

metacarpophalangeal

NMD

neuromuscular disorders

OA

osteoarthritis

PIP

proximal interphalangeal joint

PROMs

patient‐reported outcome measures

Pw

persons with

pwALS

persons with amyotrophic lateral sclerosis

PWC

power wheelchair

pwDM1

persons with DM1

pwFA

persons with friedreich ataxia

pwNMD

person living with a neuromuscular disorder

pwSMA

persons with spinal muscular atrophy

QOL

quality of life

ROM

range of motion

SEGT

sensor‐engineered glove test

SMA

spinal muscular atrophy

SMART

Specific, Measurable, Achievable, Relevant, and Time‐Bound.

SWMF

Semmes‐Weinstein monofilaments

1. INTRODUCTION

There is no doubt that the hand, extolled by Aristotle as “the tool of tools,” ¹ plays an important role in every aspect of our lives. Hand function impacts quality of life (QOL) in people living with neuromuscular conditions, ² , ³ , ⁴ , ⁵ , ⁶ and is important for self‐care, occupation, leisure activities, communication, and even healthcare access such as registering for a doctor's appointment. Beyond physical tasks, hands are integral to human connections. Hand movements convey emotions and are intrinsic to communication. Touch can mediate bonds by projecting empathy, care, and love. While this review focuses on issues related to the hand, we acknowledge that optimizing hand function does not happen in a vacuum. There must also be adequate function higher in the kinetic chain including truncal balance and proximal upper limb control for optimal hand function. This review will discuss assessment methods, exercise, assistive devices, and technologies to improve participation for people with decreased hand function due to neuromuscular disorders, emphasizing the essential holistic approach in formulating a rehabilitation plan. We have engaged people with lived experience throughout the development of this review paper, and wish to open our review with the following quote:

Maintaining and improving hand function is vital to enhancing the QOL of those affected by neuromuscular disorders, supporting people like me to live our best lives. From access to technology to support employment, community participation and recreation, to tools to simplify activities of daily living (ADLs), to treatments and therapies to enhance strength, these much‐needed options are vital for allied healthcare professionals to have access to in order to best meet the needs of individuals who are impacted by these progressive disorders.—49‐year‐old male affected by spinal muscular atrophy (SMA)

2. REHABILITATION AND HAND IMPAIRMENTS IN NEUROMUSCULAR DISORDERS

Hand impairment and associated reduced function occur in many neuromuscular disorders. A rehabilitation approach to impairment, assessment, and management provides a “patient‐centered” comprehensive evaluation and treatment plan with focus on maximizing function and improving QOL. The World Health Organization defines “rehabilitation” as a set of interventions designed to optimize functioning and reduce disability in individuals with health conditions in interaction with their environment. ⁷ Rehabilitation of a person living with a neuromuscular disorder (pwNMD) requires assessment of the impairment(s), of how such impairment(s) affect activities (such as dressing, feeding) and participation (for instance attending school or work), and in the context of personal (e.g., age, gender, cognition), social (e.g., relationships, education), and environmental (e.g., laws, terrain, attitudes) factors. ⁸ Treatment strategy is informed by both the assessment and the pwNMD/family goals. The rehabilitation process is cyclical with overarching objectives of maximizing function and optimizing QOL; reassessment identifies the outcomes of prescribed interventions and updates functional status, and goals are modified accordingly. Members of the rehabilitation team can include health professionals from disciplines such as occupational therapy, physiotherapy, prosthetics and orthotics, medicine, nursing, and engineering working together with community partners, peers, and family (see Figure 1). Importantly, there is no “one size fits all” algorithm to drive therapeutic choices or timing; the vision of the collaborative rehabilitation process is the ability to provide proactive individualized pwNMD‐centered care, which is particularly relevant in progressive conditions.

FIGURE 1.

A vision of rehabilitation for person living with a neuromuscular disorder (pwNMD). The goals are to maximize function and optimize quality of life. With the pwNMD at the center, the rehabilitation team (physicians, physiotherapists, occupational therapists, orthotists, speech language pathologists, dietitians, social workers, etc.), peers and family and community partners use multimodal interventions and regular monitoring to help pwNMD achieve their goals. PROM, patient–reported outcome measures; SMART, specific, measurable, achievable, relevant, and time‐bound.

Impairment evaluation occurs with initial assessment and recurs throughout rehabilitation. Recognizing that there are many neuromuscular disorders that involve the hand, to illustrate various ways how impairments may present we have selected a few representative examples of neuromuscular conditions from among the broader categories of inherited ataxias, motor neuron diseases, polyneuropathies, and myopathies.

2.1. Friedreich ataxia

Friedreich ataxia (FA) is the most common inherited ataxia ⁹ ; the disease affects multiple regions of the central nervous system; the cerebellum, visual and vestibular systems in the brain, posterior columns, corticospinal, and spinocerebellar tracts in the spinal cord. ⁴ There is also evidence of a sensory neuronopathy in up to 98% of people, that is often severe, but insidious in onset, so those with FA may not always notice a lack of sensation. ⁹ Persons with FA (pwFA) can therefore exhibit both sensory and cerebellar ataxia, nonlength‐dependent sensory loss, spasticity, weakness, and fatigue, leading to decreased hand strength and dexterity. ⁴ A study examining hands in pwFA found spasticity in 68%, contractures of at least one joint in 36.8%, weakness of intrinsic muscles in 84%, and finger hypermobility in nearly 95%, with the Functional Independence Measure significantly correlated with hand weakness and spasticity. ⁴ Oculomotor dysfunction, including unstable fixation, is common, ⁹ and can hinder both general function and the use of eye‐tracking computer controls intended to compensate for reduced hand function. Most pwFA develop varying degrees of scoliosis, which can impact positioning. ⁹ Impaired trunk control and postural responses then contribute to upper extremity instability which further affects reaching capacity and hand function. ¹⁰

2.2. Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease causing loss of motor neurons. ¹¹ In about 70% of cases, manifestations first occur in a single limb, ¹² most often distally, with approximately equal distribution between upper and lower limbs, ¹¹ and subsequent progressive involvement of other body regions. Persons with ALS (pwALS) display both upper motor neuron (spasticity, loss of motor speed, and precision) and lower motor neuron (fasciculations, atrophy, muscle weakness, and cramps) signs with few or no sensory changes. ¹² Many pwALS, especially with limb‐onset presentation, ¹³ show a “split hand” pattern, in which the radial side of the hand, is preferentially affected compared with the muscles of the hypothenar eminence, ¹⁴ despite sharing the same myotomes and, in the case of first dorsal interosseus and abductor digiti minimi, peripheral innervation. ¹⁵ Reported in about 20% of pwALS by Kuwabara et al., ¹⁴ 50% in a 2021 systematic review, ¹⁶ and even observed in up to 95% at follow‐up by some authors, ¹³ this phenomenon seems to occur early in the disease course and is very rarely found in other neuromuscular diseases. Some pwALS may progressively develop bilateral, severely debilitating “flail arms” ¹⁷ with complete upper extremity paralysis and secondary balance and mobility impairments. Whether through spasticity, loss of dexterity, or weakness, pwALS with hand involvement will demonstrate fine motor difficulties with functional implications. ¹⁸

2.3. Spinal muscular atrophy

Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused, in about 95% of cases, by a homozygous deletion of the SMN1 gene, resulting in progressive loss of anterior horn cells in the spinal cord, ¹⁹ causing flaccid paralysis. The disease phenotype correlates with the number of SMN2 copies in one's genome, with a broad spectrum ranging from infants with symptom onset at birth or in the first weeks of life and never being able to sit, to milder adult‐onset forms. ¹⁹ When upper limbs become involved, persons with SMA (pwSMA) usually develop earlier and more severe proximal weakness ²⁰ and contractures, ²¹ but eventually face issues with hand strength and range of motion (ROM), especially in cold conditions, that worsen with age and progressively impair independence. ²² Some pwSMA also demonstrate hand joint hypermobility ²² and up to 59% of patients will demonstrate a fine hand tremor. ²³

2.4. Hereditary sensorimotor neuropathy

Hereditary neuropathies encompass a broad range of heterogeneous genetic and phenotypic presentations from mild to severe disability, the most common of which is Charcot–Marie–Tooth disease (CMT) or hereditary sensory motor neuropathy (HSMN). ²⁴ Overall, about 75% of neuropathies (including HSMN) are length dependent, producing impairments of distal foot, ankle, hand, and finger strength, muscle atrophy, fatigue, sensory disturbance, cramps, pain, tremor, loss of motion and dexterity, and musculoskeletal deformities. ²⁵ , ²⁶ , ²⁷

In CMT, the intrinsic hand muscles (interossei, lumbricals, thenar, and hypothenar) are preferentially affected, resulting in progressive clawing of the fingers due to unopposed long extensors and flexors, and loss of thumb opposition. ²⁸ With prolonged hyperextension of the metacarpophalangeal (MCP) and flexion of proximal interphalangeal joints, stiffness and contractures develop which further impacts power grip. In total, there is a slowly progressive loss of tactile sensation, synchronistic finger flexion, adduction/abduction, thumb stability, hand grip and pinch strength. ²⁹ , ³⁰ Hand involvement is not necessarily a late‐onset experience for people with CMT. In fact, hand impairments may be under‐recognized in early CMT1A. Burns et al. ³¹ found in a cohort of 84 children aged 2–16 years reduced strength and function at the earliest stages of disease, which never improved to normal range throughout childhood.

People with CMT commonly report hand weakness as an issue that leads to restricted participation. The CMT Health Index was developed from an international registry survey of 407 participants (50.9% response rate) as a disease‐specific patient‐reported outcome measure (PROM) of symptom burden for all subtypes of CMT. Hand and finger weakness was the fourth most prevalent theme at 97%. ³² In addition, Videler et al. ³³ reported that upper limb dysfunction was perceived by 98% of a CMT1A cohort (N = 49) and strongly correlated with restricted participation, while lower limb disability did not. Upper extremity weakness was cited as the most sensitive marker of overall disability and hand dexterity noted as essential for autonomy in ADLs. ³⁴ , ³⁵ The most noted disease‐related limitations include difficulties with handwriting, dressing and undressing, personal care, and household activities. ²⁷ , ³¹

2.5. Inclusion body myositis

Myopathies are a diverse group of conditions that include inherited or sporadic forms, some with systemic involvement such as the mitochondrial myopathies, and cause progressive muscle degeneration resulting in muscle weakness, atrophy, and joint contractures. Inclusion body myositis (IBM) is a progressive inflammatory myopathy characterized by weakness of the deep finger flexors (flexor digitorum profundus) and knee extensors, and dysphagia. Onset is usually after 40 years of age, more common in males, and diagnosis is based on muscle histopathological features. ³⁶ Progressive loss of hand function is a feature of IBM, with hand grip and fine motor task impairments associated with inability to flex distal and sometimes proximal interphalangeal (IP) joints. Typical progression, although generally slow, is associated with marked morbidity and reduced QOL, ³⁷ , ³⁸ with limited hand function associated with loss of independence in daily activities.

Difficulty with active finger flexion creates challenges with capturing hand and grip strength using standard tools such as handheld dynamometry. Compensatory techniques such as MCP and thenar flexion to produce a functional grip are often used. ³⁹ A study reported upper extremity function in 74 persons with IBM, identifying weaker grip strength than controls and more time to complete a functional dexterity test. ³⁹ Transverse volar grip strength correlated with disease duration, and hand weakness was greater in nonambulatory participants. Performance‐based tests are suggested to identify impairments and activity limitations that may not be captured with common grip strength tests.

2.6. Muscular dystrophies

Muscular dystrophies are disorders characterized by progressive muscle weakness and atrophy due to mutations in genes responsible for normal muscle fiber function. Muscular dystrophies include, among others, Duchenne (DMD), Becker, myotonic (described in more detail below), facioscapulohumeral, limb‐girdle, and oculopharyngeal.

Hand involvement varies widely across and within disorders; a study of upper limb function in adults with DMD found large variation in strength of wrist extension and thumb adduction, with wrist severely weak compared with reference values. Distal muscles were generally less affected than proximal, with index finger extension and thumb adduction best preserved. ⁴⁰ This same study found most men had lost supination and wrist extension range, with preserved index flexion and extension. These myopathies are associated with limb contractures, with faster rates of disease progression resulting in earlier and more severe contractures, particularly when an individual becomes nonambulatory. Dystrophic myopathies result in muscle fiber necrosis, fatty tissue infiltration and fibrosis with shortened resting muscle length, which contribute to contracture development. ⁴¹ While distal upper limb strength may be relatively less affected in many myopathies and dystrophies, impairment in proximal upper limb strength and range secondarily limits positioning of the hand for functional tasks.

Myotonic dystrophy type 1 (DM1) is an example of a dystrophy that preferentially affects the distal upper limb. An autosomal dominant multisystem disease, it causes a range of cardiorespiratory, genitourinary, ocular, endocrine, central nervous system and skeletal muscle impairments and is the most common inherited adult‐onset myopathy, with a prevalence estimated at 1 in 20,000 worldwide. There are four subtypes; congenital, childhood, adult and late onset, with more severe symptoms occurring at earlier ages of onset, and wide individual variability. ⁴² , ⁴³ Skeletal muscle weakness and atrophy generally begin in distal upper (particularly long finger flexors) and lower limbs, the neck, and slowly progress to the trunk. Myotonia, or delay in muscle relaxation, most commonly affects the forearm and finger muscles, is present in almost 100% of adult‐onset patients and is worsened with cold temperatures. DM1 may cause stiffness, pain, and prolonged hand grip, thus reducing manual dexterity. Upper limb muscle impairments have been associated with activity and participation limitations such as lifting and grasping objects, opening doors or jars, and household activities. ⁴² , ⁴⁴ An MRI study of upper limb weakness in 17 people with DM1 (pwDM1) demonstrated high‐intensity signals in the flexor digitorum profundus, abductor pollicis longus, extensor pollicis, with proximal muscle involvement in prolonged disease. ⁴⁵ A study of a large cohort of adult and late onset pwDM1 confirmed a slow annual rate of decline on validated tests of hand grip strength, lateral pinch strength, gross and fine motor dexterity over a 9‐year period, with men demonstrating greater weakness and decline than women. ⁴⁴

3. IMPAIRMENT AND FUNCTION ASSESSMENT

PwNMDs known to involve the hand should be assessed at regular intervals, evaluating and monitoring stability and changes in symptoms, signs and function expected for their diagnosis. Interestingly, measures of hand motor impairment such as strength have been demonstrated to correlate poorly with activity limitations, potentially attributable to compensatory strategies and that strength is but one factor in hand function. ⁴⁶ This underscores the importance of a whole‐person “holistic” approach to assessment, selecting the appropriate tools and measures to capture performance, QOL, and pwNMDs' priorities.

Neuromusculoskeletal history, physical exam, and outcome measures should be documented at each visit for monitoring, informing care, and supporting advocacy needs. If a pwNMD presents with atypical features, or more rapid than expected neurological deterioration, concomitant or superimposed diagnoses should be considered. For example, comorbid pathologies such as entrapment neuropathies, radiculopathy, neurotoxic exposures, or endocrinopathies such as diabetes and thyroid disorders, should be investigated and treated as appropriate. ⁴⁷ , ⁴⁸ Carpal tunnel syndrome affects 3%–6% of adults, with prevalence much higher across CMT subtypes (as high as 31%). For clinicians and electrodiagnostic medicine specialists, diagnosis of CTS can be challenging in inherited neuropathies as there is a significant overlap in symptoms between both conditions, electrodiagnostic criteria are less reliable due to demyelination and axonal loss, median and ulnar sensory nerve action potentials are often absent and are preferentially involved vs. radial. A minority of patients are also known to have baseline asymmetry of CMT disease. ⁴⁹ A Boston Carpal Tunnel Questionnaire Symptoms Severity Score ≥20 may be used as a high sensitivity, moderate specificity threshold to guide CTS diagnosis in patients with inherited neuropathy. In their retrospective study of 309 patients, Panosyan et al. ⁵⁰ also identified a higher risk of CTS in CMT patients with comorbidities of RA, wrist fracture, renal disease with dialysis, osteoarthritis (OA) of the wrist, and collagen vascular disease. Both splints and surgical decompression have been found to be effective treatments in CMT1A. ⁵¹ In patients with hereditary neuropathy with liability to pressure palsies, there have been case reports and small series suggesting benefit from CTS surgical release, especially in those who reported activity‐provoked symptoms including nocturnal awakening. Nerve enlargement by ultrasound at the carpal tunnel has not been successful in predicting surgical outcomes and nerves are diffusely enlarged in CMT1A. ⁵² , ⁵³ Diabetes is also known to worsen motor and sensory impairment in CMT1A. ⁵⁴

Persons with DM1 require regular monitoring for cardiac and metabolic disease, which confer additional risk of polyneuropathy and stroke. ⁴² As pwDM1 can have variable central nervous system (CNS) involvement, including developmental disabilities, which can potentially complicate the neurological presentation, careful evaluation of all factors is necessary. ⁴² While IBM affects an older cohort, with greater likelihood of common conditions of aging such as spinal arthropathy or stroke, IBM is also associated with a higher rate of polyneuropathy, Sjögren's syndrome, and T‐cell large granular lymphocytic leukemia. ⁵⁵ Therefore, it is important not to assume that all symptoms, signs, and functional decline are a result of the neuromuscular condition.

An excellent detailed approach to functional assessment of the hand and wrist was described by Kimmerle illustrating several key points. ⁵⁶ Firstly, basic Physical factors of ROM, strength, sensation, pain, myotonia, and swelling should be evaluated; in particular, hand intrinsics, long finger flexor and extensor muscles, resting upper limb posture, active and passive wrist flexion, extension, supination, pronation, MCP, and IP ROM, and strength. Psychological factors may impact function, such as developmental/perceptual and cognitive limitations, motivation, and self‐perception or body image relating to hand use. Hand roles identify how each hand may be used in bimanual or unimanual tasks. Object‐related hand and body actions refers to specific movements used for reaching, grasping, and manipulating such as stabilization and movement of the trunk, types of power and pinch grasps (Figure 2). Finally, Task parameters include characteristics of the object such as size, shape, texture, and performance demands such as force, endurance, speed, and physical environment that may directly affect ADLs, work, or leisure function. This model is useful to guide selection of specific treatment targets and appropriate outcome measures for objective monitoring by clinicians. Even in cases of severe impairments, pwNMD have often been observed to demonstrate remarkable ability to adapt with their own unique long‐standing compensatory strategies. Ultimately, the practical ability to accomplish a task will determine if a rehabilitation recommendation is accepted or rejected by a pwNMD. ⁵⁷

FIGURE 2.

The six key types of grasps. Copyright W. Peter Kyberd; Used with permission. Tip and tripod grasps have precision function, whereas lateral, flexion, power, and extension are power grasps.

There are several tools developed to measure upper limb function and outcomes in the neuromuscular population, with a variety of intended purposes; characterize natural history, clinical trials, clinical assessment, guide management, monitor treatment response, access resources, or patient advocacy. Tables 1, 2, 3 list examples of hand function and impairment, manual dexterity, and patient, caregiver, or clinician reported outcome measures and tools.

TABLE 1.

Examples of hand function and/or impairment assessments for PwNMD.

Measure	Populations	Measurement characteristics
Handheld grip dynamometry	DM1, HSMN, IBM 35 , 39 , 44 , 165	Power grip quantitative muscle strength Highly reliable and valid in DM1, HSMN May not take into account power grip compensations used in IBM such as flexion of MCPs and thenar to produce measurable result
Pinch dynamometry—lateral, tripod, tip‐to‐tip	DM1, HSMN, IBM 28 , 35 , 39 , 44 , 165	Pinch grip quantitative muscle strength Lateral pinch likely best measure for long‐term change or upper limb function in DM1 Tripod pinch considered major determinant of manual dexterity in HSMN Tip to tip may have higher correlation to patient‐reported outcomes in IBM than Functional Rating Scale (FRS)
Thumb opposition test	HSMN 28 , 166	Clinical test – considered a major determinant of manual dexterity in HSMN
Sollerman Hand Function Test	HSMN, IBM 35 , 39	20 standardized subtests of 8 main hand grips involved in ADL function administered by clinician Time intensive to administer in clinic Transverse volar grip found to correlate with disease duration in IBM in small cross‐sectional study May have ceiling effect at higher levels of function
RULM—Revised upper limb module	SMA 167	20 subtests of upper limb functional tasks, SMA‐specific scale Good reliability and validity
PUL 2.0—Performance of the upper limb	DMD, BMD, LGMD, SMA, IBM 39 , 168 , 169	Standardized clinician assessment of proximal and distal upper limb function Ceiling effect in disorders without significant proximal weakness

Abbreviations: BMD, Becker muscular dystrophy; DM1, Myotonic dystrophy type 1; DMD, Duchenne muscular dystrophy; HSMN, Hereditary sensory motor neuropathy; IBM, inclusion body myositis; LGMD, limb girdle muscular dystrophy; SMA Spinal muscular atrophy.

TABLE 2.

Examples of timed performance assessments for manual dexterity in PwNMD.

Measure	Populations	Notable measurement characteristics
Purdue Pegboard	DM1 44	Assesses right, left, and both hands Normal values determined for various manual occupations
9 Hole Peg Test	DM1, SMA, HSMN 35 , 170 , 171	In SMA adapted to detect and quantify fatigability of the upper limb
Functional Dexterity Test	Peripheral Neuropathy (leprosy), IBM 39 , 54 , 55 , 172 , 173	The addition of assessing tripod grasp without forearm rotation and in hand manipulation when compared with other pegboard tests
Jebsen‐Taylor Hand Function Test	HSMN, Muscular dystrophies (including DMD, LGMD, BMD, DM1, FSHD) 174 , 175 , 176	7 items that assess fine motor, weighted and nonweighted gross motor hand tasks that simulate ADLs 15–45 min to complete

Abbreviations: ADLs, activities of daily living; BMD, Becker muscular dystrophy; DM1, myotonic dystrophy type 1; DMD, Duchenne muscular dystrophy; FSHD, fascioscapulohumeral muscular dystrophy; HSMN, hereditary sensory motor neuropathy; IBM, Inclusion body myositis; LGMD, limb girdle muscular dystrophy; PwNMD, person living with a neuromuscular disorder; SMA Spinal muscular atrophy.

TABLE 3.

Examples of patient, caregiver, or clinician reported hand function assessment measures for PwNMD.

Measure	Populations	Measurement characteristics
DASH (Disability of Arm, Shoulder and Hand scale) DASH‐W (work), DASH‐SM (sport, music)	HSMN, IBM 35 , 39	Patient self‐report 30‐item questionnaire of upper limb disability Added questions related to work, sports and musical instrument play
Disease‐Specific Functional Rating Scales (FRS)	Ex: IBM, ALS (−revised), SMA 56 , 57 , 58	Patient self‐report or clinician 10‐item questionnaire to assess common ADL functions. Some but not all items pertain to the upper limbs (0–4 score on each item) Not sensitive to hand function changes until more severe weakness present in IBM
IBM‐PRO	IBM 39	Patient self‐report 12‐item self‐report questionnaire of perceived difficulty with daily hand and arm functional activities (0–4 score on each item) Higher correlation with grip and pinch strength than IBM‐FRS
DMD upper limb patient‐reported outcome measure	DMD 59	Self or caregiver report ‐ 32‐item questionnaire measuring four domains of ADL function

Abbreviations: ADLs, activity of daily living, ALS, Amyotrophic lateral sclerosis; DMD, Duchenne muscular dystrophy; HSMN, hereditary sensory motor neuropathy; IBM, Inclusion body myositis; SMA Spinal muscular atrophy.

Neuropathies that cause sensory loss can worsen fine motor incoordination and impair protective sensation due to hyper or hyposensitivity to touch, pain, proprioception, and temperature. Object manipulation relies on accurate tactile perception and precise grasp force through synergistic control of multiple joints and sensory feedback about an object's characteristics such as texture, shape, weight, and slipperiness. ⁵⁸ Therefore, changing object characteristics to a slip‐resistant surface or interface and modifying task parameters (speed, repetitions, weight) may improve function and reduce fatigue with hand activities. In CMT neuropathies variable loss of large myelinated, thinly myelinated A δ sensory axons and unmyelinated C fibers has been demonstrated. Neuropathic and/or nociceptive hand pain was described by Laurà ⁵⁹ and Bjelica ⁶⁰ in 22%–40% of their respective cohorts of CMT type 1A patients. Sensory evaluation in CMT may include joint position sense, vibration, two‐point discrimination, the DN4 and painDETECT questionnaires to evaluate neuropathic pain, and quantitative assessment with Semmes‐Weinstein monofilaments (SWMF). ⁵⁹ , ⁶¹ , ⁶² Both sensory function and strength correlated with hand dexterity, though muscle strength was found to be a better predictor in a SWMF reliability study in CMT patients. Some examples of compensatory strategies and adaptive equipment to optimize hand function and safety due to sensory loss are included in Tables 4 and 5.

TABLE 4.

Assistive devices to improve activities of daily living for PwNMD with reduced hand function.

Activity	Adaptive equipment ideas to consider (sensory protection/friendly ideas in italics)
Feeding	Large handled/built‐up, or rubber/nonslip utensils High‐sided bowls, plate guards, suction/nonslip plates/bowls, nonslip mats Travel mugs with large handles and lids, two‐handed mugs Weighted and/or angled cutlery for ataxia Spill proof cups
Dressing	Large‐teeth plastic zippers, elastic waistbands, magnetic/Velcro closures for clothing Slip‐on shoes, elastic laces, dial/velcro closure for shoes/boots, AFO‐friendly shoes, button hooks, zipper pulls, shoehorns, dressing aids for hooks (e.g., bras and jewelry), sock aids, doff and donner for compression socks
Toileting	Adaptive clothing with back openings Bidet or wash/dry toilet, long‐handled wiping aids and sponges Spill‐proof urinals Touch/motion‐activated faucets/soap dispensers
Grooming	Electric toothbrushes and razors, strap‐fitted hairbrushes Long handled/mounted nail clippers, brushes/combs Suction cup hair dryer holder
Bathing	Thermo‐regulating devices/preset temperature controls on taps, tap turners Long‐handled sponges Toweling robes Mirrors/selfie sticks to regularly checks for wounds, lotion for hands to prevent drying/cracking skin
General	Universal Cuff Paddle‐shaped button pushers Stationary scissors Phone and tablet mounts Nonslip mats

Abbreviations: AFO, ankle foot orthosis; PwNMD, person living with a neuromuscular disorder.

TABLE 5.

Assistive devices to improve participation in instrumental activities of daily living, vocational activities, and leisure for PwNMD with reduced hand function.

Participation	Adaptive equipment ideas to consider (sensory protection/friendly ideas in italics)
Meal preparation	Rubber/nonslip material for jar/bottle opening, electric can openers, automatic jar openers Rocker knives, pizza cutters, electric knives, adaptive/one‐handed cutting boards, vegetable chopper/dicer Wheeled cart Oven mitts/cover hands for skin protection while cooking/baking
Medication management	Automated medication dispensers Pill organizers, Blister packs, rubber bottle opener
Home maintenance	Environmental controlled (voice, microswitches, phone apps) lights, media, doors, window shades, Robotic vacuum, Wheeled laundry cart, Electric tools (e.g., screwdrivers) Doorknob extenders, tap turners Rubber gloves for dishwashing and protective gloves for handling products that can harm skin, such as bleach
Banking and shopping	See Section 7 for computer/phone access Automatic bill payments, contactless credit cards Wheeled cart, shoulder bags
Vocational or educational	Workplace/station accessibility; ergonomic assessments (see Section 7) Note takers at schools/universities
Community mobility	Automatic doors and elevator controls with motion sensors or app‐based opening Driving: Steering knobs, seat belt helpers, key levers
Leisure	Playing card holders, card shufflers Book holders, page‐turning devices, rubber thimbles Adaptive chord players for guitar playing, T strap for golfing, Bowling/bocce ramps Adapted gaming controls, antidrop hand straps for controllers Use sunscreen or UPF 50+ sun protective clothing to prevent burns book holder, page turner, thimble, and remote control page turner for e‐readers Voice activated remotes
Relationships	Fashion‐forward adapted clothing Sex toys incorporated with hand orthoses, positioning straps, automatic action

Abbreviation: PwNMD, person living with a neuromuscular disorder.

In an era of more accessible technology, new tests and tools in development have the potential to remotely monitor biometrics in real time, for more accurate reflection of daily function or performance through wearable devices, ⁶³ such as the SEGT (sensor‐engineered glove test). ³⁵ , ⁶⁴ Disease‐specific patient‐reported outcomes may reduce the burden on annual clinic visits, and capture important preferences and values of the person with lived experience. ⁶⁵

4. THE POSITIVE ROLE OF EXERCISE

In general, moderate‐intensity aerobic, low–moderate‐intensity resistance (strength) training, flexibility (ROM, stretching) and proprioceptive training is recommended for pwNMD. Exercise should be targeted and closely monitored by healthcare providers due to a lack of high‐level evidence for optimal exercise prescription. Recommendations should be individualized and evolve based on underlying disease, stage, progression, impairments, prognosis, and functional limitations. ⁶⁶ , ⁶⁷ To increase adherence, key elements of any exercise prescription should clearly specify the type, intensity, frequency, duration, and level of supervision as well as identify the individual's preferred forms of physical activity. ⁶⁸ More personally relevant, enjoyable activities, and motivation through music or the social nature of groups can also be explored when individualizing care. Further adjustments to the prescription should occur based on response to training and the general rule that ADLs should not be negatively impacted. Adherence to physical activity has also been increased in studies of ALS, postpolio, FSHD, and myotonic dystrophy with the use of cognitive behavioral therapy. ⁶⁸ Patient‐centered approaches aiding self‐management are important, and can also improve job satisfaction in health care professionals. ⁶⁹

Hand rehabilitation programs should include stretching/ROM of the wrists, fingers, and thumbs in all movement planes, strengthening of intrinsic and extrinsic hand muscles, functional fine motor, and manipulation skills practice. Some patient organizations have excellent practical guides outlining useful tools such as therapeutic putty, clothes pins, scissors, rubber bands, bubble wrap, or tongs, and engaging in activities such as instrument playing (piano, flute), sign language, or games and crafts that include blocks, cards, beads, lacing, manipulating marbles, golf, or Baoding balls. ⁷⁰

A recent systematic review and meta‐analysis by Stefanetti et al. ⁷¹ of 130 articles with over 1800 patients and 35 different forms of NMD concluded that exercise training appears to be safe across a range of outcomes. The review mainly reported on adult patients, with few studies specifically measuring upper extremity outcomes limited to hand grip and isometric elbow flexion strength by handheld dynamometry. Djordjevic et al. ⁷² observed that self‐directed exercise is associated with greater elbow flexion strength in both CMT types 1 and 2, but found no association of exercise with hand grip. In DM1, an association was found between physical activity and stronger hand grip in those who were active versus sedentary. ⁷³ More recent reviews of exercise training programs in DM1 have confirmed the safety of supervised and home exercise programs, but physiological adaptation and optimal prescription are still under investigation. ⁴³ , ⁷⁴

Few studies have addressed formal rehabilitation programs aimed at improving hand function in pwNMD. Prada et al. ⁷⁵ designed a 4‐week, 2×/week, 45‐min hand rehabilitation program including submaximal effort muscle strengthening, stretching, and proprioceptive exercises, and subsequently conducted an open‐label pilot study of nine participants with genetically confirmed CMT. One‐week pre‐ and posttreatment outcome measures included hand grip and tripod pinch dynamometry, thumb opposition test, and Sollerman Hand Function Test. The program was well tolerated, and all measures showed some improvement, especially the dominant hand.

An initial pilot study of hand training in five pwDM1 demonstrated improved muscle power and dexterity. A follow‐up single‐blind crossover randomized control trial of a 12‐week hand training program in 35 adults with DM1 showed significant positive intervention effects on wrist flexor isometric force, self‐perception of occupational performance, and satisfaction without adverse effects in an intention‐to‐treat analysis. The intervention included silicon‐based putty strength, endurance, and stretching exercise. Limitations of the study were a low initial recruitment rate, low training adherence, and high dropout rate. ⁷⁶ , ⁷⁷

5. GRIPS AND ORTHOSES

Upper extremity use depends on functional grip, hand dexterity, and the ability to position its different segments in space. The distal upper extremity “position of function” places the wrist in ~20° extension, and approximate flexion of MCPs, proximal and distal IPs at 45°, 30°, and 20°, respectively, with the thumb straight and relaxed. ⁷⁸

With progressive intrinsic muscle atrophy, the hand adopts a clawed, “intrinsic minus” position. Wrist extension weakness further contributes to this nonfunctional configuration, ⁷⁸ impairing ability to perform key types of grip, which are typically divided into two main types: power and precision (or pinch), ⁷⁸ as illustrated in Figure 2. Orthotic devices, defined as “externally applied device[s] used to compensate for impairments of the structure and function of the neuromuscular and skeletal systems” ⁷⁹ aim to maintain or support one's capacity to perform these grips (Figure 3).

FIGURE 3.

Examples of hand and wrist orthoses—assistive, protective, corrective, and compensatory. (A) Protective—carpal tunnel syndrome or neutral wrist. (B) Protective and corrective—resting wrist hand. (C) Assistive—volar cock‐up wrist. (D) Assistive—dorsal cock‐up wrist. (E) Protective and assistive—rigid short opponens. (F) Assistive—short opponens. (G) Corrective and assistive—proximal interphalangeal joint hyperextension block. (H) Corrective and assistive—metacarpophalangeal extension block. (I) Compensatory—Universal cuff. Illustration by Myra Rudakewich, MScBMC.

Evidence for orthoses in neuromuscular diseases remains anecdotal, possibly related to research funding availabilities and methodological challenges for efficacy studies of orthoses. A review of wrist‐hand orthoses in motor neuron disease found only 5 case reports and 17 expert opinions on the subject, ⁸⁰ and the only CMT study we found was a 13‐participant, 5‐week trial. ⁸¹ In the authors' clinical experience, orthoses can be beneficial for hand function. These devices, which may be static, dynamic, or hybrid, have three main objectives: protect (such as a carpal tunnel orthosis Figure 3A), correct, and/or assist function ⁷⁸ Upper limb orthotic devices are often more complex and variable than lower extremity orthoses, and may be used for specific tasks requiring both gross and fine motor abilities. ⁸²

Resting wrist‐hand orthoses (Figure 3B) are often a mainstay of contracture prevention and management. They aim to maintain the hand in a functional or “intrinsic plus” position, with the wrist neutral or slightly extended, MCPs flexed, and IPs extended, to prevent clawing. ⁸⁰ Recommendations on wear vary; many advise at least every other night, ⁸³ but some state minimally five times a week is required to be effective. ⁸⁴ A study on teenagers with DMD who previously demonstrated contracture progression reported maintenance, and even improvement, over 8 months with nighttime orthoses (alternating sides each night); benefit, however, was only maintained long‐term in patients who continued utilization. ⁸⁵

Various orthoses assist function. Wrist extension supports (Figure 3C,D) can improve grip strength ⁷⁰ , ⁸⁰ and functional reach. ⁸⁶ Thumb opposition and tripod pinch strength are fundamental aspects of manual dexterity, ²⁸ and can be aided by positioning orthoses such as thumb opponens (“spica”) splints or spacers (Figure 3E,F). ⁷⁰ , ⁸¹ , ⁸⁷ Finger orthoses stabilize hypermobile IP joints for fine movements (Figure 3G). ⁷⁰ Some hand orthoses are designed to reduce clawing by limiting MCP hyperextension (Figure 3H). ⁷⁸ Many of these aspects can be combined into a single device.

Dynamic arm supports can be attached to a power wheelchair or stand to help move the limb in a gravity‐eliminated plane. Such support can improve endurance and independence for activities such as feeding ⁸⁸ , ⁸⁹ and personal hygiene. However, the devices can be expensive, cumbersome, interfere with wheelchair control, ⁸⁹ and disease progression may prevent long‐term use, ⁸⁸ necessitating careful evaluation before prescription. For some individuals with ataxia, weighted items and cuffs may be beneficial during activities such as writing or eating, but may also contribute to increased fatigue; evidence is equivocal. ⁹⁰ Finally, orthoses can support the use of other assistive devices, ⁸² such as universal cuffs (Figure 3I).

Despite their usefulness, compliance with orthotic devices is often low. ⁸³ Collaboration with experienced occupational therapists and orthotists is essential, as setting clear goals and expectations, and optimizing fit, comfort, weight, ease‐of use, and proactive troubleshooting can facilitate adherence. ⁸³

6. ACTIVITIES, INDEPENDENCE, AND PARTICIPATION

ADLs encompass the various tasks involved in fulfilling one's usual needs. They are customarily divided into basic ADLs, for managing physical needs, such as eating, dressing, and bathing, and instrumental ADLs, comprised of the more elaborate tasks for autonomy in domestic (e.g., managing medication, meal preparation) and community (e.g., driving, shopping) settings. ⁹¹ Performance of these activities requires sufficient hand strength, endurance, ROM, movement control, sensation, and cognition. ⁹ Hand function is thus critical to maintaining independence, which extends beyond the basic ADLs, to meaningful social, vocational, and leisure participation.

Overall, literature is sparse on how adults with neuromuscular disorders participate in daily life activities, and what interventions might optimize participation. Recommendations by treating therapists are mostly guided by experience, and published best practices are predominantly consensus‐based, informed by clinical experience and expert opinion. ⁹⁰ A qualitative study among persons living with SMA identified the desire to live a normal life and fully participate in social activities including family and work roles. ⁹² However, a survey study of 62 adults with SMA (types 1–4) in the Netherlands found perceived restrictions among 97% in household chores, 66% in visits to family/friends, and 67% in work/education. Among the participants, 31% expressed dissatisfaction with going out and outdoor activities. ⁹³

Assessing and addressing a person's participation in life's activities requires a personalized approach consisting of individual assessment, partnering with pwNMD, caregivers, and interprofessional teams to identify the priorities, goals, and to problem solve. There is a wide array of strategies, including equipment, techniques, supports to optimize independence and facilitate participation. ⁹⁴ Interventions must be tailored to pwNMD and caregiver preferences, finances, insurance allowances, supports, and living environment. For progressive conditions, proactive care and emphasis on planning ahead is crucial, especially when considering home modifications and substantial equipment for which significant investments and potential delays are foreseeable.

Energy conservation strategies are essential for all, as fatigue is very common in most NMDs. ⁹ , ⁹⁵ In a large study, 90% of pwALS reported fatigue; although this was the most common symptom it was rarely treated. ⁹⁶ Prioritizing, pacing (allowing enough time and taking rest breaks), sitting, and adaptations can help reduce fatigue and improve endurance, ⁹⁷ and preserve energy for more enjoyable or priority activities. Some tasks can be accomplished either with assistance, or by delegation; this is not only dependent on capabilities, but also individual preferences.

Of note, educating and training caregivers and families on hands‐on care skills and techniques have been shown to reduce stress as caregiver confidence in this area is a major concern. ⁹⁸ While task delegation may be necessary due to physical limitations, individuals can enjoy meaningful participation; autonomy and inclusion can be realized in everyday activities such as deciding on menus and wardrobe, self‐directing care, and managing household ²² and work responsibilities.

The following section illustrates principles of various adaptations that may be useful in taking advantage of neuromuscular patients' strengths, while compensating for limitations. Please refer to Tables 4 and 5 for specific examples of adaptive equipment. Occupational therapists are experts in the areas of self‐care, productivity, and leisure, as well as hand function, and should be consulted when addressing challenges in this domain. Many aids described may be available through nonmedical sources; for example, a Bluetooth‐enabled doorbell used as a call bell can be found in hardware stores. Patient support associations may have resources to identify or access regionally available adaptive tools, including potential funding options or equipment loan programs.

There exists a wide variety of adaptors for all kinds of objects, from keys to zippers to mugs. The same adaptive principles are relevant across many daily situations. A variety of hand straps, such as universal cuffs, can be used or custom fabricated to improve grip. These items provide low‐tech, inexpensive options to compensate for finger function loss, allowing for independent use of utensils, styluses, and the like.

Larger handles with anti‐slip texture, either premade or built‐up with foam, make items easier to handle. ⁸⁶ Mouldable material or 3D printing can be used to optimize the shape or size of objects to improve ease of use in a very customizable and individualized manner. A table or counter raised at chest height can compensate for proximal weakness and stabilize the arms in a gravity‐reduced position when eating, grooming, or working. Limited clutter and optimized workspace/utensil organization (keep items at reachable heights) can simplify tasks. ⁹⁰ Devices that hold or stabilize items allow patients to focus on dexterity and the activity itself.

In the home, lever faucets and paddle handles are preferable to knobs. ⁹⁰ Rolling carts can help transport items in the kitchen or between rooms. Rubber jar openers, nonslip, self‐adhesive strips, or rubber gloves allow for better friction and can provide protection to avoid dropping objects, especially in the case of sensory loss. Electric razors and toothbrushes have a larger grip and minimize the need for rapid alternating movements. ¹⁰ A bidet or wash/dry toilet can help with perineal hygiene, and many people find it improves dignity ⁹⁷ ; portable add‐ons are available as well. ⁹⁹

Dressing requires both sufficient proximal upper extremity ROM and hand strength and dexterity to don and doff items and manage fasteners. ¹⁰⁰ Buttons and conventional shoelaces are often challenging, some fabrics are easier to manage, ¹⁰¹ and equipment can facilitate the process (see Table 4). Patients show a high degree of satisfaction with slip‐on shoes, ¹⁰² and some sneakers are ankle foot orthosis‐friendly.

Most pwNMD require some form of mobility aid; however, hand weakness and, in some cases, upper extremity contractures, can impede their safe use. ¹⁰³ Offset canes are usually easier to grip, and their handles can be built up for further ease. ¹⁸ Rolling walkers require significantly less energy than standard ones, ⁸⁶ but care must be taken to ensure ability to grip the brakes; if this is an issue, a two‐wheel walker, slowdown brakes or push‐down brakes can be an alternative. ¹⁰⁴ Forearm walkers can offload the hands and improve posture. ¹⁰⁵ Wheelchairs are ubiquitous in neuromuscular populations and can tremendously enhance QOL by increasing independence, reducing pwNMD and caregiver strain and fatigue, and improving social participation. ¹⁰⁶ Manual wheelchairs are rarely a long‐term solution due to upper extremity weakness, but power assists can be helpful for select individuals. Motorized, or power wheelchairs (PWCs), can be independently controlled even with very limited hand function, using control options such as joysticks (different shapes, sizes, and sensitivities) that may be operated by hands, feet or chin, switches, head arrays, and even eye gaze control, among others. ⁸⁶ , ¹⁰⁶ , ¹⁰⁷ Attendant controls can be added as well. PWCs allow position changes (tilt for resting and pressure relief, raising the legs, etc.), provide support for postural deformities if required, and can accommodate equipment such as ventilators, mobile arm supports, and environmental controls (see Section 7). Persons with neuromuscular disease report high levels of satisfaction with PWCs, ⁸⁶ and maintaining the ability to operate the chair is a major concern for many. ²²

7. TECHNOLOGY ACCESS

From participating in social networks and computer games for leisure, to using handheld digital devices and computers for work or healthcare access, our world is becoming increasingly dependent on technology. Hand impairments as a barrier to technology use for people living with neuromuscular disease may also lead to increased loneliness and poor mental health. ¹⁰⁸ This section will describe adaptive technologies for pwNMD to access computers and handheld digital devices. These assistive technologies can be accessed through occupational therapy, augmented and assisted communication clinics, and for some devices/software, purchased directly online.

7.1. Cursor control

Several adaptive options are available to facilitate cursor control for people with hand weakness. Instead of a mouse, easier‐to‐use touchpads, trackballs (Figure 4A), joysticks, and touch screen monitors ¹⁰⁹ , ¹¹⁰ are readily available from big‐box consumer electronics retailers. In addition, switches (Figure 4B) operated by either a limb or via a sip and puff mechanism can be used in conjunction with software or a mouse emulator to allow users to navigate and control their device. ¹¹¹

FIGURE 4.

Examples of assistive devices to facilitate cursor control. (A) Trackballs. (B) Switches. (C) Head pointer.

For those who are unable to use the aforementioned options for cursor control, eye tracking is an alternative. Infrared light shines on a user's eye, and the resulting inputs sent to a sensor are then triangulated by an algorithm to determine the movement of the user's eye. ¹¹² Similarly, head tracking (Figure 4C), which uses either infrared light with sensors or cameras ¹¹² , ¹¹³ to translate head movements can be used for cursor control. Head pointing also leverages head movements, but instead mounts low‐intensity lasers on the head which then activates a laser‐sensing screen. ¹¹⁴ Finally, brain–computer interfaces are being developed which can translate EEG signals into cursor movements ¹¹⁵ for clinical use in pwNMD.

7.2. Keyboard input

A number of low‐ and high‐tech options exist for people with hand weakness to facilitate or improve keyboard input. At the lowest technological level, hand orthoses for typing (Figure 5A) use sticks with a regular keyboard to help translate proximal limb movements into keyboard input. ¹¹⁶ The sticks can be strapped to the hand or be in the form of a wraparound “mitten.” People without proximal upper limb function can use mouth sticks, which are sticks for keyboard input attached to a bite plate that the user holds on to with their mouths. ¹¹⁷ For those who have decreased oral control or a need to mouth‐breathe, a pointer can be strapped on to the user's head for typing. ¹¹⁷

FIGURE 5.

Examples of assistive devices to facilitate keyboard control. (A) Orthosis for typing. (B) A keyguard on a standard‐sized keyboard is shown in the front. In the back, there is an accessible touchscreen keyboard which requires less hand strength to operate on the left, and a number pad that was reconfigured as a mouse to use with an on‐screen keyboard on the right.

To prevent keyboarding errors from finger weakness, tremor or ataxia, a low‐tech solution is a rigid keyguard (Figure 5B) with holes for each key, which also has the benefit of promoting hand stability by allowing a weak distal limb to rest directly on the keyboard while typing. ¹⁰⁹ Higher tech accessible keyboard options (Figure 5B) include ergonomic keyboards that are split or contoured to reduce wrist movement, keyboards with larger keys, smaller keyboards with fewer keys, touchscreen keyboards that require less force to operate, and chording keyboards with few keys that can be pressed in different combinations for input. ¹⁰⁹ Virtual keyboards can also be used with the cursor control options described in the previous section. ¹¹⁸

Speech‐to‐text software such as Dragon, ¹¹⁹ Windows 10 Speech Recognition ¹²⁰ and Apple Dictation ¹²¹ can be used as an alternative to keyboarding. People living with dysarthria can feasibly use speech‐to‐text systems designed for the general population, ¹²² with training that focuses on pausing between words, increasing breath control and volume, and practicing with the systems. Google's Project Euphonia ¹²³ is actively conducting research into speech recognition models for people with dysarthria, and there are also smartphone dictation apps in development such as SPEESH. ¹²⁴ Word prediction to assist with keyboarding is another assistive software approach which has been shown to increase text input speed, ¹²⁵ and can be customized for frequency of prediction and number of words predicted.

7.3. Smartphone use

Android and iOS operating systems for smartphones both offer accessibility features that may help pwNMD experiencing hand weakness. iOS and Android have AssistiveTouch and Gesture features respectively, which helps to simplify the swiping and pressing motions needed to operate a smartphone. These features allow simpler hand motions such as double tapping the phone and other custom gestures to activate frequently used actions such as turning on the camera or switching between apps. ¹²⁶ , ¹²⁷ Both phone operating systems also support switch control, and have a head/facial control feature that allows the smartphone user to control the cursor via head movements, and assign custom actions to facial expressions such as smiling or sticking out the tongue. ¹²⁸ , ¹²⁹

Some people with hand weakness benefit from an adaptive stylus (Figure 6) for use with a smartphone touch screen that allows use of the weight of their hand rather than a single digit that might buckle when swiping, tapping, or using a virtual keyboard. Various off‐the‐shelf options exist, ¹³⁰ or a stylus can be customized for diameter, grip shape, weight and flexibility or as a mitten‐style stylus. ¹³¹ The recent expansion of 3D printing technologies also allows for adaptive styluses to be printed using conductive filaments. Similarly, people with hand weakness may have difficulty holding on to their smartphones, and there are a variety of mass‐produced and custom smartphone case, mount, and stand options. ¹³² , ¹³³

FIGURE 6.

Two examples of 3D‐printed adaptive styluses. Left: “Mitten,” also known as a “sixth digit”, stylus that is worn around the metacarpophalangeals. Right: Palm grip stylus.

7.4. Voice activation technologies

Voice activation technologies are very helpful in limited hand function. In one survey of 171 pwNMD, voice activation was the most desired control method for assistive devices among participants. ¹³⁴ Voice assistants such as Siri ¹³⁵ and Google Assistant ¹³⁶ can be used to control smartphones and tablets. There are smart voice speakers such as Google Nest ¹³⁷ and Amazon Echo ¹³⁸ which can provide information and facilitate communication (e.g., make phone calls) and can also be paired with home appliances, thermostats, and lights. ¹³⁹ , ¹⁴⁰

However, speech recognition algorithms may not recognize dysarthric speech thus posing a barrier for use when neuromuscular disorders affect speech production. A workaround while voice‐activation technologies become adapted for dysarthria is to use augmented and assisted communication devices or apps to speak the commands. Another approach is to use an application such as CapisciAme ¹⁴¹ and Voiceitt ¹⁴² that links with voice assistants and smart home applications by recognizing users' uniquely pronounced voice commands.

8. PHARMACOLOGICAL AND SURGICAL CONSIDERATIONS

Pharmacological treatments have a small role in improving hand function in pwNMD. People affected by ALS may have distal upper limb spasticity, for which guidelines recommend antispasticity medications such as baclofen and tizanidine. ¹⁴³ Originally considered a contraindication, chemodenervation with botulinum toxin for people affected by ALS can be used safely with proper precautions and dosing ¹⁴⁴ ; drug monographs still advise “close monitoring” for pwNMDs. Extrapolating from evidence in other populations with spasticity, ¹⁴⁵ botulinum toxin can be considered as an option for distal upper limb spasticity affecting hand function in ALS. However, while FA occasionally causes spasticity in the upper limbs, available guidelines recommend against botulinum toxin for the upper limb. ¹⁴⁶ Many neuromuscular diseases are associated with either neuropathic or nociceptive pain, ¹⁴⁷ , ¹⁴⁸ , ¹⁴⁹ and pain has the potential to significantly reduce function. As an example, we can extrapolate from the evidence for musculoskeletal conditions in the general population to offer corticosteroid injections, topical and oral nonsteroidal anti‐inflammatory drugs, duloxetine and acetaminophen, ¹⁵⁰ or surgical consultation to consider candidacy for ligament reconstruction, tendon interposition, arthroplasty, or arthrodesis ¹⁵¹ for pwNMD with pain from OA. Mexiletine may be helpful for functionally limiting grip myotonia based on the results of one randomized controlled trial of 42 participants with DM1 who experienced an improvement in objectively measured hand grip myotonia; cardiac evaluation should be considered prior to use, given its contraindications or cautions for use in patients with conduction abnormalities and heart failure. ¹⁵² , ¹⁵³ As disease‐modifying medications have become available for some conditions in certain countries, such as nusinersen, onasemnogene abeparvovec, and risdiplam in SMA, there is now potential for improvement and/or stabilization in hand function, ¹⁹ highlighting the importance of identifying and using valid and sensitive measures to assess hand outcomes. DMD is another example with new approved therapies such as vamorolone, delandistrogene moxeparvovec, and givinostat. ¹⁵⁴ , ¹⁵⁵ , ¹⁵⁶ There are also many targeted therapeutics in development thanks to recent advances in molecular biology. ¹⁵⁷ These are outside of the scope of a review focused on rehabilitation care, but we wanted to acknowledge these options for interested readers.

Surgical procedures for improving hand function are rarely considered in neuromuscular disease, and there is little literature available to guide decision‐making. An experienced hand surgeon familiar with neuromuscular patients should be consulted, in collaboration with the pwNMD, their caregivers and rehabilitation team. Personal goals, stability of disease, adequate support in the perioperative period, and ability to follow through on postoperative care and rehabilitation are important factors in selecting surgical candidates. Contracture release may be employed on rare occasions when nonsurgical treatments have failed and there is a clear attainable functional goal or prevention/treatment of complications identified. ⁴¹ In the case of CMT and polyneuropathy due to distal to proximal progression of muscle weakness, surgery is palliative but has potential to prolong dexterity and autonomy. Thumb and index instability, pinch weakness, lack of opposition, and synchronized finger movements as well as clawing are primary concerns that may benefit from surgical consultation following initiation of conservative options. Dynamic procedures can be considered for patients with strong wrist muscles. Forearm muscle weakness follows preferential involvement of median and ulnar innervated intrinsic muscles in CMT, thus radial muscles are least affected early on, and are most useful for tendon transfers. Additional considerations: donor muscle‐tendon units will lose one grade of strength after transfer, work best when synergistic with recipient muscles, and force is proportional to muscle cross‐sectional area at rest. Pre‐operative EMG is helpful to confirm the health of donor muscles during surgical planning. ³⁰ , ¹⁵⁸ , ¹⁵⁹

Opponensplasty procedures have been used successfully to improve thumb opposition in CMT and low median neuropathy, such as radial innervated ECU to EPB and EIP to APB, or fourth digit FDS to APB transfers. The Zancolli lasso dynamic procedure for claw fingers divides FDS at its insertion on the middle phalanx, loops around the A1 pulley and is sutured onto itself to create flexion at the MCP joints when there are limited donor tendons and active extension is observed in IP joints when MCPs are flexed. A suggested alternative is the Brand ECRB transfer with grafting. Only static procedures can be used in patients without active wrist or hand muscles as joint stabilization is the goal and weakness cannot be corrected. Examples include thumb metacarpal, trapeziometacarpal, and IP joint fusions, or Zancolli volar plate advancement and various tenodeses for finger clawing. ³⁰ , ¹⁵⁸ When clinically indicated, nerve entrapment release may also be performed in select patients as discussed previously in this review.

In the case of IBM, a recent scoping review of the literature and survey by Hua et al. ¹⁶⁰ identified three case reports of wrist extensor tendon transfers treating finger flexion weakness. The authors distributed a survey worldwide to Myositis Support and Understanding (MSU patient advocacy group) members to assess patient perception and expectations of tendon transfer surgery on hand function. In total, 470 participants responded with 232 self‐identifying as pwsIBM. Of those, 193 respondents fully completed the surgical questionnaire and associated patient–reported outcome measures. Only 8% indicated they had been referred to hand surgeons, despite 54% of respondents reporting they would consider hand surgery if benefits lasted 1–2 years.

Overall, there is a paucity of evidence on optimal timing, procedures and long‐term results of surgical interventions in pwNMD of a progressive nature, but further investigation is warranted.

9. THE NEUROMUSCULAR CLINICIAN AS ADVOCATE

Besides working with pwNMD, caregivers and the interprofessional team to assess and address functional limitations, barriers to participation and QOL issues related to hand impairments; it is also important for neuromuscular clinicians to advocate for increased accessibility and help educate the public and policy makers against stereotypes and negative attitudes that stem from ableism. Of course, the priorities of and actions for such advocacy and education efforts by clinicians must be informed by people with lived experience.

Taking healthcare settings as an example, there are numerous barriers to access for people with hand impairments such as self‐check‐in kiosks, pre‐appointment patient questionnaires on paper, booking appointments online using a computer, donning a mask without assistance when required for infection control reasons, and even turning a door handle to access a clinic. Clinicians should advocate for universal design and encourage healthcare organizations to accommodate by upgrading appointment booking systems to accessibility standards, ¹⁶¹ making available administrative staff or volunteers to assist with appointment check‐in, questionnaires and masking, installing touchless access elevators and automatic doors, and the judicious use of telemedicine and telerehabilitation options. ¹⁶²

The United Nations Convention on the Rights of Persons with Disabilities ¹⁶³ came into force in 2008, and includes the principle that persons with disability have the right to “full and effective participation and inclusion in society.” To truly improve community participation, clinicians should advocate for the development of and adherence to municipal, regional, and national policies or legislation supporting accessibility and inclusion. At the person level, clinicians can facilitate participation in school and work through recommending appropriate accommodations for hand function such as extra time for tests, access to note takers, accommodative workstations, and rest break allowances.

We fully recognize that many socioeconomic barriers exist in terms of access to the assistive devices and technologies described in this review. The World Health Assembly in 2022 adopted a resolution that called for strengthening rehabilitation in health systems, ¹⁶⁴ calling for universal health coverage inclusive of rehabilitation and assistive technologies. Neuromuscular clinicians should consider advocating at a macro‐system level for government and insurance programs that fully meet the needs of people living with neuromuscular disorders.

10. CONCLUSION

When I got back home from a 10‐week ICU (intensive care unit) visit, I was no longer able to drive my power wheelchair. I never realized that my wrist and hand was solicited to just drive my chair and move around. I then realized how important hands, fingers and wrists were and taken for granted. 1 hand, 1 wrist was my complete autonomy. Losing the little strength I had left in my right hand would make me so much more dependent of others. Luckily, my story allows me to gain some back after starting nusinersen. Gaining a little strength back in my hand and wrist allows me to be so much more independent. My hand and wrist allow me to use a computer and my phone on my own. I can work, read, do researches, keep contacts with friends… I feel complete, useful and never alone. I guess I can say that a big part of my autonomy is at the end of my fingers… 45‐year‐old female affected by SMA and advocate

The hand is important in all aspects of our lives. A broad range of neuromuscular disorders including polyneuropathies, motor neuron diseases, and muscle disorders can cause hand and wrist impairments, which limits participation in self‐care, occupation, leisure activities, communication, social participation, and even healthcare access. Hand function should be assessed on a regular basis in pwNMDs. There is emerging evidence for the role of exercise in improving hand function and there are many adaptive devices to assist with daily activities and technology use which can be custom‐made or purchased off‐the shelf. Clinicians should get “all hands on deck”, partnering with persons with lived experience, family, caregivers, and allied health professionals such as occupational therapists to develop personalized solutions to improve hand function and QOL. More research is needed to identify the best modalities and optimal exercise prescriptions for people with different kinds of neuromuscular disorders, and the impact of assistive devices on functional outcomes and QOL. Beyond the clinic, neuromuscular clinicians should contribute to advocacy for increased accessibility to facilitate full participation and inclusion for people living with neuromuscular diseases.

AUTHOR CONTRIBUTIONS

All authors contributed to the conception, writing, reviewing, and editing of the manuscript.

CONFLICT OF INTEREST STATEMENT

The authors declare no conflicts of interest.

ETHICS STATEMENT

We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.

O'Connell C, Guo M, Soucy B, Calder M, Sparks J, Plamondon S. All hands on deck: The multidisciplinary rehabilitation assessment and management of hand function in persons with neuromuscular disorders. Muscle & Nerve. 2025;71(5):869‐888. doi: 10.1002/mus.28167

DATA AVAILABILITY STATEMENT

Data sharing is not applicable–no new data generated.