Received: 26 July 2022 Revised: 26 May 2023 Accepted: 27 May 2023

DOI: 10.1002/mus.27921

CLINICAL RESEARCH ARTICLE

Generation of percentile curves for strength and functional

abilities for boys with Duchenne muscular dystrophy

Mariana Angélica De Souza Leon MSc, PhD 1 | Daiane Leite Da Roza MSc, PhD 2 |
1
Gabriela Barroso De Queiroz Davoli PT, MSc |
Cyntia Rogean De Jesus Alves De Baptista MSc, PhD 1 |
3
Claudia Ferreira Da Rosa Sobreira MD, PhD |
Ana Claudia Mattiello-Sverzut MSc, PhD 1

1
Department of Health Science, Ribeirão Preto
Medical School, University of São Paulo, São
Paulo, Brazil
2
Department of Epidemiology, School of
Public Health, University of São Paulo, São
Paulo, Brazil
3
Department of Neurosciences and Behavior,
Ribeirão Preto Medical School, University of
São Paulo, São Paulo, Brazil
Correspondence
Ana Claudia Mattiello-Sverzut, Department of
Health Science, Ribeirao Preto Medical School,
University of São Paulo Av. Bandeirantes,
3900, Campus, 14049-900, Ribeirão Preto, SP,
Brazil.
Email: acms@fmrp.usp.br
Funding information
Fundação de Amparo à Pesquisa do Estado de
São Paulo, Grant/Award Number:
2018/07581-2
Abstract
Introduction/Aims: Considering the heterogeneity of the clinical manifestations of
Duchenne muscular dystrophy (DMD), it is important to describe their various clinical
profiles. Thus, in this study we aimed to develop percentile curves for DMD using a
battery of measures to define the patterns of functional abilities, timed tests, muscle
strength, and range of motion (ROM).
Methods: This retrospective data analysis was based on the records of patients with
DMD using the Motor Function Measure (MFM) scale, isometric muscle strength (IS),
dorsiflexion ROM, 10-meter walk test (10 MWT), and 6-minute walk test (6 MWT).
Percentile curves (25th, 50th, and 75th percentiles) with MFM, IS, ROM, 10 MWT,
and 6 MWT on the y axis and patient age on the x axis were constructed using the
generalized additive model for location, scale, and shape, with Box-Cox power expo-
nential distribution.
Results: There were records of 329 assessments of patients between 4 and 18 years
of age. The MFM percentiles showed a gradual reduction in all dimensions. Muscle
strength and ROM percentiles showed that the knee extensors were the most
affected from 4 years of age, and dorsiflexion ROM negative values were noted from
the age of 8 years. The 10 MWT showed a gradual increase in performance time with
age. For the 6 MWT, the distance curve remained stable until 8 years, with a subse-
quent progressive decline.
Discussion: In this study we generated percentile curves that can help health profes-
sionals and caregivers follow the trajectory of disease progression in DMD patients.

KEYWORDS
motor function, neuromuscular disorders, rehabilitation, walking, weakness

Abbreviations: 10 MWT, 10-meter walk test; 6 MWT, 6-minute walk test; BCPE, Box-Cox
power exponential; D1, dimension 1 of Motor Function Measure scale; D2, dimension 2 of
1 | INTRODUCTION
Motor Function Measure scale; D3, dimension 3 of Motor Function Measure scale; DMD,
Duchenne muscular dystrophy; GAMLSS, generalized additive model for location, scale, and
The natural history of Duchenne muscular dystrophy (DMD), charac-
shape; MFM, Motor Function Measure; MRI, magnetic resonance imaging; ROM, range of
motion. terized by a progressive decline in muscle function and motor

198 © 2023 Wiley Periodicals LLC. wileyonlinelibrary.com/journal/mus Muscle & Nerve. 2023;68:198–205.

LEON ET AL.
capacity, is well-documented throughout the scientific literature.
However, in clinical practice, the rate of progression varies consider-
1,2
ably between patients. Considering the heterogeneity of clinical
manifestations1,3 and the absence of a correlation between patients'
clinical and genetic characteristics,4 it is important to describe the clin-
ical profile of a specific population so we can gain some insight into
the relationship between these factors.4
Usually, patients' motor abilities are monitored using motor scales
in addition to muscle strength and timed tests.5–7 Disease stage can
determine a factorial combination of the responses observed in mus-
cle strength and timed tests. For example, a 9-year-old patient pre-
senting with a knee extensor strength of zero can perform a 10-meter
walk test (10 MWT) in 10 seconds and cover 220 meters during the
6-minute walk test (6 MWT). A 13-year-old patient may walk
220 meters during the 6 MWT and reach 4 seconds in the 10 MWT
while achieving 4 kg with the knee extensors via dynamometry mea-
surements. In both cases, if we examined only the muscle strength
response or the outcome of the 6 MWT, we may incorrectly judge
their motor capacity. This hypothetical example demonstrates that an
isolated variable is not sufficient for characterizing the patient as a
whole. Corroborating this reasoning, Buckon et al. demonstrated that
combining information about muscle strength, muscle function, and
8
timed tests is relevant in detecting disease progression. Thus, per-
centile curves may be useful for monitoring these outcomes with dis-
ease progression.9
The development of percentile curves---such as those proposed by
the World Health Organization to assess children's weight and
height---is necessary to monitor the limitations imposed by the disease,
even though these curves do not consider the child's ethnic or cultural
background. Although motor functions decline throughout the clinical
course of DMD, a set of estimated curves developed by a single refer-
ence hospital containing the most common variables would be a good
resource to follow individual progression and increase the efficacy of
physiotherapy interventions. With the reasons just noted in mind, the
primary purpose of this study was to develop percentile curves for
DMD using a battery of assessment measures to define the patterns of
functional abilities, timed tests, muscle strength, and range of motion
(ROM). These insights are crucial for monitoring the evolution of motor
functions and evaluating early intervention measures in DMD patients.
2 | METHODS
This retrospective analysis uses data obtained from the records of
patients treated at the Clinical Hospital of Ribeirao Preto Medical
School of the University of São Paulo, Brazil (HCFMRP-USP), between
January 2010 and December 2018. This hospital is a tertiary public
hospital that assists patients from all regions of Brazil. The diagnosis
of DMD was based on the absence of dystrophin in a muscle biopsy
and/or the identification of a pathogenic mutation (deletion, duplica-
tion, or point mutation) in the dystrophin gene. The inclusion criterion
was patient age between 4 and 18 years. The exclusion criteria were:
(a) history of fractures or surgeries and (b) inability to understand
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199
simple instructions. Parents or guardians provided informed consent.
Children and adolescents assented to the study or, when appropriate,
provided informed consent. The study was approved by the ethics
committee of the HCFMRP-USP (No. 15508/2016).
Physical therapy records were compiled based on age (in years),
MFM scale score, isometric muscle strength (IS), dorsiflexion ROM,
and the 10 MWT and 6 MWT results. The database of admissions to
the outpatient rehabilitation center---a reference center for neuro-
muscular diseases---was queried to identify the first assessment. Gen-
eral information was collected from participating patients' medical
records and included age at first evaluation, age at symptom onset,
corticosteroid use, and physiotherapy follow-up. Patients were ques-
tioned about corticosteroid use and physiotherapy care because these
are considered standard care according to national and international
guidelines.10,11 The data presented here were derived from multiple
patient records, with an interval of 6 to 9 months between each
assessment, and include both ambulatory and nonambulatory
patients. The number of evaluations per patient depended on the
patient's age at admission throughout this study and on the patient's
hospital attendance on the return days. Trained physiotherapists per-
formed all necessary assessments.
The IS was evaluated using a handheld dynamometer (Lafayette
Instrument, Lafayette, UK). Following previous methodology,12 muscle
groups were measured bilaterally (hip flexors, knee flexors and exten-
sors, shoulder abductors, elbow flexors and extensors). The patients
performed three repetitions of maximal contractions maintained for
5 seconds (30-second interval between each contraction). The mean
value of the data obtained directly from the dynamometer (kilograms)
was used. The patients received the verbal encouragement command
of “push” during data acquisition. Interrater reliability was obtained
previously.12 Statistical analysis used right-sided values, in consider-
ation of the symmetrical effect of the disease. For ankle dorsiflexion
ROM, we used a classic goniometer.13 Measurements were obtained
using both the knee-extended and 90 knee-flexed motions.
The MFM was administered following the recommendations con-
tained in a manual validated in Brazilian Portuguese.14 Thirty-two items
comprised three dimensions, scored from 0 to 3 according to a patient's
performance on a task's execution: Dimension 1 (D1), standing position
and transfers, with 13 items; Dimension 2 (D2), axial and proximal motor
function, with 12 items; and Dimension 3 (D3), distal motor function,
with 7 items. The three dimensions provided a total score.14
The 10 MWT was performed for each patient without devices or
shoes, starting from the standing position. A standardized verbal com-
mand was used: “Go as fast as you can and only stop when I give you
the command to stop.” They received an explanation of the test
before starting, following the procedure outlined by Pizzato et al.5
and Baptista et al.15 The time required to walk or run was subse-
quently recorded. The 6 MWT was assessed according to the
American Thoracic Society's guidelines (modified test for DMD
patients)16: patients wearing shoes were instructed to walk comfort-
ably, with their distance walked recorded in meters. We used two
examiners: one to record the time, and another to record the distance
covered by staying close to the patient.
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200 LEON ET AL.

2.1 | Statistical analysis 146 assessments; IS, 230 assessments; and ROM, 258 assessments.
Descriptive statistics of the variables analyzed by age are presented in
Raw data are presented as mean and standard deviation (SD) and fre- Supplementary Table 1.
quency. To facilitate clinical use, we constructed percentile curves Regarding MFM, DMD patients 4 and 5 years of age demon-
(25th, 50th, and 75th percentiles) with MFM, IS, ROM, 10 MWT, and strated mean percentages of D1, D2, and D3, and a total score
6 MWT on the y axis and the patient's age on the x axis by using the between 87% and 99% (Supplementary Table 1). In contrast, 16-year-
generalized additive model for location, scale, and shape (GAMLSS) old boys demonstrated lower percentages of D1 (<5%). A gradual
with a Box-Cox power exponential (BCPE) distribution.15 The BCPE reduction in the total MFM score was observed between 5 and
distribution has four parameters: μ, σ, ν, and τ. These parameters rep- 18 years of age (92.3% and 36.2%, respectively). According to MFM
resent the location (median), scale (approximate coefficient of varia- data, most patients showed some degree of ambulation for up to
tion), skewness (power transformation to symmetry), and kurtosis 16 years of age (Supplementary Table 1). Wheelchair use started at
(degrees of freedom or power exponential parameter), respectively. It 7 years of age (one wheelchair user), reaching 28 wheelchair users at
is an appropriate distribution given that these parameters are useful age 13 (Supplementary Table 1).
for data exhibiting both skewness and kurtosis, and they allow for the Using the 6 MWT, patients showed fluctuations in the distance
determination of z scores at any point in the distribution. The percen- covered from 4 to 12 years (maximum mean 332.7 meters, minimum
tiles for a given age group were calculated using the values of the four mean 240.5 meters). From 12 years of age, most DMD patients were
parameters for the corresponding age groups. The use of GAMLSS wheelchair users (Supplementary Table 1).
models with a BCPE distribution to develop percentile growth curves Regarding the 10 MWT, the time to complete the test increased
is recommended in the existing body of literature because they pro- with age (mean of 5.2 seconds and 11.93 seconds for patients 4 and
vide highly flexible models for various types of distributions with dif- 15 years of age, respectively), but the number of assessments of this
ferent characteristics.16 Residual quantile plots were used to assess test that each patient could perform decreased from 23 at 10 years of
the goodness of fit of each model component. Statistical analyses age to 3 at 15 years of age (Supplementary Table 1).
were performed using SAS software version 9.4 for Windows (SAS All assessed muscle groups exhibited strength reductions over
Institute) and R software version 3.6.3 (R Core Team). The GAMLSS time (Supplementary Table 1). The highest strength value was reached
package of the R software was used to construct the curves. at 7 years of age for knee extensor muscles (mean 7.5 [SD 4.1]) and
the lowest for elbow flexor muscles at 18 years of age (0.9 [0.7]).
Knee extensor muscles demonstrated the largest reduction in strength
3 | RESULTS over the years (5.2-kg decline) (7.5 [4.1] at 7 years and 2.3 [1.7] at
18 years), whereas elbow extensor muscles demonstrated the smallest
Seventy-three patients met the inclusion criteria for this study. The reduction (2.5-kg decline) (3.8 [2.8] at 5 years and 1.3 [1.1] at
mean age at symptom onset was 4.0 (SD 2.1) years (Table 1). 18 years). Regarding DF ROM, the highest value was observed at
All patients were treated with glucocorticoids. There were 4 years of age, both with the knee flexed and extended (8.0 ; 14.0
329 recorded assessments of patients between 4 and 18 years of age: [8.5], respectively), and negative values were noted from 8 years of
MFM scale, 329 assessments; 6 MWT, 116 assessments; 10 MWT, age with the knee extended ( 2 [6.1]) and 9 years of age with the
knee flexed ( 7.8 [16.1]).
Percentile curves for measures are presented in Figures 1 through 4.
TABLE 1 Characterization of patients with Duchenne muscular
dystrophy Regarding MFM percentiles, a gradual reduction was observed
across all dimensions. For D1, there was a sharp decline in score
Mean (SD) Range
between 10 and 12 years of age, and, for both D3 and total score, the
Age (years) (years) (years)
score reduction was slight and linear over the progression of years
Age at onset of symptons (n = 73) 4.0 (2.1) 0.5–9.0
(Figure 1). For D2, the 25th and 75th percentile curves show a spread
Nonambulatory at time of first 9.5 (1.6) 6.0–13.0
from 14 years of age (Figure 1).
evaluationa (n = 21)
The percentiles of muscle strength show that the less-
Characteristics Rating Percentage
preserved muscle groups were the knee and elbow extensors,
Diagnosis DNA 64% followed by the hip and elbow flexors, which had time curves
Biopsy 19% showing a downward slope between 5 and 10 years of age
DNA + biopsy 17% (Figure 2). Isometric muscle strength of the knee flexors and shoul-
Physiotherapy Yes 69% der abductors showed stable time curves up to 12 years of age,
No 31% followed by a decline in subsequent years.

Abbreviation: SD, standard deviation.

A gradual reduction in dorsiflexion ROM was observed (with the
a
Performed at the physiotherapy outpatient clinic of the rehabilitation knee extended and flexed) (Figure 3). In Figure 3, both (knee flexed
service. and knee extended) are similar in shape, but the time curves on the
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LEON ET AL. 201

F I G U R E 1 Percentile curves for motor function measure (MFM). Individual assessments appear as data points for each age. D1, Dimension
1 (standing position and transfers, 13 items); D2, Dimension 2 (axial and proximal motor function, 12 items); D3, Dimension 3 (distal motor
function; 7 items) and Total Dimension (32 items).

FIGURE 2 Percentile curves for isometric muscle strength. Individual assessments appear as data points for each age. IS, isometric strength.

left are slightly displaced upward in relation to the figure on the right, In the 10 MWT, a gradual increase in performance time was
demonstrating greater degrees of freedom in the ankle passive motion observed with age progression, and the slope of the test performance
for the first. time curve became steeper after 10 years of age (Figure 4). For the
 10974598, 2023, 2, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/mus.27921 by <Shibboleth>-member@javeriana.edu.co, Wiley Online Library on [11/09/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
202 LEON ET AL.

FIGURE 3 Percentile curves for range of motion (ROM). Individual assessments appear as data points for each age.

F I G U R E 4 Percentile curve for

10-meter walk test (10 MWT). Individual
assessments appear as data points for
each age.

F I G U R E 5 Percentile curve for

6-minute walk distance (6 MWD).
Individual assessments appear as data
points for each age.

LEON ET AL.
6 MWT, the curve for distance traveled remained stable until 8 years
of age, with a progressive decline thereafter (Figure 5).
4 | DISCUSSION
In this study we have established percentile curves from more than
one test, with a set of variables closely related to each other and
which are widely used in clinical practice for the evaluation of DMD
patients. The percentile curves highlighted a progressive decline in
motor function, a sharp decline in knee and elbow extensor muscle
strength from years 4 to 10, the evident worsening in timed tests, and
a pathological shortening in ankle plantar flexors at 9 years of age.
In our study, in which all patients used corticosteroids, the MFM
percentile curves and knee extensor muscle strength percentile curves
are close to the percentile curves of the patients treated with cortico-
steroids from the studies by Hafner et al.9 and Buckon et al.8 Cortico-
steroids improve muscle strength and, in combination with the
management of the ankle contractures, contribute to the maintenance
17
of walking, climbing, and standing.
The most severe loss of knee extensor strength begins at 4 years
of life. This can be explained by analysis with magnetic resonance
imaging (MRI), which showed that patients with more significant func-
tional damage have a higher degree of muscle fiber degeneration in
18
the lower limbs. Specifically, in the case of knee extensors, the
higher the measured muscular fat fraction, the lower the subsequent
19
measured muscle strength. This loss of knee extensor muscle
strength is a predictive factor for timed tests. Thus, the lower the
degree of muscle strength, the worse the patient's performance in
timed tests, as shown in previous studies with the 10 MWT.5,17
20
In agreement with the literature, our study has shown a promi-
nent decline in the D1 scores on the MFM scale starting at age
10, which was accompanied by a steepening of the percentile curves
of the time to perform the 10 MWT and a reduction in distance on
the 6 MWT starting at the same age. The timed tests and MFM scale
assess disease severity and are correlated with muscle degeneration
as measured by MRI.20 The association between the 10 MWT and D1
of the MFM results is able to distinguish between ambulatory and
nonambulatory patients.21 In addition, D1 of the MFM is valid, reli-
able, and provides a unidimensional measure of motor function in
ambulatory DMD patients. Nevertheless, D2 and D3 of the MFM
must be used with caution with this group of patients.22
5
According to Pizzato et al., the 10 MWT can be used as a predic-
tor of gait loss.5 Those authors found that, between two consecutive
tests, if the ratio of increase in time is over 1.25, then the patient
would become wheelchair-dependent.5 A ratio of 1.1 on the 10 MWT
and a score of 26 on the MFM-D1 may represent a red flag for DMD
ambulation status. Awano et al.23 studied longitudinal changes in
10 MWT performance of 145 DMD patients over 19 years of age and
found that 97.4% of them were unable to perform the test by
23
15 years of age. This result coincides with ours, as we had no
patients with ambulatory ability by the age 16. In addition, among
patients 10 to 13 years of age, the same study showed an inversion
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203
between proportion of ambulatory and nonambulatory patients,
which was also shown by Awano et al.23
In the 6 MWT, the distance traveled curve remained stable until
8 years of age, with a progressive decline thereafter. McDonald
et al.24 reported an annual improvement in 6 MWT performance for
patients younger than 7 years of age (>350 meters) and an annual
decline in those older than 7 years.24 Our curves regarding the
25th, 50th, and 75th percentiles do not demonstrate a visual incre-
ment, but instead a nearly a flat line up until 7 years of age. The best
explanation for our findings was reported by Mercuri et al.25 They
reported four trajectory classes following the patterns of the
6 MWT: a fast decline (approximately 117 meters per year), a mod-
erate decline (approximately 50 meters per year), stable (approxi-
mately 10 meters decline after 3 years), and improved (gain of
approximately 100 meters over 3 years).25 The authors also
observed that most patients had a “stable” pattern, followed by the
“fast,” “moderate,” and “improvement” patterns. The youngest
showed an “improvement” pattern, with those older than 7 years of
age having a “fast decline” pattern.25
The decline in walking ability measured by the 6 MWT occurs
despite small changes in strength values of the knee extensor muscles
across 1 year of longitudinal evaluation.6 This may be explained by
the fact that the 6 MWT and muscle strength of the knee extensor
have a negative and significant correlation with muscle fat.21,26 Thus,
as the disease progresses, the amount of fat in the muscle tissue
increases, emphasizing knee extensor weakness and, consequently,
worsening of the patient's performance on the 6 MWT.
Despite being influenced by knee muscle strength, the timed test
results are also influenced by dorsiflexion ROM.17 The study's percen-
tile curve for dorsiflexion ROM (with knee extended and knee flexed)
indicates that negative values of DF occurred from years 4 to 10 for
the majority of patients (considering the 25th and 50th percentile
time curves). These results corroborate those of Willcocks et al.,27
suggesting that, with advancing age and disease progression, an
accentuation in muscle shortening and damage to functional abilities
occur.
The limitations of the study are as follows: (1) across the age
range of 4 to 18 years, the number of participants analyzed was dif-
ferent, with fewer patients at younger (4 to 6 years) and older (16 to
18 years) ages; (2) throughout the study period, the number of evalua-
tions for each of the variables analyzed was different; (3) because this
was a study developed in a tertiary hospital in the interior of São
Paulo State, the number of evaluations was limited, even though we
compiled data from 8 years of DMD patients' care; (4) we analyzed
only those patients who regularly took corticosteroids; and (5) analyses
of subgroups based on genetic findings in the DMD gene were not
performed. Future studies should fill these gaps and aim to address
the association between patients' activity levels and psychosocial
scales.
In conclusion, we have generated percentile curves that can help
health professionals and caregivers to track and predict the disease
trajectory of boys with DMD. The most important variables typically
used to evaluate this disease are included in the percentile curves.

204
AUTHOR CONTRIBUTIONS
Mariana Angélica De Souza Leon: Investigation; methodology;
writing – original draft; writing – review and editing. Daiane L. da
Roza: Data curation; formal analysis; methodology; writing – original
draft; writing – review and editing. Gabriela Barroso de Queiroz
Davoli: Investigation; writing – original draft; writing – review and
editing. Cyntia Rogean de Jesus Alves Baptista: Conceptualization;
supervision; writing – original draft; writing – review and editing.
Claudia Ferreira Da Rosa Sobreira: Conceptualization; methodology;
project administration; supervision. Ana Claudia Mattiello-Sverzut:
Conceptualization; project administration; supervision; writing – original
draft; writing – review and editing.
ACKNOWLEDGMENTS
The authors thank the participants of this study and their caregivers,
along with Joyce Aline Paganelli, Larissa de Oliveira Okama, Marjory
I. Bena, Livia M. Zampieri, and Ananda Cezarani. D.L.R. received fellow-
ships from the São Paulo Agency Foundation (Grant No. 2018/07581-2).
DATA AVAI LAB ILITY S TATEMENT
The authors will make available the data from this study in the supple-
mentary material.
E THI CAL P UBLI CATI ON S TAT EME NT
We confirm that we have read the Journal's position on issues
involved in ethical publication and affirm that this report is consistent
with those guidelines.
ORCID
Mariana Angélica De Souza Leon https://orcid.org/0000-0003-
4274-1301
Daiane Leite Da Roza https://orcid.org/0000-0003-2911-2236
Gabriela Barroso De Queiroz Davoli https://orcid.org/0000-0002-
4981-3868
Cyntia Rogean De Jesus Alves De Baptista https://orcid.org/0000-
0001-5850-9940
Claudia Ferreira Da Rosa Sobreira https://orcid.org/0000-0002-
7420-1644
Ana Claudia Mattiello-Sverzut https://orcid.org/0000-0002-4181-
0718
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How to cite this article: Leon MADS, Roza DLD,
Davoli GBDQ, Baptista Cyntia Rogean De Jesus Alves De,
Sobreira CFDR, Mattiello-Sverzut AC. Generation of
percentile curves for strength and functional abilities for boys
with Duchenne muscular dystrophy. Muscle & Nerve. 2023;
68(2):198‐205. doi:10.1002/mus.27921