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Neuromuscular Disorders 22 (2012) 980–986

www.elsevier.com/locate/nmd

Quadriceps strength is a sensitive marker of disease progression

in sporadic inclusion body myositis
Yves Allenbach a,1, Olivier Benveniste a,b,⇑,1, Valérie Decostre c, Aurélie Canal c,
Bruno Eymard b, Serge Herson a,b, Coralie Bloch-Queyrat a, Jean-Yves Hogrel c
a
Université Pierre et Marie Curie, Assistance Publique – Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France
b
Université Pierre et Marie Curie, Assistance Publique – Hôpitaux de Paris, GH Pitié-Salpêtrière, Centre de Référence des Pathologies
Neuromusculaires Paris Est, Institut de Myologie, Paris, France
c
Institut de Myologie, GH Pitié-Salpêtrière, UPMC UM 76, INSERM U 974, CNRS UMR 7215, Paris, France

Received 17 January 2012; received in revised form 29 March 2012; accepted 11 May 2012

Abstract

There are currently no effective treatments to restore the muscle function in sporadic inclusion body myositis. Natural history studies
of this disease are scarce. The goal of this study consisted in defining the functional pattern of patients with sporadic inclusion body
myositis and to follow its change over a 9-month period to determine the most sensitive outcome measures for future clinical trials.
Twenty-two patients with definite sporadic inclusion body myositis were assessed using clinical and functional scales. Dynamometry
was used to evaluate the strength for hand grip and wrist, elbow, ankle and knee flexion and extension. Among the patients, 16 were
reassessed 9 months later. The mean whole composite index was at 43.3 ± 16.5% of the predicted normal values. The weakest muscle
functions were hand grip, wrist flexion and elbow flexion at the upper limbs and knee extension and ankle flexion at the lower limbs.
Muscle weakness was generally asymmetrical, especially for upper limbs where all tested functions were significantly stronger at the
dominant side. The patient strength was correlated with the disease duration only for knee extension, which was also the only muscle
function to change significantly over 9 months. Knee extension strength seems to be the most relevant marker of disease progression
in sporadic inclusion body myositis when measured with suitable dynamometry.
Ó 2012 Elsevier B.V. All rights reserved.

Keywords: Inclusion body myositis; Natural history; Outcome measures; Strength; Dynamometry

1. Introduction approaches needs sensitive and reproducible evaluation

methods to assess their effect on the neuromuscular func-
Sporadic inclusion body myositis (sIBM) is the most tion. Evaluation of strength in sIBM patients is challenging
common acquired inflammatory myopathy in patients over because of the asymmetric weakness of both proximal and
50 years of age [1]. The physiopathology is unknown but distal upper and lower limb muscles and the slowness of its
degenerative and auto-immune inflammatory mechanisms progression [3]. Natural histories are rather scarce and lim-
are involved [1,2]. To date, there is no validated treatment ited in the number of patients involved. Muscle strength
for this disabling disease. Development of new therapeutic was measured using either handheld dynamometry
(HHD) [4] or fixed dynamometry (QMT: Quantified Mus-
cle Testing) [5,6] and was shown to decrease in most
⇑ Corresponding author. Address: Service de Médecine Interne 1, patients, but not all.
Groupe Hospitalier Pitié-Salpêtrière, 47-83, boulevard de l’Hôpital, As new treatments will emerge, robust methods to assess
75651 Paris Cedex 13, France. Tel.: +33 142161088; fax: +33 142161058. their effects are needed. This study describes the pattern of
E-mail address: [email protected] (O. Benveniste).
1 neuromuscular abilities of patients suffering from sIBM
These authors contributed equally to the manuscript.

0960-8966/$ - see front matter Ó 2012 Elsevier B.V. All rights reserved.
http://dx.doi.org/10.1016/j.nmd.2012.05.004
 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986 981

using various functional rating scales and dynamometric

measures of strength. The aim was to assess the motor
function changes within a period of time of 9 months and
to determine the most sensitive outcome measures over this
period.

2. Materials and methods

2.1. Patients

All patients involved in the study had definite sIBM

(i.e. pathological examination of their biopsies showed fibres
invaded by lymphocytes, vacuoles and amyloid deposits) [7]
and were not treated by any immunosuppressive or
immunomodulator drugs for more than 6 months before
inclusion. Creatine kinase level at baseline was 434 ±
296 UI/l. The institutional review board approved the study
protocol (ClinicalTrials.gov Identifier: NCT00898989); all
patients were enrolled after written, informed consent was
obtained. Patients were evaluated during two visits
9 months apart.

2.2. Clinical and functional evaluations

During each visit, the same physician performed a con-

ventional clinical muscle strength evaluation by manual
muscle testing (MMT using the Medical Research Council
(MRC) scale) of 32 muscle groups. Patients were also
assessed using two non-specific functional scales (Walton
(functional scales evaluating functions of lower limbs) [8]
and Rivermead Mobility Index (RMI) [9]) and two specific
scales (sIBM weakness composite index (IWCI) [3] and
Inclusion Body Myositis Functional Rating Scale
(IBMFRS) [10]). The walking ability of the patients was
assessed by a six minute walk test.

2.3. Strength assessment

Fig. 1. Strength measurements of several muscle functions by dynamom-
Specific dynamometric measurements were performed to etry. (A) Hand grip. (B) Wrist flexion and extension. (C) Ankle extension.
(D) Ankle flexion. (E) Knee flexion and extension. (F) Elbow flexion and
measure hand grip strength and extension and flexion tor- extension.
ques for wrist, elbow, ankle and knee. All the tests were
performed according to standardized operating procedures respect to the hand length. The MyoWrist sensitivity is
(Fig. 1) and were always ordered as follows: grip, wrist, 0.01 Nm.
ankle, 6MWT, knee and elbow. Rest periods of 15 s or Ankle flexion and extension were measured on a specific
more if needed were respected. device (namely, the MyoAnkle) (respectively, Fig 1C and
Isometric hand grip strength was measured using the D). The ankle dynamometer was designed to measure the
MyoGrip handle (Fig. 1A) [11]. Its sensitivity is 10 g. isometric strength generated around the ankle joint in the
Wrist flexion and extension strength was assessed by extension and flexion directions. It consists in an alumin-
measuring the maximal isometric torque generated on a ium plate below which are held two load cells on which
torque meter (Fig. 1B). The device (namely, the MyoWrist) strain is applied through an inelastic strap going through
is equipped with a foam cradle and two Velcro straps in slots in the plate. The strap is placed and held either on
order to firmly maintain the forearm within the cradle. dorsal part of the foot at the level of the first metatarsian
Two vertical bars are positioned at the elbow level aiming for flexion measurement, or above the knee at the level of
to avoid lateral movements of the elbow. A U-shaped sup- epicondyles for extension measurement. To ensure a larger
port is fixed over the torque meter to receive the hand palm and stiffer support on the knee, the strap was reinforced
within a dense foam cradle. This support is adjustable with with a composite material made of two layers of dense
982 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986

foam between which was held a stiff but flexible metal distally on the thigh in regard with the malleolus. The sub-
sheet. The sensitivity of the MyoAnkle is 0.01 kg. The data ject was asked to pull against the strap by extending its
of force were translated into torque by measuring the lever ankle while pushing with the sole of the foot. The length
arm to the nearest millimetre (see next paragraph). of the lever arm was carefully measured between the distal
Knee (Fig. 1D) and elbow (Fig. 1E) extension and flex- extremity of the lateral malleolus and the fifth metatarso-
ion torques were assessed using a Biodex 3 pro dynamom- phalangeal joint. The torque was computed as the product
eter in isometric conditions. The sensitivity of such a of the force (in Newtons) and the lever arm (in meters).
dynamometer is 0.7 Nm. For knee and elbow extension and flexion assessment,
subjects were seated in the Biodex chair with a hip flexion
2.4. Experimental procedures of 95°. Stabilization straps were placed across the trunk
and around the waist. For the knee, the knee joint axis
All strength measurements were performed in isometric was aligned with the measurement axis of the system.
conditions (Fig. 1). The patients were asked to produce The thigh was strapped around the mid-thigh of the leg
maximal voluntary isometric contractions. For each muscle to be tested. The knee angle was placed at about 90° to
function, the maximum of two reproducible (within 10%) have the leg vertically, in order to cancel the effect of grav-
trials was kept as the maximum voluntary isometric con- ity. For the elbow assessment, the upper arm was placed
traction (MVIC) of the evaluation session. The first side horizontally in a support on which it was firmly attached,
tested for each dynamometer was chosen as random. the angle between the arm and the sagittal plane adjusted
Strong verbal encouragements were given to the patients to 20°. The elbow rotation axis was precisely aligned with
to stimulate them to produce their true MVIC. the measurement axis of the system. The elbow was placed
For grip strength assessment, the handle width was at about 90° to have the forearm vertically, in order to can-
adapted to the hand size of the subject. The patient was cel the effect of gravity. The forearm was in a neutral posi-
evaluated sitting on a plinth or in their wheelchair with tion of pro-supination.
the armrests removed, the knee and hip at 90°, the trunk The 6MWT was performed in a corridor, between two
in an upright position. The height of the plinth could be cones separated by a distance of 25 m. The test was per-
adjusted so that the feet lay flat on the floor. The subject formed in comfortable shoes. Walking aids were accepted.
sat close from the lateral border of the seat, the upper limb Standardized verbal instructions were given every minute.
to be evaluated extended along the vertical direction. The Same conditions were respected in both visits.
contralateral hand remained restful on the thigh. One hand
of the evaluator held the wrist of the patient to avoid com- 2.5. Data analysis
pensatory movements and the other hand supported the
measurement device so that the patient did not need to All the data for torque and 6 min walk distance were
carry it. converted into percentages of normal reference values from
For wrist extension and flexion assessment, the subjects strength databases and predictive equations [12]. For hand
were tested sitting on a chair or in their wheelchair with the grip, wrist and ankle flexion and extension, norms obtained
armrests removed along a plinth with adjustable height. on 288 healthy adult subjects were used to compute per-
The trunk was in an upright position. The forearm was centage of predictive normal theoretical strength from mul-
positioned horizontally within the cradle placed on the tilinear models with age, sex and weight as variables. For
plinth. The height of the plinth was adjusted so that the knee and elbow extension and flexion, norms obtained on
arm was positioned at 45° (±10°) shoulder abduction and 270 healthy adult subjects were used with multilinear mod-
at 30° (±10°) shoulder flexion. The elbow angle was set els using the same variables [13]. Composite strength scores
at 110° (±10°). The feet were flat on the floor. The contra- were computed for the upper limb, the lower limb and the
lateral hand was kept on the thigh. The forearm was in whole body.
neutral prosupination position and the axis or rotation of Wilcoxon signed rank tests were used to test the differ-
the wrist was aligned with the axis of the torque meter. ence for strength between genders and between dominant
The hand palm was then firmly entered and locked in the and non-dominant sides and for strength changes over
hand support such as the wrist was in neutral position with 9 months. Spearman rank correlation coefficients (Spear-
regard to extension and flexion. man Rho) were computed to assess correlations between
For ankle extension and flexion assessment, subjects sat functional scales and strength features. The level of statis-
upright with the hip, knee and ankle flexed at 90°. The sub- tical significance was set at 0.05.
jects were seated on a plinth which height was adjustable to
obtain a 90° knee angle; the leg being vertical or in their 3. Results
wheelchair. The foot was flat on the dynamometer. For
ankle flexion (dorsiflexion) measurement, the top of the 3.1. Baseline measures
foot was tightly strapped to lock up the ankle. The patient
was asked to pull up against the strap to flex the ankle. For Twenty-two patients (8 men, 14 women) were
ankle extension (plantarflexion), the strap was placed recruited with a mean age of 71.2 ± 8.1 years old. The
 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986 983

patients were diagnosed about 5 years after the first symp- Fig. 2). At lower limbs this dominance effect was not
toms which onset was at 59.6 ± 9.3 years for men and observed. For example, ankle flexion was significantly
62.4 ± 10.4 years for women. The duration of the disease stronger at the dominant hand side compared to non-dom-
at the beginning of the study was 9.8 ± 5.1 years. Two inant hand side (p = 0.049), whereas the opposite was
patients were wheelchair dependant and 13 used a walk- observed for knee flexion, which was weaker at the domi-
ing device occasionally or permanently. All patients were nant hand side compared to the non-dominant side
right handed. (p = 0.002). Furthermore, no significant difference of later-
The baseline measures were performed in all of the 22 ality was observed concerning ankle and knee extension.
sIBM patients except ankle extension which was not per- MMT could not detect this asymmetry.
formed by one patient due to feet oedema. As expected,
we observed a significant alteration of the muscular
strength compared to normal values for all muscle groups 3.2. Correlations between clinical, motor and strength
tested (Fig. 2) with a mean whole composite index of variables
43.3 ± 16.5 % of the predicted normal values. The weakest
muscle functions were hand grip, wrist flexion and elbow A significant correlation was observed between the whole
flexion at the upper limbs and knee extension and ankle strength composite index and all others functional tests, par-
flexion at the lower limbs. The most spared muscle func- ticularly the specific functional scales IWCI and IBMFRS
tions were wrist, elbow and ankle extensions. The same (Table 1). The scales were also significantly correlated with
overall pattern of muscle weakness was observed from most of the muscle functions tested. IWCI and IBMFRS
MMT measurements (Fig 3). were better correlated with upper limb muscle functions,
Muscle weakness was generally dissimilar between sides, while Walton score and RMI were better correlated with
especially for upper limbs where all tested functions were lower limb muscle function. So did also the 6MWT.
significantly stronger at the dominant side (except for The patient strength was correlated with the disease
elbow extension for which only a trend was observed, see duration only for knee extension (Spearman Rho:

% predicted

0 10 20 30 40 50 60 70 80 90 100

Upper limb Hand grip

*
left
right
Wrist flexion
*

Wrist extension
*

Elbow flexion
*

Elbow extension

Lower limb Ankle flexion

*

Ankle extension

Knee flexion
*
Knee extension

Fig. 2. Distribution on muscle weakness on the non-dominant and dominant sides for all the muscle functions tested by dynamometry. *Significantly
different between sides (Wilkoxon signed rank test). Data are means + standard deviation.
984 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986

100
shoulder abductors
Left
ankle extensors 90 Right

% predicted normal strength

neck extensors 80

hip adductors 70

hip abductors 60

elbow flexors 50
elbow extensors 40
finger extensors
30
wrist flexors
20
knee flexors
10
hip flexors
0
neck flexors 0 5 10 15 20
palmar interosseous Disease duration (years)

ankle flexors Fig. 4. Relationship between knee extension strength and the disease
opponent of the thumb duration.
finger flexors
knee extensors

0 1 2 3 4 5 percentage of predicted normal values (Table 2). Only knee

mean MRC score extension changed in a significant way over 9 months,
whereas a trend towards a decrease was clearly detectable
Fig. 3. Distribution on muscle weakness for all the muscle functions tested for hand grip and elbow flexion. Composite scores com-
by MMT.
puted on the upper limb, the lower limb and the whole
body did not significantly change (Fig. 5).
For clinical and functional scales, MMT scores, Walton
0.721, p = 0.001 for left side; 0.680, p = 0.002 for right score, IWCI, IBMFRS and 6MWT did not significantly
side) (Fig. 4). change over the 9 months of follow-up. Only RMI pre-
sented a significant change (see Table 2).
3.3. Changes after 9 months
4. Discussion
The baseline data and changes over 9 months were com-
puted for the 16 patients who completed the follow-up The present study provides the clinical, motor and
study. Six patients were not reassessed, mostly for organi- functional profiles of 22 patients suffering from sporadic
zational reasons. The changes over 9-months are provided inclusion body myositis and the natural history over
for strength measured by dynamometry and expressed in 9 months of this disease for 16 of these patients.

Table 1
Correlations (Spearman Rho) between functional tests and muscle strength assessed by dynamometry.
Upper limb
Hand grip Wrist flexion Wrist extension Elbow flexion Elbow extension Composite score
Left Right Left Right Left Right Left Right Left Right
* * * * *
6MWD 0.474 0.690 0.543 0.538 0.626 0.533
* * * *
Walton 0.482 0.668 0.463 0.479 0.578 0.429 0.511
* *
RMI 0.501 0.666 0.551 0.501 0.627 0.682 0.457 0.459 0.583
IWCI 0.642 0.740 0.606 0.818 0.733 0.611 0.808 0.798 0.729 0.699 0.824
IBMFRS 0.556 0.816 0.520 0.758 0.671 0.526 0.784 0.752 0.707 0.664 0.772
Lower limb Whole body
Ankle flexion Ankle extension Knee flexion Knee extension Composite score Composite score
Left Right Left Right Left Right Left Right
* *
6MWD 0.724 0.707 0.564 0.607 0.502 0.482 0.781 0.741
*
Walton 0.544 0.616 0.538 0.569 0.626 0.625 0.593 0.834 0.710
*
RMI 0.498 0.766 0.682 0.613 0.600 0.600 0.539 0.821 0.791
* * * *
IWCI 0.499 0.556 0.610 0.598 0.605 0.873
* * *
IBMFRS 0.511 0.552 0.561 0.849 0.890 0.670 0.883
*
No significant correlation was observed. When p < 0.005, the cells are italicized.
 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986 985

Table 2 pattern by including finger flexor and quadriceps weakness

Changes of outcome measures over 9 months. The percentages of change as diagnostic criteria for sIBM [7]. This pattern of muscle
were computed as relative changes with respect to the baseline values.
weakness was also described in previous studies using
Baseline SD % Change over SD p MMT [4,14,15], handheld dynamometry [4] or QMT [6]
9 months Value
and corroborates previous findings stating that knee exten-
QMT sors and finger flexors are the most severely affected mus-
Composite score upper 37.99 16.49 4.35 11.83 0.205
limb
cles [15].
Hand grip 31.99 19.31 4.66 13.51 0.070 Using whole body composite strength score we
Wrist flexion 38.62 18.80 2.84 16.94 0.469 observed a decrease of 1.6% of the predicted normal val-
Wrist extension 57.88 17.68 4.12 22.30 0.679 ues during 9 months of follow up corresponding to a
Elbow flexion 44.07 20.60 5.80 10.64 0.098 mean decrease of 0.2% per month. This decrease is
Elbow extension 60.75 27.08 5.75 13.97 0.148
Composite score lower 44.18 16.88 6.51 13.06 0.109
lower than the one observed by Rose et al. with a
limb decrease of 0.7% per month (QMT, n = 11) [6], by Lind-
Ankle flexion 37.43 15.51 6.17 25.22 0.433 berg et al. with a decrease of 1.4 % per month (hand-
Ankle extension 67.07 30.93 2.95 17.39 0.605 held dynamometry, n = 10) [4] or by Dalakas et al. with
Knee flexion 48.11 21.55 6.88 15.24 0.163 a decrease of 1.2% per month observed during a pilot
Knee extension 27.55 25.66 12.79 14.80 0.026
Composite score total 40.71 15.01 3.30 8.68 0.301
study testing efficacy of Alemtuzumab (QMT, n = 13)
[5]. However, the procedures for strength measurements,
Mean MMT score 3.73 0.69 2.29 13.75 0.477
the muscle functions tested and the methods of computa-
6MWD 63.81 19.69 2.26 11.01 0.551
Walton 5.19 2.17 2.80 18.59 0.666 tion of the composite scores were not always the same.
RMI 10.69 3.79 7.00 32.78 0.011 Methods to compute the percentages of changes may also
IWCI 56.56 23.50 1.50 17.18 0.727 differ since they can be expressed as percentage of changes
IBMFRS 28.31 8.17 2.60 13.03 0.455 with respect to baseline, or in variation of percentage of
QMT and 6MWD data are expressed in % predicted normal values. The predicted normal values. All these methodological differ-
scales are expressed at baseline in their own units. ences make difficult the comparison of the results. By
instance, computing a composite strength score with val-
80 ues expressed in Newton meter (except for some functions
like hand grip or pinch which are by default in Newton)
70
composite MVIC score (% predicted normal)

may not be consistent because ankle extension torque is

naturally about four times stronger than ankle flexion tor-
60
que. Thus, composite scores must be computed from rel-
ative values expressed, as percentage of predicted normal
50
values or as Z-scores. In the present work, strength data
40 were transformed in percentages of normal values calcu-
lated from predictive equations. Furthermore in a sIBM
30 clinical trial, age and weight of patients may change sig-
nificantly during the course of treatment. This underlines
20 the interest to recompute the relative strength at each
measurement sessions especially in a slow progressive dis-
10 ease involving elderly people.
The decrease of whole body composite strength score
0
observed in the present study at months 9 were not signif-
0 50 100 150 200 250 300
icant. The same observation was made after 6 months of
time (days)
follow-up by Rose et al. using a similar index [6]. The anal-
Fig. 5. Composite MVIC score obtained by dynamometry in 16 patients yses of each muscle groups showed significant decrease of
assessed 9 months apart. strength only for knee extension in line with other studies
[4,6,15]. From the results of the present study, the particu-
At upper limbs, muscle strength was stronger at the dom- lar role of knee extension strength needs to be underlined
inant side than at the non-dominant side in all the 22 patients owing to its direct correlation with the disease duration,
(which were right handed) as it was previously reported its significantly detectable change over 9 months of natural
[14,15]. Indirectly, it may suggest that physical activity could history and its consequent effect on motor function, partic-
be achievable and efficient in sIBM patients. Actually, a ularly on gait abilities.
recent study showed that an aerobic exercise program could Knee extension strength is also closely related to the
improve muscle strength in such patients [16]. 6MWD, which can be explained by the direct link
In this study we observed at baseline that hand grip and between quadriceps strength and gait kinematics and
knee extension were the most altered muscle function in kinetics [17]. This is also in accordance with the recent
sIBM patients, as Griggs et al. already emphasized this transversal study of Lowes et al. on 85 patients where
986 Y. Allenbach et al. / Neuromuscular Disorders 22 (2012) 980–986

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This study was supported by the Société Francßaise de
Médecine Interne (SNFMI) and the Association Francßaise
contre les Myopathies (AFM).