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Received : 19-07-2024

Accepted : 29-08-2024



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Tanrıverdi and Yurdalan: Physiotherapy rehabilitation program augmented with virtual reality exergame in myotonic dystrophy: Case report


Introduction

Myotonic Dystrophy (MD) is a slowly progressive neuromuscular disorder characterized by myotonia. Symptoms of MD include distal-axial muscle weakness, gait abnormalities, and balance impairments. Individuals with MD often express concerns about their ability to perform daily living activities due to the disease's progression.1 Moreover, they are advised to limit physical exertion, leaving the efficacy of strength or aerobic exercise training in muscle diseases uncertain.2

Virtual reality (VR)-based exergaming has emerged as a preferred approach for long-term rehabilitation due to its sustainability and motivational benefits.3, 4 In this report, we present the effects of a six-month intervention involving VR games combined with a physiotherapy rehabilitation program (PTR) in a case of MD.

Case Presentation

A 13-year-old girl diagnosed with MD presented to the physiotherapy rehabilitation department with primary complaints of numbness, fatigue, and a slowing of her movements. She specifically reported fatigue in her feet, worsening ability to sit and stand weakness in her flexor muscles, particularly in the distal group, and poor control over her extensors. Additionally, she exhibited weakness in movement initiation, stiffness in hand opening, difficulty standing, and limited knee extension. She reported experiencing falls approximately once a week, often resulting in a prone position, mostly occurring outside her home.

Table 1

The anthropometric measurements, shortness tests,and posture analysis of a case

Right

Left

Anthropometric measurement of Spine*

Spina iliaca anterior superior-medial malleol

73

73

Spina iliaca anterior superior-tuberositas tibia

43

43

Tuberositas tibia-medial malleol

30

30

Spina iliaca anterior superior-umblicus

15

15

Umblicus-medial malleol

81

81

Umblicus-tuberositas tibia

55

55

Acromion-earlobe

13.5

13.5

Acromion-place of sitting

47

48

Spina iliaca posterior superior-place of sitting

13.5

13.5

Lower angle of the scapula-spine

10.5

10

Lower angle of the scapula-place of sitting

33.5

31.5

Shortness tests

Hip flexors

1

4

Hamstrings

+

+

Quadriceps femoris

-

-

Tensor fascia latae

-

-

Gastrocnemius

+

+

Lumbal extensor

+

+

Pectorals

-

-

Posture Analysis

Feet

Inversion

-

-

Eversion

+

+

Hallux valgus

-

-

Pes planus

0.5 cm

1 cm

Pes cavus

-

-

Crop

-

-

Knee

Genu recurvatum

-

-

Flexion

-

-

Tibial torsion

-

-

Genu varum

-

-

Genu valgum

-

-

Popliteal line inequality

42.5

43

Pelvis

Anterior pelvic tilt

+

Posterior pelvic tilt

-

Hip

Height difference

74.5

75

Gluteal line level

67

66

Columna vertebralis

Lordosis

+

Kyphosis

-

Scoliosis

Left thoracic C

Chest deformity

-

Shoulder

Protraction

+

Retraction

-

Rounded

+

Height difference

120

120

Head

Anterior tilt

+

Posterior tilt

-

Lateral flexion

-

-

Rotation

-

-

Table 2

The Results of Nerve Conduction and EMG (needle) Tests for Case

Rec. Site

Latency (ms)

Peak Ampl (µV)

Distance (cm)

Velocity (m/s)

Sensory

R Median digit II (wrist)

II

1.80

6.6

10.5

58.3

R Ulnar digit V (wrist)

V

1.65

9.0

9

54.5

R Sural Lat Malleolus Calf

Lat Malleolus

2.50

11.2

14

56.0

L Sural Lat Malleolus 1

Lat Malleolus

2.20

14.8

14

63.6

R Sup Peroneal Foot Lateral Leg

Foot

2.10

13.5

11

52.4

L Sup Peroneal Foot Lateral Leg

Foot

2.40

12.8

12

50.0

Motor

R Median APB

Wrist

2.75

10.9

Elbow

6.05

10.7

22

66.7

R Ulnar ADM

Wrist

2.05

11.6

B.Elbow

4.75

11.2

19

70.4

A.Elbow

6.05

10.8

10

76.9

R Common Peroneal EDB

Ankle

4.00

4.6

Knee

9.35

3.4

30

56.1

L Common Peroneal EDB

Ankle

3.15

10.8

Fib head

7.70

9.9

30

65.9

R Tibial AH

Ankle

2.95

10.4

Knee

10.10

10.4

34

47.6

L Tibial AH

Ankle

2.45

9.1

Knee

8.60

8.6

34

55.3

Muscle

Spontaneous

MUAP

Recruitment

IA

Fib

PSW

Fasc

H.F.

Amp.

Dur.

Pattern

L Tibialis anterior

None

None

None

None

R Tibialis anterior

None

None

None

2+

R Gastrocnemius (Med)

None

None

None

2+

R Vastus Lateralis

None

None

None

2+

R First D Interrosseus

None

None

None

3+

R Extensor Digitorum Communis

None

None

None

3+

R Biceps

None

None

None

3+

[i] EMG=electromyography, L=left, R=right, N=no activity

Table 3

The Results of Intervention on Fatigue, Muscle Strength Test, and Nintendo-Wii-Fit-Plus® (Body Test, Game Scores)

Muscle

Before intervention

After intervention

Right

Left

Right

Left

Trunk

Back extensors

4

4

Neck extensors

5

5

Neck flexors

3

4

Rectus abdominus

5

5

Oblic abdominals

5

5

5

5

Hip

Flexors

4

4

4

4

Extensors

3

4

4

4

Abductors

4

4

4

4

Adductors

3

3

3

3

Internal rotators

3

3

3

3

Eksternal rotators

3

3

3

3

Knee

Flexors

4

5

5

5

Extensors

5

5

5

5

Shoulder

Internal rotators

5

4

5

5

Eksternal rotators

5

4

5

5

Extensors

3

3

4

4

Arm

Supinator

5

5

5

5

Pronator

5

5

5

5

Wrist

Flexors

5

5

5

5

Extensors

5

5

5

5

Isolated Muscles

Tibialis anterior

5

5

5

5

Gastrocnemius

4

4

5

5

Foot inventer

5

4

5

5

Tibialis posterior

5

5

5

5

Serratus anterior

5

5

5

5

Trapezius top piece

5

5

5

5

Trapezius middle piece

5

5

5

5

Trapezius lower piece

5

5

5

5

Rhomboideus

5

5

5

5

Deltoideus anterior piece

4

4

4

4

Deltoideus middle piece

5

4

5

5

Deltoideus posterior piece

4

4

5

5

Biceps brachii

5

5

5

5

Triceps brachii

5

5

5

5

Fatigue

Visual Analog Scale (VAS)

8

6

Nintendo Wii Fit Plus®

Body Test

Center of gravity-COG (%)

45.6

54.4

49.9

50.1

Differences of COG (%)

8.8

0.2

Stability (%)

30

34

Fit age

31

30

Game Scores

Free jogging (meter)

3238

3779

Step (point)

60

106

Figure 1

The conventional physiotherapy rehabilitation program

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/6ba2cef4-fa7f-4a7a-b2d4-41d2675e217aimage1.png

Anthropometric measurements, shortness tests, and posture analysis of the case are detailed in Table 1. Balance assessments were conducted using Nintendo-Wii-Fit-Plus® (NWFP), which measured body test values including center of gravity (COG), stability, and fit age. During sessions, activities such as "Free Jogging" and "Step" from NWFP were selected, and game scores were recorded.

The NWFP body test revealed a return of COG to the center of equilibrium, while the one-leg standing test on the right side could not be completed. Electromyography results (Table 2) showed stimulation of peripheral nerves in the right upper and bilateral lower extremities. Notably, significant myotonic discharges were observed at rest, particularly prominent in the distal muscles of the upper extremities. These findings consistently indicated the presence of diffuse myotonic discharges in the distal limb muscles.

The case attended the sessions twice per week, each session lasting 45 minutes, over a period of six months. The conventional PTR program (see Figure 1) commenced with warm-up exercises. Virtual reality game exercises were administered using the NWFP game console system. Two aerobic exercises, 'Free Jogging' and 'Step,' were chosen for the intervention. Free jogging entailed a 10-minute session scored based on the distance covered, while Step involved stepping on and off the balance board in sync with on-screen prompts.

Following the intervention, improvements were observed in various parameters. According to the body test, the discrepancy between sides of the COG decreased by 8.6%, stability increased by 4%, and the fit age decreased by one year. The outcomes of the intervention on fatigue, muscle strength, NWFP body test results, and game scores are detailed in Table 3. Notably, the NWFP COG decreased from 8.8% to 0.2%, stability increased from 30% to 34%, and the gap between chronological age and fit age reduced from 18 to 17 years. Game scores also exhibited improvement, with initial scores (Free Jogging= 3238, Step= 60) increasing to final session scores (Free Jogging= 3779, Step= 106).

Discussion

This study examines the effectiveness of a PTR program in a case of MD, wherein conventional PTR is augmented with VR-based exergaming. Early evaluation of such cases is crucial for initiating timely interventions, essentially constituting preventive rehabilitation efforts. Fatigue emerges as a significant limiting factor in evaluations, guiding evaluators' focus. Despite MD being classified as a neuromuscular disease, there is a lack of specific protocols in the literature, prompting researchers to explore this field further.5

Neuromuscular diseases pose progressive challenges in management, necessitating specialized approaches. Voet et al., in their review, suggest that rehabilitation research encompassing various muscle disorders should present findings separately. They recommend considering participants' pre-training activity levels (sedentary vs. active) and specify intervention parameters such as exercise types, intensity, progression rate, frequency, session duration, muscle groups targeted, and supervision. Moreover, they advocate for interventions lasting at least six weeks to yield meaningful results 2.

Few studies have investigated interventions for MD, and those that exist often lack robust diagnostic verification of trial participants. Additionally, some trials did not employ intention-to-treat analysis, partly due to matched-pair designs, resulting in significant methodological limitations and an overall unclear risk of bias. However, certain case reports have demonstrated promising outcomes. For instance, one study found that various types of exercise programs led to improvements in both static and dynamic balance. Another case report by Maresca et al. explored the use of VR for cognitive and behavioral rehabilitation in eleven patients with MD Type-1. They advocated for integrating cognitive rehabilitation into the treatment framework to potentially enhance cognitive and behavioral functions and address neuropsychological symptoms in patients with MD Type-1.6

The rehabilitation mechanism remains inadequately elucidated. Neuroplasticity appears to play a central role, particularly in relation to delayed central motor conduction time and abnormal sensory-motor plasticity, with no discernible alteration of cortical excitability.7 Future research is warranted to elucidate effective PTR strategies. Lagrue et al. reported that musculoskeletal impairment in 314 children was mild.8 Additionally, Naro et al. proposed that gait impairment in MD patients might stem from muscle network deterioration, suggesting it could be a primary trait rather than a consequence of muscle degeneration.9 This insight is valuable for tailoring rehabilitative strategies for MD patients, emphasizing the need to address not only muscle weakness but also the muscle connectivity underlying gait function. Although this study primarily involves adults, similar mechanisms should be considered. Understanding these intricacies is crucial for optimizing rehabilitation outcomes. Longitudinal exercise programs are essential for addressing physical impairments and restoring postural stability. Future research should focus on developing protocols that maximize clinical benefits.

Conclusion

In conclusion, our case report highlights the potential benefits of integrating virtual reality (VR) gaming consoles into physiotherapy rehabilitation programs (PTR) for pediatric patients diagnosed with Myotonic Dystrophy (MD). Over a six-month period, the combined intervention led to significant improvements in various metrics, including reductions in the discrepancy between sides of the center of gravity and increases in stability. These findings underscore the therapeutic potential of VR-based exergaming in enhancing motivation and engagement in rehabilitation while addressing neuromuscular symptoms associated with MD. However, it is essential to note that further well-designed studies are needed to confirm the efficacy of this approach and its generalizability to larger patient populations. Additionally, future research should explore personalized treatment strategies based on neurophysiological motor patterns and consider integrating cognitive rehabilitation into the treatment framework to address cognitive and behavioral symptoms associated with MD. Understanding the mechanisms underlying rehabilitation in MD and developing longitudinal exercise protocols tailored to individual patient needs are critical for optimizing clinical outcomes and enhancing the quality of life for individuals living with this progressive neuromuscular disorder.

Source of Funding

None.

Conflict of Interest

None.

References

1 

C Turner H Jones Myotonic dystrophy: diagnosis, management and new therapiesCurr Opin Neurol2014275599606

2 

NB Voet EL Van Der Kooi BG Van Engelen Strength training and aerobic exercise training for muscle diseaseCochrane Database Syst Rev201912123907

3 

L Li F Yu D Shi Application of virtual reality technology in clinical medicineAm J Transl Res201799386780

4 

TD Parsons AA Rizzo S Rogers Virtual reality in paediatric rehabilitation: a reviewDev Neurorehabil200912422462

5 

MJ Kiefer EL Townsend Stepping Activity in Children With Congenital Myotonic DystrophyPediatr Phys Ther2018304340

6 

G Maresca S Portaro A Naro Look at the cognitive deficits in patients with myotonic dystrophy type 1: an exploratory research on the effects of virtual realityInt J Rehabil Res2020431904

7 

S Portaro A Naro A Chillura Toward a more personalized motor function rehabilitation in Myotonic dystrophy type 1: The role of neuroplasticityPLoS One2017125178470

8 

E Lagrue C Dogan D Antonio F Audic N Bach C Barnerias A large multicenter study of pediatric myotonic dystrophy type 1 for evidence-based managementNeurology202094985265

9 

A Naro S Portaro D Milardi Paving the way for a better understanding of the pathophysiology of gait impairment in myotonic dystrophy: a pilot study focusing on muscle networksJ Neuroeng Rehabil2019161116



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