IndoJPMR Vol.
13 - 2nd Edition - December 2024 | CASE REPORT The Effect of Static Ergocycle on Cardiorespiratory Endurance in an Early Ambulatory Duchenne Muscular Dystrophy Patient: A Case Report Rizky Kusuma Wardhani1.
Fiona Lestari1 Department of Physical Medicine and Rehabilitation.
Faculty of Medicine Universitas Indonesia.
Cipto Mangunkusumo General Hospital Jakarta.
Indonesia9
ABSTRACT
Introduction: Duchenne muscular dystrophy (DMD) is a debilitating genetic disorder affecting approximately 1 in 3,600 to 6,000 live male births, caused by mutations in the dystrophin gene.
The early ambulatory stage of DMD is typically characterized by difficulty standing, muscle weakness, frequent falls, and impaired motor skills.
Previous research suggests that assisted bicycle training may improve patientsAo quality of life and lung function.
Case Description: A multidisciplinary approach involving medical and rehabilitative interventions is essential to improve quality of life and delay disease progression.
The rehabilitation program included physical activities, stretching exercises, and environmental modifications.
Spirometry and the 6-Minute Walk Test .
MWT) indicated restrictive lung patterns and reduced exercise capacity.
Adherence to corticosteroid treatment, continuous monitoring, and a comprehensive rehabilitation strategy were crucial for maintaining optimal muscle function and enhancing overall quality of life.
Pulmonary function evaluation and management are particularly important, given the association of pulmonary complications with mortality in DMD.
Keywords: Duchenne muscular dystrophy, rehabilitation management INTRODUCTION Correspondence Detail:
Rizky Kusuma Wardhani Department of Physical Medicine and Rehabilitation.
Faculty of Medicine Universitas Indonesia.
Cipto Mangunkusumo General Hospital Jakarta.
Indonesia Email: rizkikusumawardhani@yahoo.
Duchenne muscular dystrophy (DMD) is a severe, progressive disorder that affects 1 in 3,600-6,000 male births.
The condition is caused by gene mutations in the dystrophin gene .
ocus Xp21.
1Ae3 Currently, there is no definitive cure for DMD.
However, multidisciplinary approach combining medicine, surgery and | IndoJPMR Vol.
13 - 2nd Edition - December 2024 rehabilitation can change the diseaseAos natural progression, thus improving patientAos quality of life (QoL) and longevity.
4 Previous research has shown that low-intensity aerobic exercise, particularly assisted bicycle training, may enhance motor function in DMD patient.
This case report aims to evaluate the effect of static ergocycle in early ambulatory DMD case to maintain cardiorespiratory function.
CASE PRESENTATION
A 5-year-old boy, suspected to have Duchenne Muscular Dystrophy (DMD), was referred to the Physical Medicine and Rehabilitation Outpatient Clinic.
Approximately 7 months ago, he began experiencing several functional limitations, such as needing to take breaks after walking 200-300 meters, crawling to climb stairs, difficulty lifting heavy objects, and slower learning capacity compared to his peers.
Since the onset of these symptoms, he has preferred spending most of his time on his phone rather than engaging in outdoor Physical examination revealed an anterior pelvic tilt, hyperlordotic lumbar posture, pseudohypertrophy of both deltoid and calf muscles, and hypotrophy of all muscles.
Range of motion (ROM) was full in both the upper and lower extremities, while manual muscle testing (MMT) indicated weakness in all extremities, more prominent in the proximal regions.
The Valley sign and Gowers sign were positive, while the Meryon sign was negative.
Gait analysis showed a waddling gait, characterized by initial total foot contact and increased arm swing.
Figure 1.
Lateral View of Patient Posture.
Valley Sign.
Figure 2.
Gowers Sign.
IndoJPMR Vol.
13 - 2nd Edition - December 2024 | Spirometry results indicated restrictive lung disease, with a forced expiratory volume (FEV.
44 L .
% of predicte.
, forced vital capacity (FVC) of 0.
45 L .
% of predicte.
, a FEV1/FVC ratio of 98%, and weak peak cough flow .
L/ The 6- Minute Walk Test .
MWT) was terminated prematurely at 5 minutes 15 seconds due to fatigue, covering a distance of 210 meters .
% of predicte.
, with normal vital signs and no His Brooke Scale score was 1 point.
Vignos Scale score was 3 points, and his ambulation function classification system for DMD (AFCSD) was Level II.
The Muscular Dystrophy Functional Rating Scale showed the following: 81% for the mobility domain, 75% for the basic ADL domain, 100% for the arm function domain, and 95% for the impairment domain.
Creatine Kinase (CK) levels were elevated at 9,872 U/L.
Electromyography (EMG) of the right tibial anterior muscle revealed a myogenic lesion with chronic denervation.
Musculoskeletal ultrasonography of both upper and lower extremities showed decreased muscle fiber with dominant hyperechoic homogeneous fibrosis, suggestive of DMD.
Figure 3.
Ultrasonography of gluteus maximus muscle on .
left side and .
right side.
Note that both pictures depict minimal muscle fibre and hyperechoic features with homogeneity.
He was placed on a regimen consisting of intermittent Methylprednisolone at 0.
75 mg/ kg/day, divided into two doses for 10 days, followed by 10 days off, along with omeprazole and Vitamin D as prescribed by his pediatrician.
The patientAos rehabilitation program includes the following:
A Mild to moderate intensity aerobic exercise .
tatic ergocycle: 3 x 10 minutes, with a 10minute break between each session, 5 times per week or as tolerated by the patien.
A Mild to moderate intensity isometric strengthening exercises .
ip extensors, knee extensors, and dorsiflexors: 3 x 10 repetitions.
A A A A A with a 6-second hold each, 3 times per week or as tolerated by the patien.
Functional upper extremity exercises .
all throwing activity: 3 x 10 repetitions, 3 times per week or as tolerated by the patien.
Passive stretching .
ip, knee, and ankle joints: 3 x 10 repetitions, with a 10-second hold each, 7 days per wee.
Incentive spirometry .
x 5 repetitions, with a 3-second hold each, 7 days per wee.
Active air stacking exercise .
x 5 repetitions, 7 days per wee.
Voluntary coughing 3 times per day | IndoJPMR Vol.
13 - 2nd Edition - December 2024 Furthermore, optimization of the home environment was recommended, such as using a seated toilet or a commode chair.
After 3 months of intervention, the 6-Minute Walk Test .
MWT) was repeated, resulting in a distance of 185 meters .
0% of predicted from RizkyAos Formul.
, with the test being stopped at 5 minutes 5 seconds due to exhaustion.
He also completed the 10-Meter Walk Test .
mWT), with a time of 24 seconds.
After 4 months of intervention, his subsequent 6MWT showed a distance of 210 meters .
of predicted from RizkyAos Formul.
, during which he completed the full 6-minute course recruitment, or AuearlyAy recruitment, observed with slight effort.
Complex repetitive discharges and abnormal spontaneous rest activity were also present, indicating membrane instability.
without stopping or falling.
However, the patient continued to report experiencing multiple falls during daily activities, which made him unwilling to participate in outdoor activities.
to reduced bone mineralization.
6 Routine spine radiography is also recommended, a s scoliosis may develop in patients taking glucocorticoids, particularly after discontinuation of the Annual follow-up assessments will be necessary as the patient progresses towards a non-ambulant state.
Echocardiography is planned, as DMD can affect the cardiac muscles, leading to progressive dilated cardiomyopathy, congestive heart failure, cardiac insufficiency, conduction abnormalities, and an increased risk of sudden early death.
DISCUSSION