Rolul kinetoterapiei în conservarea capacităţii motrice în distrofia
musculară Becker – studiu de caz
Teodora Alina Codrea1, Nicolae Horaţiu Pop2
1Graduate, Faculty of Physical Education and Sport, ”Babeş-Bolyai” University Cluj-Napoca, Physical therapist at the Maria-Beatrice Center of Social and Medical Services, Alba Iulia 2Faculty of Physical Education and Sport, ”Babeş-Bolyai” University, Cluj-Napoca
Abstract
Background. This paper approaches Becker’s dystrophy: a muscle disease which determines gradual and slow loss of mo- tor skills.
Aims. In this case study, motor skills were evaluated using the Motor Function Measure (MFM) test.
Methods. The physical therapy protocol applied comprises stretching, stamina, aerobic and skill exercises.
Results. The comparative analysis of the data indicates the maintenance of a high level of motor skills, the improvement of coordination and distal motor control.
Conclusions. The long term objectives set are reached, given the regressive characteristic of the disease.
Keywords: muscular dystrophy, dystrophin, motor control, motor skill, MFM test.
Rezumat
Premize. Lucrarea studiază distrofia musculară progresivă Becker, boală care determină diminuarea lentă a capacităţii motrice.
Obiective. Urmărim prevenirea contracturilor şi deformǎrilor osteo-articulare, menţinerea şi creşterea forţei musculare, menţinerea funcţionalităţii aparatului cardiovascular.
Metode. Programul de kinetoterapie constă în exerciţii de stretching, creştere a forţei musculare, aerobice şi de abilitate.
Rezultate. Analiza comparativă a rezultatelor la testul Motor Function Measurement (MFM) indică menţinerea unui nivel ridicat al capacităţii motrice, îmbunătăţirea coordonării şi a controlului motor distal.
Concluzii. Se ating obiectivele pe termen lung propuse, având în vedere specificul bolii spre involuţie.
Cuvinte cheie: distrofie musculară, distrofină, control motor, capacitate motrice, testul MFM (evaluarea controlului mo- tor).
Copyright © 2010 by “Iuliu Hațieganu” University of Medicine and Pharmacy Publishing Received: 2012, January 11; Accepted for publication: 2012, February 20
Address for correspondence: Babeş-Bolyai University, Cluj-Napoca, Faculty of Physical Education and Sport, Pandurilor Str. No. 7 E-mail: [email protected]
Introduction
The outcome of approximately 3% of all pregnancies is the birth of a child with a genetic or congenital disease which leads to disability, mental retardation or even death. Becker’s dystrophy is such an example, occurring in 1 out of 20,000 male individuals. Etiologically it is explained as a deletion of the gene codifying the synthesis of dystrophin, located on the X chromosome, band 21, at the level of exon 45, consequently dystrophin is synthesized in low amounts or with abnormal molecular configuration (Ciofu & Ciofu, 2001; Siegel, 1999). Molecular modifications refer to the lack of some sections of the molecule: the central domain, - COOH group, the amino group (Monaco & Kunkel, 1996); dystrophin is also found in the myocardium, the brain, the
retina, which means that the disease does not only occur in skeletal muscles (Bushby & Anderson, 2001).
The patient with Becker’s dystrophy presents alterations of physical muscle capacities, contractility, elasticity, tonicity (Pop & Zamora, 2007), where a prolonged contraction of the muscle and at the same time a diminution of muscle elasticity and tonicity can be observed, as well as generalized muscular hypertrophy, pseudo-hypertrophy of the posterior calf region. Atrophy of the proximal and axial musculature appears subsequently with exaggerated lumbar lordosis, prominent abdomen, scoliosis, weakening of muscle strength (Ciofu & Ciofu, 2001; Popa, 1997; Emery, 2000). A waddling walk and the Gower’s sign diminish transfer and locomotion capacity.
The role of physical therapy in Becker’s muscular dystrophy
motor capacity of the patient through specific methods and techniques for the objectives set (Tarnopolski & Mark, 2009). The Barthel index usually presents good results in the case of progressive dystrophies with slow evolution. Good results were obtained with the Vignos scale in the case of Becker’s dystrophy (Lue et al., 2009).
Cardiac involvement is rare, but can occur during evolution as tachycardia in 18% out of 56 bearers (Finsterer, Stöllberger, 2008). Diminished cognitive functions were seldom described (Ciofu & Ciofu, 2001).
Hypothesis
Referring to progressive muscular dystrophies, the idea is claimed that through physical exercise the contractures are diminished and muscular strength is preserved. The positive effects on the cardiovascular system and respiratory system are also well known. The study is aimed at preserving the motor capacity of the patient through therapeutic physical exercise, specific methods and techniques.
Material and methods
The case study presents patient (LD) aged 19, diagnosed with Becker’s progressive muscular dystrophy with onset at 8 years of age. Presently the patient has difficulties when walking and low stamina during exercise.
The suggested protocol is aimed at preventing con- tractions and osteoarticular deformations in order to delay the point when the patient becomes dependent on the wheelchair as much as possible. The aim is to maintain and improve muscle strength through specific techniques applied to the shoulder and hip girdle, and to postural muscles, which play a part in maintaining balance and stability, according to a specific scheme, as well as to maintain the function of the cardiovascular system through aerobic exercise three times a week, with a maximum duration of 15 minutes and an intensity not exceeding heart rate values of 120-140 bpm. Stretching exercises are used at the beginning and at the end of every physical therapy session.
Studies show that different forms of muscular dystrophy exercises for improving/maintaining muscular strength do not present negative effects (Voet et al., 2010).
The starting point for applying therapeutic physical exercise is the model suggested by Tarnopolsky and Mark (2009), which was particularized and individualized according to some defining criteria presented by Sbenghe (2008) and Marcu (2006). Ability exercises applied as ball exercises or the use of the Bobath ball completed the program (table I).
The patient was supervised over a period of 6 months,
from November 11th, 2009 until April 30th, 2010. The
evaluation was performed using the ”Motor Function Measure” test (1).
The Motor Function Measure offers a standard evaluation of the motor capacity of a subject, and is validated for per- sons with muscle dystrophy, among other neuromuscular diseases. The test consists of 32 items (table II) and refers to 3 dimensions as follows: D1: transfer to and maintenance of the standing position (13 items); D2: axial and proximal motor capacity (12 items); D3: distal motor capacity (7 items). The tasks follow a logical rule. For each task the dimension noted with ”D”, the initial position, the action, instructions and the evaluation are mentioned.
Each item is scored on a 4 point scale (0–3); a higher score means a better performance. 0: cannot initiate the task; 1: performs the task partially; 2: performs the task partially or completely, but imperfectly; 3: performs the task completely and normally. Data are reported as tables and diagrams on all 3 dimensions.
Statistical analysis
Statistical analyses were performed by one way Anova analysis of variance and Dunnett’s multiple comparison tests using GraphPad Prism version 5.00, GraphPad Software Inc. (California, USA). The data were expressed as average score means ± SEM and the average scores of the second and third evaluation times (week 12 and 24) were compared
to the first evaluation time (week 1). Differences at p≤0.05
were considered to be significant. Results
The patient diagnosed with Becker’s muscular dystrophy at the age of 8 presents walking difficulties and low resistance during exercise. The data obtained confirm the maintenance and improvement of muscle strength through specific techniques at the level of the shoulder and hip girdle, and of postural muscles, which play a part in maintaining balance and stability. 0.5 1.0 1.5 2.0 2.5 **
W eek 1 W eek 12 W eek 24
*
Average score of MFM test
Fig. 1 – Average scores of the MFM test for the standing and
transfer dimensions. Legend: p=0.0087, R2= 0.2319, **p<0.01 (very significant), *<0.05 (significant).
Table I
Working methods applied according to periods of time.
No. Period of time
Working methods
Warm up and post- exercise appeasement Stretching
(4x/ex. with 20 sec. pause)
Muscle strength improvement (1-2 sets/ex.; 5-8x/set with 2 minutes
pause between the ex.)
Aerobic exercise (8-15 minutes;
120-130 CF)
Ability (Game with the
Bobath ball) 1 09.11- 22.11 2x/ day 2x/ week 3x/ week 1x/ day 15 minutes 1 ex./ session 2 23.11- 04.12 3x/ week 3 07.12- 03.01
2x/ day 3x/ week 1x/ day
4 04.01- 07.02 5 08.02- 28.02
6 01.03- 12.03 2x/ day 3x/ week 1x/ day
7 15.03- 01.04
Teodora Alina Codrea, Nicolae Horaţiu Pop 2.2 2.4 2.6 2.8 3.0 ns ns
W eek 1 W eek 12 W eek 24
Average score of MFM test
Fig. 2 – Average scores of the MFM test for the axial and
proximal motor function. Legend: p=0.6156, R2= 0.0289, ns-not significant. 2.2 2.4 2.6 2.8 3.0 ns ns
W eek 1 W eek 12 W eek 24
Average score of MFM test
Fig. 3 – Average score of the MFM test for the distal motor
function. Legend: p=0.0881, R2= 0.2366, ns-not significant.
Discussion
D1: Standing and transfers
The data obtained for dimension 1 show a very significant statistical decrease of about 19% in the average score obtained at the second evaluation (week 12), compared to the average score obtained at the first evaluation (week 1). The average scores obtained at the third evaluation were statistically significantly lower, about 13% lower than those obtained in the first week of evaluation (Fig. 1). According to our data, the disease progression was stopped after the second and third evaluation. Stagnation at the level of item 8 – transfer from supine position to seated position and item 6 – raising the pelvis from supine position was also observed. Even though the patient can walk independently after the first week, the decrease of the average scores could be due to walking as a losing skill in muscular dystrophy.
D2: Proximal motor function
The data obtained for dimension 2 show no significant statistical difference between the first (week 1) and the second (week 12) or third evaluation, respectively (Fig.2). Also a decrease of the average scores after 24 weeks for item 10, movements such as bending forward from seated position, or item 13, incapacity of maintaining the correct position of the body in a seated position, can be noticed. This tendency could be due to the characteristic situation Table II
Dimensions and items of the Motor Function Measure (MFM).
Dimensions Item (I)
D1: Standing and transfers
I6– Supine, limbs half-flexed: raising the pelvis I8 – Supine: transfer to seated position
I11 – Seated on a mat: transfer to standing position without the support of the upper limbs
I12 – Standing position, feet slightly apart next to a chair: transfer to sitting position without the support of the upper limbs
I24 – Seated position, feet slightly apart: transfer to standing position without help or support
I25 – Standing position, with support: holding the standing position for 5 sec. without support
I26 – Standing position with support: transfer to standing position without support and lifting a lower limb from the ground
I27 – Standing position without support: bending forward, touching the floor with the fingers, standing up without support
I28 – Standing position without support: walking on the heels
I29 – Standing position in front of a line 6 m long, 2 cm wide: walk on the line
I30 – Standing position: run 10 m
I31 – Standing position on one foot within a circle with 60 cm diameter: hop on the spot 10 times without leaving the circle
I32 – Standing position: squat and stand up again twice in a row
D2: Axial and proximal motor
capacity
I1– Supine: hold the head for 5 sec in midline position, then turn it completely to the right, then completely to the left
I2 – Supine: raise the head, hold for 5 sec I3 – Supine: bring one knee to the chest
I5 – Supine: bring one hand to the opposite shoulder I7 – Supine: turn over onto the stomach and free both arms
I9 – Sitting on the mat: remain seated for 5 sec., lift arms, touch hands and hold contact for 5 sec. I10 – Seated position feet apart, tennis ball in front of the feet: bend and touch the tennis ball
I13 – Seated position on a chair, crossed upper limbs, hands on the shoulders: hold the position for 5 sec., straight back I14 – Seated position on a chair, head in complete flexion: raise head, hold for 5 sec.
I15 – Seated, forearms without elbows on the table: lifting the arms and placing the hands on the head at the same time
I16 – Seated, pencil on the table at the distance from the thorax corresponding to the upper limb, forearm on the table: extend the
elbow, raise the forearm from the table, touch the pencil
I23– Seated in front of a table placed at a distance of a forearm: place both hands on the table at the same time
D3: Distal motor capacity
I4 – Supine, knees at a 90 degree angle, leg parallel to the mat, foot in plantar flexion, lower limbs supported by the examiner: pull up the foot
I17 – Seated in front of a table with 10 coins in front: pick up the coins one at a time with one hand without sliding them on the
table and hold them in the same hand
I18 – Seated position, forearm on the table next to a CD attached to a piece of cardboard: go round the edge of the CD with the finger, the hand is lifted from the table
I19 – Seated, on the table a paper and a pen: draw a series of loops inside the rectangular frame and over its full length touching
the top and bottom line of the frame
I20 – Seated, the elbow resting on the table, holding an A4 paper folded in 4: tear the paper over at least 4 cm from where it was folded.
I21 – Seated, elbow and forearm on the table in pronation, tennis ball in the hand: pick up the ball from the table and turn hand
over to complete supination
I22 – Seated, the elbow rests on the table next to a diagram with 9 squares: move the finger to the 8 squares (the “start” is placed
The role of physical therapy in Becker’s muscular dystrophy
of the disease, taking into consideration the atrophy of axial and proximal muscles, so that compensatory movements are required.
D3: Distal motor function
The data obtained for dimension 3 (Fig. 3) show an increase (5%) of average scores at the third evaluation (week 24) as compared to the first evaluation (week 1), the difference being statistically insignificant. For item 19 it was noticed that the execution of some fine motor skills such as writing for the upper limb (draw a spiral in a rectangle) can be improved in time. The patient obtained a higher score for each item of dimension 3, except for item 4, maybe due to the specific limitation of the dorsal flexion of the leg due to the contraction of the Achilles tendon. Conclusions
1. Given the results obtained at the initial and final evaluation, we consider that the long term objectives are reached, especially taking into consideration the regressive characteristic of the disease (improvement of transfer capacity and prolonged maintenance of standing position; maintenance of proximal and axial motor capacity; maintenance of distal motor capacity).
2. The patient presents the same motor skills: he walks, runs, jumps, catches, handles and releases small objects with/from his hand as he has muscle strength, flexibility, skill, stamina, speed at a high level, as suggested by the results obtained for the three dimensions of the MFM test – standing and transfer, axial and proximal motor capacity and distal motor capacity – which were compared after applying these three evaluations.
Conflict of interests Nothing to declare. Indications
The research is based on the partial use of the results of the
first author’s graduation thesis.
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