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

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

person Sionara Ferreira Silva

conceptualization · data curation · formal analysis · investigation · writing – original draft

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

0009-0008-6356-2365

person Hugo Leonardo de Magalhães

conceptualization · data curation · formal analysis · writing – original draft

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

0000-0002-8156-0637

person Franciele Angelo de Deus

formal analysis · investigation · methodology

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

0000-0002-8028-0916

person Keysy Karoline Souza Andrade

formal analysis · writing the original draft

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

0000-0002-9979-8985

person Vanessa Pereira Lima

conceptualization · formal analysis · methodology · supervision · writing - review and edit

schoolUniversidade Federal dos Vales do Jequitinhonha e Mucuri, Faculdade de Ciências Biológicas e da Saúde, Departamento de Fisioterapia, Diamantina MG, Brazil.schoolUniversidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

0000-0002-0349-3248

person Thaís Peixoto Gaiad

conceptualization · formal analysis · project administration · supervision · writing - review and edit

schoolUniversidade Federal dos Vales do Jequitinhonha e Mucuri, Faculdade de Ciências Biológicas e da Saúde, Departamento de Fisioterapia, Diamantina MG, Brazil.schoolUniversidade Federal dos Vales do Jequitinhonha e Mucuri, Programa de Pós-Graduação em Reabilitação e Performance Funcional, Diamantina MG, Brazil.

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)²⁰	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)²¹	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)²²	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)²³	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)²⁴	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)²⁵	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)²⁶	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)²⁷	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)²⁸	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)²⁹	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)³⁰	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)³¹	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)²⁰	Y	N	Y	N	N	N	N	N	Y	Y	4/10
Dal Bello-Haas et al. (2007)²¹	Y	Y	N	N	N	Y	Y	Y	Y	N	6/10
Lindeman et al. (1995)²²	Y	N	Y	N	N	Y	Y	N	Y	Y	6/10
Nakajima et al. (2021)²³	Y	N	Y	N	N	N	Y	N	Y	Y	5/10
Okkersen et al. (2018)²⁴	Y	N	Y	N	N	Y	Y	Y	Y	Y	7/10
Öksüz et al. (2011)²⁵	Y	Y	Y	N	N	N	N	N	Y	Y	5/10
Raglio et al. (2016)²⁶	Y	Y	Y	Y	N	N	Y	Y	Y	Y	8/10
Sherief et al. (2021))²⁷	Y	Y	Y	N	N	Y	Y	N	Y	Y	7/10
Van Groenestijn et al. (2019)²⁸	Y	N	Y	N	N	Y	N	Y	Y	Y	6/10
Veenhuizen et al. (2019)²⁹	Y	Y	Y	N	N	Y	Y	Y	Y	Y	8/10
Voet et al. (2014)³⁰	Y	N	Y	N	N	Y	Y	Y	Y	Y	7/10
Zivi et al. (2018)³¹	Y	Y	N	N	N	Y	Y	Y	Y	Y	7/10

	Sample size (Studies)	Evidence quality (GRADE)
Mobility	847 (12 RCT's)	very low2,3
Participation	487 (05 RCT's)	very low1–3
Communication	285 (02 RCT's)	very low1,2,3
Self care	526 (05 RCT's)	very low2,3
Grade of evidence (GRADE)³⁴ 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

[TFN1] Abbreviations: 10MWT (speed), 10 min walk test; 2MWT (gait distance), 2 min walk test; 5STS, 5-time sit-to-stand time; 6MWT, 6-min walk distance; ALS, amyotrophic lateral sclerosis; ALSFRS, Functional Rating Scale; BBS, Berg Balance Scale; CG, Control group; CIS-activity, Checklist Individual Strength; COPM, Canadian Occupational Performance Measure; DASH, Disabilities of the Arm Shoulder and Hand Questionnaire; DGI, Dynamic gait index; DM1-Activ-C, DM1-Activ-c scale; EG, Exercise group; FIM, Functional Independence Measure; FSHD1, facioscapulo-humeral muscular dystrophy type 1; HIT, high-intensity training; HMSN, hereditary motor and sensory neuropathy; HRR, heart rate reserve; IPAQ, International Physical Activity Questionnaire; LE, lower extremity; MD, myotonic dystrophy; MD1, myotonic dystrophy type 1; MDHI, Myotonic Dystrophy Health Index; MTRS, Music Therapy Rating Scale; NMD, neuromuscular diseases; sd, standart deviation; SIP-68, Sickness Impact Profile; SS, Sample size; TUG, Timed Up and Go; UE, upper extremity; WOMAC, functional subscale of the Western Ontario and McMaster University.