Rehabilitation interventions targeting the activity and participation of patient with neuromuscular diseases: what do we know? A systematic review

Silva, Sionara Ferreira; Magalhães, Hugo Leonardo de; Deus, Franciele Angelo de; Andrade, Keysy Karoline Souza; Lima, Vanessa Pereira; Gaiad, Thaís Peixoto

doi:10.1055/s-0044-1779295

Sionara Ferreira Silva

conceptualization

data curation

formal analysis

investigation

writing – original draft

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Faculdade de Ciências Biológicas e da Saúde, Departamento de Fisioterapia, Diamantina MG, Brazil.

http://orcid.org/0009-0008-6356-2365

Hugo Leonardo de Magalhães

conceptualization

data curation

formal analysis

writing – original draft

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

http://orcid.org/0000-0002-8156-0637

Franciele Angelo de Deus

formal analysis

investigation

methodology

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

http://orcid.org/0000-0002-8028-0916

Keysy Karoline Souza Andrade

formal analysis

writing the original draft

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

http://orcid.org/0000-0002-9979-8985

Vanessa Pereira Lima

conceptualization

formal analysis

methodology

supervision

writing - review and edit

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Faculdade de Ciências Biológicas e da Saúde, Departamento de Fisioterapia, Diamantina MG, Brazil.

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

http://orcid.org/0000-0002-0349-3248

Thaís Peixoto Gaiad Address for correspondence Thaís Peixoto Gaiad (email: thais.gaiad@ufvjm.edu.br)

conceptualization

formal analysis

project administration

supervision

writing - review and edit

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Faculdade de Ciências Biológicas e da Saúde, Departamento de Fisioterapia, Diamantina MG, Brazil.

Universidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

http://orcid.org/0000-0002-4688-330X

Address for correspondence Thaís Peixoto Gaiad (email: thais.gaiad@ufvjm.edu.br)

Conflict of Interest

There is no conflict of interest to declare.

Domains	Definition according to ICF²
d3–Communication	Communication through language, signs and symbols, including receiving and producing messages, maintaining conversation and using communication devices and techniques.
d4–Mobility	Movement when changing the position or location of the body, transporting, moving or handling objects from one place to another, walking, running or going up/down and using different forms of transport.
d5–Self care	Self-care such as washing and drying, taking care of the body and body parts, dressing, eating and drinking, and taking care of one's own health.
d6 - Domestic life	Shopping, storage, positioning of materials, food, clothing, planning and preparing meals, doing household chores, cleaning utensils and equipment, removing trash, helping family members.
d7–Interpersonal interactions and relationships	Respect, tolerate, express opinions, criticize appropriately, establish physical contact, interact according to social rules, and maintain social space. Establishing relationships when necessary with strangers, relating formally in certain environments, creating and developing friendly relationships with acquaintances, colleagues and family, relating intimately with others on an emotional, physical and sexual basis.
d8 - Social, community and civic life	Participate in community social life, participate in organizations, ceremonies, clubs. Participate in group recreational activities, go to the cinema, theater, play instruments, participate in religious or spiritual activities, enjoy nationally and internationally recognized human rights.
d9 - Main areas of life	Learning informal education with family members, such as crafts, leisure/fun, acquiring preschool and school education skills, performing lessons, fulfilling obligations and responsibilities, participating in professional tasks, acquiring and maintaining work.

	NMD type	Sample size	Age (mean ± sd)	Severity	Intervention details	time and frequency of intervention	Outcomes
	NMD type	Sample size	Age (mean ± sd)	Severity	Intervention details	time and frequency of intervention	Mobility (d4)	Self care (d5)	Communication (d3)	Participation (d7, d8, d9)
Andersen et al. (2017)²⁰20 Andersen G, Heje K, Buch AE, Vissing J. High-intensity interval training in facioscapulohumeral muscular dystrophy type 1: a randomized clinical trial. J Neurol 2017;264(06):1099–1106	FSHD1	(13) EG: N = 6 CG: N = 7	EG: 53 ± 15 (26–67) CG: 46 ± 9 (32–59)	FSHD score = 1–11	EG: HIT (cycle-ergometer) CG: Usual care	8 weeks (3 × 10-min)	6MWT 5STS IPAQ
Dal Bello-Haas et al. (2007)²¹21 Bello-Haas VD, Florence JM, Kloos AD, et al. A randomized controlled trial of resistance exercise in individuals with ALS. Neurology 2007;68(23):2003–2007	ALS (ALSFRS early stage)	(27) EG = 13 CG = 14	not informed	not informed	EG: stretching exercise program (as CG) and an individualized UE and LE moderate-load intensity resistance exercise program CG: Usual care UE and LE stretching exercises	6 months	ALSFRS-R
Lindeman et al. (1995)²²22 Lindeman E, Leffers P, Spaans F, et al. Strength training in patients with myotonic dystrophy and hereditary motor and sensory neuropathy: a randomized clinical trial. Arch Phys Med Rehabil 1995;76(07):612–620	MD HMSN (types I or II)	(62) MyD = 33 HMSN = 29	MyD EG: 40 ± 11 CG: 37 ± 10 NHMS EG:35 ± 10 CG: 38 ± 11	not informed	EG: Strength training with weights of the proximal lower extremity muscles CG: nonexercising (CG were encouraged to refrain from training	3x week for 24 weeks	Time scored activities performed as fast as possible: ascending and descending stairs, rising from a chair, rising from supine on a physical exercise table and walking 50m 6MWT WOMAC functional part of the Western Ontario and McMaster Universit	SIP-68		SIP-68: Social participation
Nakajima et al. (2021)²³23 Nakajima T, Sankai Y, Takata S, et al. Cybernic treatment with wearable cyborg Hybrid Assistive Limb (HAL) improves ambulatory function in patients with slowly progressive rare neuromuscular diseases: a multicentre, randomised, controlled crossover trial for efficacy and safety (NCY-3001). Orphanet J Rare Dis 2021; 16(01):304	NMD	(24) EG = 11 CG = 13	CG= 55,5 ± 7.2 EG = 56 ± 13.2	no rapid changes in gait symptoms for three months prior to the start of the trial	GE: Walking program with partial body support and exoeskeletal device for LE GC: Walking program with partial body support (30min)	13 weeks (9 sessions)	2MWT (gait distance) 10MWT (speed) Barthel index	Barthel index
Okkersen et al. (2018)²⁴24 Okkersen K, Jimenez-Moreno C, Wenninger S, et al; OPTIMISTIC consortium. Cognitive behavioural therapy with optional graded exercise therapy in patients with severe fatigue with myotonic dystrophy type 1: a multicentre, single-blind, randomised trial. Lancet Neurol 2018;17(08):671–680	MD1	(255) EG = 128 CG = 127	EG: 44,8 ± 11,7 CG: 46,4 ± 11,3		EG: Cognitive behavioural therapy intervention and usual care or aerobic exercise CG: Usual care	10 months (10–14 sessions)	DM1-Activ-C 6MWT MDHI	DM1-Activ-C	MDHI	DM1-Activ-C MDHI
Öksüz et al.(2011)²⁵25 Öksüz Ç, Akel BS, Bumin G. Effect of occupational therapy on activity level and occupational performance in patients with neuromuscular disease. Fizyoterapi Rehabilitasyon 2011;22 (03):231–239	NMD	(60) EG = 30 CG = 30	EG: 37.5 ± 15.1 CG:38.7 ± 15.4	can stand in long leg braces but unable to walk even with assistance and who uses a wheelchair full time	EG: Client centred occupational therapy program and conventional therapeutic home exercise program by a physiotherapist CG: Conventional physiotherapeutic home exercise program by a physiotherapist	6 months	FIM DASH	FIM		COPM
Raglio et al. (2016)²⁶26 Raglio A, Giovanazzi E, Pain D, et al. Active music therapy approach in amyotrophic lateral sclerosis: a randomized-controlled trial. Int J Rehabil Res 2016;39(04):365–367	ALS	(30) EG = 15 CG = 15	EG: 62.9 ± 9.83 CG: 65.1 ± 12.10	mild–moderate disability (ALS Functional Rating Scale- Revised ≤40)	EG: Active Music Therapy and usual care CG: Usual care	12 sessions, 3x/weeks (4 weeks)	ALSFRS-R	ALSFRS-R	MTRS
Sherief et al. (2021)²⁷27 Sherief AEAA, Abd ElAziz HG, Ali MS. Efficacy of two intervention approaches on functional walking capacity and balance in children with Duchene muscular dystrophy. J Musculoskelet Neuronal Interact 2021;21(03):343–350	DMD	(30) EG = 15 CG = 15	EG: 8.49 ± 0.83 CG: 8.34 ± 0.88	Able to walk alone level I and II of Ambulation function classification system for DMD (AFCSD)	EG: physical therapy program and aerobic exercise training by treadmill CG: physical therapy program and aerobic exercise training by bicycle ergometer	20 min, 3x/week, 3 months 20 min, 3x/week, 3 months	6MWT
Van Groenestijn et al. (2019)²⁸28 van Groenestijn AC, Schröder CD, van Eijk RPA, et al. Aerobic Exercise Therapy in Ambulatory Patients With ALS: A Randomized Controlled Trial. Neurorehabil Neural Repair 2019;33(02): 153–164	ALS	(57) EG = 27 CG = 30	EG: 60.9 ± 10.0 CG: 59.9 ± 10.7	life expectancy longer than 1 year; ability to walk with or without walking aid (≥10 min); and ability to cycle on a cycle ergometer (≥15 min)	EG: Aerobic cycling exercise program (50-75% HRR) and usual care CG: Usual care	16 weeks	ALSFRS-R TUG			SIP-68 IPAQ
Veenhuizen et al. (2019)²⁹29 Veenhuizen Y, Cup EHC, Jonker MA, et al. Self-management program improves participation in patients with neuromuscular disease: A randomized controlled trial. Neurology 2019;93(18): e1720–e1731	NMD	(53) EG = 29 CG = 24	EG: 52 (37-63) CG: 50 (41-60)	not informed	EG: Aerobic exercise training (50-70% HRR) and self-management strategies CG: Usual care	16 weeks 3x week of 30 min	6MWT			COPM
Voet et al. (2014)³⁰30 Voet N, Bleijenberg G, Hendriks J, et al. Both aerobic exercise and cognitive-behavioral therapy reduce chronic fatigue in FSHD: an RCT. Neurology 2014;83(21):1914–1922	FSHD1	(57) EG1 = 20 EG2 = 13 CG = 24	EG1: 59 (21–68) EG2:49 (24–69) CG: 52 (20–79)	Able to walk independently (ankle-foot orthoses and canes are accepted)	EG1: Aerobic cycling exercise training (50-65% HRR) EG2: Cognitive-behavioral therapy CG: Usual care	30 min, 3x/week, 16 weeks (GE1) minimum of 3 session (GE2)	6MWT CIS-activity	SIP-68		SIP-68
Zivi et al. (2018)³¹31 Zivi I, Maffia S, Ferrari V, et al. Effectiveness of aquatic versus land physiotherapy in the treatment of peripheral neuropathies: a randomized controlled trial. Clin Rehabil 2018;32(05):663–670	Peripheral neuropathies	(40) EG = 21 CG = 19	EG: 66.3 ± 13.0 CG: 71.8 ± 7.7	ability to maintain the upright position and to walk even if with assistance	EG: Gait and balance of aquatic physiotherapy CG: Gait and balance of on-land training	4 weeks of daily sessions of usual physiotherapy 3x week of specific treatment (aquatic vs. on-land)	DGI BBS FIM Functional Ambulation Classification

Study	1	2	3	4	5	6	7	8	9	10	Total
Andersen et al. (2017)²⁰20 Andersen G, Heje K, Buch AE, Vissing J. High-intensity interval training in facioscapulohumeral muscular dystrophy type 1: a randomized clinical trial. J Neurol 2017;264(06):1099–1106	Y	N	Y	N	N	N	N	N	Y	Y	4/10
Dal Bello-Haas et al. (2007)²¹21 Bello-Haas VD, Florence JM, Kloos AD, et al. A randomized controlled trial of resistance exercise in individuals with ALS. Neurology 2007;68(23):2003–2007	Y	Y	N	N	N	Y	Y	Y	Y	N	6/10
Lindeman et al. (1995)²²22 Lindeman E, Leffers P, Spaans F, et al. Strength training in patients with myotonic dystrophy and hereditary motor and sensory neuropathy: a randomized clinical trial. Arch Phys Med Rehabil 1995;76(07):612–620	Y	N	Y	N	N	Y	Y	N	Y	Y	6/10
Nakajima et al. (2021)²³23 Nakajima T, Sankai Y, Takata S, et al. Cybernic treatment with wearable cyborg Hybrid Assistive Limb (HAL) improves ambulatory function in patients with slowly progressive rare neuromuscular diseases: a multicentre, randomised, controlled crossover trial for efficacy and safety (NCY-3001). Orphanet J Rare Dis 2021; 16(01):304	Y	N	Y	N	N	N	Y	N	Y	Y	5/10
Okkersen et al. (2018)²⁴24 Okkersen K, Jimenez-Moreno C, Wenninger S, et al; OPTIMISTIC consortium. Cognitive behavioural therapy with optional graded exercise therapy in patients with severe fatigue with myotonic dystrophy type 1: a multicentre, single-blind, randomised trial. Lancet Neurol 2018;17(08):671–680	Y	N	Y	N	N	Y	Y	Y	Y	Y	7/10
Öksüz et al. (2011)²⁵25 Öksüz Ç, Akel BS, Bumin G. Effect of occupational therapy on activity level and occupational performance in patients with neuromuscular disease. Fizyoterapi Rehabilitasyon 2011;22 (03):231–239	Y	Y	Y	N	N	N	N	N	Y	Y	5/10
Raglio et al. (2016)²⁶26 Raglio A, Giovanazzi E, Pain D, et al. Active music therapy approach in amyotrophic lateral sclerosis: a randomized-controlled trial. Int J Rehabil Res 2016;39(04):365–367	Y	Y	Y	Y	N	N	Y	Y	Y	Y	8/10
Sherief et al. (2021))²⁷27 Sherief AEAA, Abd ElAziz HG, Ali MS. Efficacy of two intervention approaches on functional walking capacity and balance in children with Duchene muscular dystrophy. J Musculoskelet Neuronal Interact 2021;21(03):343–350	Y	Y	Y	N	N	Y	Y	N	Y	Y	7/10
Van Groenestijn et al. (2019)²⁸28 van Groenestijn AC, Schröder CD, van Eijk RPA, et al. Aerobic Exercise Therapy in Ambulatory Patients With ALS: A Randomized Controlled Trial. Neurorehabil Neural Repair 2019;33(02): 153–164	Y	N	Y	N	N	Y	N	Y	Y	Y	6/10
Veenhuizen et al. (2019)²⁹29 Veenhuizen Y, Cup EHC, Jonker MA, et al. Self-management program improves participation in patients with neuromuscular disease: A randomized controlled trial. Neurology 2019;93(18): e1720–e1731	Y	Y	Y	N	N	Y	Y	Y	Y	Y	8/10
Voet et al. (2014)³⁰30 Voet N, Bleijenberg G, Hendriks J, et al. Both aerobic exercise and cognitive-behavioral therapy reduce chronic fatigue in FSHD: an RCT. Neurology 2014;83(21):1914–1922	Y	N	Y	N	N	Y	Y	Y	Y	Y	7/10
Zivi et al. (2018)³¹31 Zivi I, Maffia S, Ferrari V, et al. Effectiveness of aquatic versus land physiotherapy in the treatment of peripheral neuropathies: a randomized controlled trial. Clin Rehabil 2018;32(05):663–670	Y	Y	N	N	N	Y	Y	Y	Y	Y	7/10

	Sample size (Studies)	Evidence quality (GRADE)
Mobility	847 (12 RCT's)	very low² 2 Imprecision (wide confidence intervals crossing a decision threshold); ,³ 3 Indirectness of evidence (differences in populations, interventions, comparators or outcomes);
Participation	487 (05 RCT's)	very low¹ 1 Study limitations (risk of bias, such as lack of allocation concealment or blinding); –³ 3 Indirectness of evidence (differences in populations, interventions, comparators or outcomes);
Communication	285 (02 RCT's)	very low¹ 1 Study limitations (risk of bias, such as lack of allocation concealment or blinding); ,² 2 Imprecision (wide confidence intervals crossing a decision threshold); ,³ 3 Indirectness of evidence (differences in populations, interventions, comparators or outcomes);
Self care	526 (05 RCT's)	very low² 2 Imprecision (wide confidence intervals crossing a decision threshold); ,³ 3 Indirectness of evidence (differences in populations, interventions, comparators or outcomes);
Grade of evidence (GRADE)³⁴34 Xie CX, Machado GC. Clinimetrics: Grading of Recommendations, Assessment, Development and Evaluation (GRADE). J Physiother 2021;67(01):66 High: The authors have a lot of confidence that the true effect is similar to the estimated effect Moderate: The authors believe that the true effect is probably close to the estimated effect Low: The true effect might be markedly different from the estimated effect Very low: The true effect is probably markedly different from the estimated effect

[1] Address for correspondence Thaís Peixoto Gaiad (email: thais.gaiad@ufvjm.edu.br)

Brasil

Brasil

Rehabilitation interventions targeting the activity and participation of patient with neuromuscular diseases: what do we know? A systematic review

Reabilitação com foco na atividade e participação para pessoas com doenças neuromusculares: o que sabemos? Uma revisão sistemática

Abstract

Background

Objective

Methods

Results

Conclusion