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com
Neuromuscular
1
Unit of Occupational
Rehabilitation and Ergonomics,
Fondazione Salvatore Maugeri,
Scientific Institute of Veruno,
Novara, Italy
2
Department of
Neurorehabilitation, ALS Centre,
Fondazione Salvatore Maugeri,
Scientific Institute of Milano,
Milano, Italy
3
Service of Bioengineering,
Fondazione Salvatore Maugeri,
Scientific Institute of Veruno,
Novara, Italy
4
Department of
Neurorehabilitation, ALS
Centre, Fondazione Salvatore
Maugeri, Scientific Institute of
Mistretta, Messina, Italy
5 factor analysis revealed three factors representing the
Department of Neuroscience,
ALS Centre, University of
Torino and Azienda
Ospedaliera Città della Salute e
della Scienza, Torino, Italy
Correspondence to
Professor A Chiò, Department
of Neuroscience, University of
Torino, ALS Centre, Via
Cherasco 15, Torino 10126,
Italy; achio@usa.net
Received 5 December 2012
Revised 8 February 2013
Accepted 28 February 2013
Published Online First
20 March 2013
To cite: Franchignoni F,
Mora G, Giordano A, et al. J
Neurol Neurosurg Psychiatry
2013;84:1340–1345.
1340
RESEARCH PAPER
Evidence of multidimensionality in the ALSFRS-R
Scale: a critical appraisal on its measurement
properties using Rasch analysis
Franco Franchignoni,1 Gabriele Mora,2 Andrea Giordano,3 Paolo Volanti,4
Adriano Chiò5
ABSTRACT
Objective To examine dimensionality, reliability and
validity of the Amyotrophic Lateral Sclerosis Functional
Rating Scale-revised (ALSFRS-R) using traditional classical
test theory methods and Rasch analysis in order to
provide a rationale for possible improvement of its metric
quality.
Methods Methodological research on ALSFRS-R
collected in a consecutive sample of 485 patients with
amyotrophic lateral sclerosis (ALS) attending three
tertiary ALS centres.
Results The ALSFRS-R items showed good internal
consistency but dimensionality analysis argues against
the use of ALSFRS-R as a single score because the scale
lacks unidimensionality. Parallel analysis and exploratory
following domains: (1) bulbar function; (2) fine and
gross motor function; and (3) respiratory function. Rasch
analysis showed that all items in each domain fitted the
respective constructs to measure, except for item No 9
‘climbing stairs’ and item No 12 ‘respiratory
insufficiency’. Rating categories did not comply with the
criteria for category functioning. Collapsing the scale’s
5 level ratings into 3 levels improved its metric quality.
Conclusions The ALSFRS-R fails to satisfy rigorous
measurement standards and should be, at least in part,
revised. At present, ALSFRS-R should be considered as a
profile of mean scores from three different domains
(bulbar, motor and respiratory functions) more than a
global total score. Further studies on ALSFRS-R using
modern psychometric methods are warranted to confirm
our findings and refine the metric quality of this scale,
through a step by step process.
INTRODUCTION
Amyotrophic lateral sclerosis (ALS) is a neurode-
generative disorder of unknown cause, charac-
terised by progressive impairment of motor
function due to degeneration of upper and lower
motor neurons. At present, the only approved
therapy for ALS is riluzole.1 2
The negative results of many recent clinical trials
in ALS have raised concern about their design.
Poor knowledge of the pathogenesis, unsatisfactory
animal models, weak trial designs and biases in
patient selection have been claimed as possible
causes.3 4 Among the criteria for a proper trial
design, a crucial issue is the choice of surrogate
markers as outcome measures.
Franchignoni F, et al. J Neurol Neurosurg Psychiatry 2013;84:1340–1345. doi:10.1136/jnnp-2012-304701
The Amyotrophic Lateral Sclerosis Functional
Rating Scale (ALSFRS)5 and its revised form
(ALSFRS-R)6 are the most widely used surrogate
markers of disease progression of ALS in clinical
practice and research. The ALSFRS-R showed a
strong correlation with disease progression and sur-
vival,7 8 and thus has been used as a primary or
secondary outcome measure of efficacy in several
therapeutic trials.1 2
To date, both ALSFRS and ALSFRS-R have been
analysed using only classical test theory (CTT) pro-
cedures, and both have demonstrated good internal
consistency, reproducibility and criterion related
validity.6 9 10 The CTT approach, as is known, does
not take into account some standard criteria and
attributes—concerning both single items and the
total score—that must be considered when evaluat-
ing the fundamental properties of a measurement
tool (eg, to place confidence in the total score uni-
dimensionality is required, otherwise outcomes
cannot be unambiguously interpreted).11 Rasch
analysis (RA) is being increasingly recommended in
the development and evaluation of clinical tools
for healthcare to verify if they comply with the
theoretical requirements of measurement, including
dimensionality analysis and item level scale
evaluation.12
The aim of our study was to test the internal val-
idity of the ALSFRS-R (mainly in terms of dimen-
sionality, rating scale functioning, and item
technical quality) using both CTT and RA methods.
METHODS
A sample of 485 subjects with a diagnosis of prob-
able or definite ALS according to the El Escorial
revised criteria,13 consecutively attending three ter-
tiary ALS centres, was evaluated with the
ALSFRS-R. The study was approved by the local
ethics committees.
ALSFRS-R is a simple, easy to administer, disease
specific scale consisting of 12 items assessing
bulbar, arm, leg and respiratory function. Its score
is usually based on a consensus between the patient
(or caregiver, if the patient cannot communicate
effectively) and clinician.14 The answer to each
item is rated according to 5 categories, from 0
(complete dependence) to 4 (normal function),
resulting in a total score ranging from 0 to 48. To
ensure reliable data acquisition, all evaluators
underwent extensive training.
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Neuromuscular

Statistical analysis
Table 1 Demographic and clinical characteristics of the patients
We combined CTT and RA approaches to investigate the follow-
(n=485)
ing psychometric properties of ALSFRS-R.
Characteristic

Internal consistency and dimensionality Age (years) (mean±SD) 61.4±11.5

The internal consistency of ALSFRS-R was assessed by means of Men/women (n) 249/236
Cronbach coefficient α and item to total correlation. Given the Time from symptom onset (months) (mean±SD) 28.4±25
unclear factorial structure of responses to the ALSFRS-R, an esti- Site of onset (n (%))
mate of the number of relevant factors was obtained with parallel Limb 362 (74.6)
analysis (PA).15 Subsequently, an exploratory factor analysis (EFA) Bulbar 118 (24.3)
for ordinal data16 with orthogonal (Varimax) and oblique Respiratory 5 (1.1)
(Promax) rotations on a randomly split half of the dataset ALSFRS-R (mean±SD) 26.5±10.3
(n=242) was used to study the contribution of each item to the Forced vital capacity (% predicted) (mean±SD) 73.6±27.3
factors identified by PA. A confirmatory factor analysis (CFA) on Percutaneous endoscopic gastrostomy (n (%)) 69 (14.2)
the second half of the dataset was used to verify the fit between Non-invasive ventilation (n (%)) 148 (30.5)
the data and the model. The following goodness of fit indexes Tracheostomy (n (%)) 9 (1.8)
were taken into account: Tucker–Lewis Fit Index (TLI), ALSFRS-R, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised.
Comparative Fit Index (CFI), root mean square error of approxi-
mation (RMSEA) and the standardised root mean square residual
(SRMR). For acceptable fit, TLI and CFI should be >0.95,
STATA V.10.1 (StataCorp LP, College Station, Texas, USA) was
RMSEA <0.80 and SRMR <0.10.17 As multidimensionality was
used to perform PA, Lisrel 8.80 (Scientific Software International
confirmed, we used the underlying factors and their relation to
Inc, Lincolnwood, Illinois, USA) for CFA and EFA, and
each item to break the scale down into subscales, the clinical mean-
WINSTEPS V.3.68.2 (Winsteps.com: Chicago; 2009) for RA.
ingfulness of which was judged by expert opinion. Then, each sub-
scale underwent RA.
RESULTS
Demographic and clinical characteristics of patients are shown
Rasch analysis
in table 1. Cronbach’s α was 0.88 for ALSFRS-R. Items showed
An introduction to RA and related concepts can be found in dedi-
an item to total correlation between 0.45 (item No 2 ‘saliva-
cated textbooks.18 Our analysis was performed on the entire
tion’) and 0.81 (item No 7 ‘turning in bed’). PA revealed three
dataset (n=485). We started with a diagnostic assessment of the
factors with empirical eigenvalues exceeding those from the
ALSFRS-R rating categories to investigate whether the response
random data. These three factors explained 50.4%, 18.1% and
levels to each item in the scale were being used effectively and con-
11% of the variance, reaching a cumulative 79.5%. As suggested
sistently.19 Based on this diagnostic evaluation and following stan-
by PA, we performed an EFA for a three factor solution to inves-
dardised procedures,19 we collapsed some adjacent categories and
tigate the contribution of each item to the scale. The results are
recoded response levels. After rating scale modifications, we per-
presented in table 2; orthogonal and oblique rotations produced
formed a second series of RA on the three subscales suggested by
very similar results, suggesting the adequacy of the orthogonal
the preliminary dimensionality analysis. The internal construct val-
solution. These results showed three factors that clearly repre-
idity of each subscale was assessed by evaluating the fit of individ-
sent the following domains: (1) bulbar function (item Nos 1–3);
ual items to the latent trait as per the Rasch model. Infit and outfit
(2) fine and gross motor function (item Nos 4–9); and (3)
mean square statistics for each item were calculated, considering
respiratory function (item Nos 10–12). A CFA on this three
values between 0.8 and 1.2 as an indicator of acceptable fit.18
factor model showed a good fit (TLI, CFI, RMSEA and SRMR
Subscale reliability was evaluated in terms of person separ-
ation reliability, an index similar to Cronbach’s α estimating
how well one can differentiate between different individuals’
performances on the measured variables: for the range 0–1, Table 2 Factor analysis for a three factor solution, as suggested
coefficients >0.70 are taken as evidence of sufficient reliability by parallel analysis
and coefficients >0.80 are considered good.18 Varimax rotated loadings
A principal component analysis (PCA) on the standardised
Item Factor 1 Factor 2 Factor 3
residuals was used to investigate the local independence of items
and the presence of subdimensions as an assessment of the uni- 1. Speech 0.92 0.16 0.12
dimensionality of the scale. The following criteria were used to 2. Salivation 0.87 0.18 0.12
confirm unidimensionality: (a) a cut-off of 50% of the variance 3. Swallowing 0.86 0.32 0.18
explained by the trait that the scale intended to measure (the 4. Handwriting 0.15 0.18 0.84
‘Rasch factor’); and (b) eigenvalue of the first residual factor 5. Cutting food and handling utensils 0.15 0.22 0.86
smaller than 3.20 6. Dressing and hygiene 0.11 0.28 0.91
In addition, we performed a differential item functioning 7. Turning in bed and adjusting bedclothes 0.17 0.23 0.92
(DIF) analysis on each subscale to examine the stability of item 8. Walking 0.10 0.29 0.78
hierarchy across the following subsamples: men versus women; 9. Climbing stairs 0.10 0.31 0.79
younger age (≤60 years) versus older age (>60 years); and 10. Dyspnoea 0.28 0.82 0.32
disease duration (≤2 years vs >2 years). DIF was investigated 11. Orthopnoea 0.31 0.74 0.39
using an item by item t test for difference in mean measures 12. Respiratory insufficiency 0.21 0.75 0.32
between the two subgroups (two sided, 1% α). Further technical
Bold type indicates loadings >0.4
aspects of our statistical analyses can be found elsewhere.21

Franchignoni F, et al. J Neurol Neurosurg Psychiatry 2013;84:1340–1345. doi:10.1136/jnnp-2012-304701 1341

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Neuromuscular

were 0.97, 0.98, 0.034 and 0.040, respectively), thus confirming
the multidimensionality of ALSFRS-R.
As for RA, rating scale diagnostics showed that response levels
of each item (score 0–4) did not comply with the pre-set criteria
for category functioning (average measures, thresholds, etc.).
Accordingly, the number of levels was revised, adopting a solu-
tion able to maximise both statistical performance and clinical
meaningfulness. For the first 11 items, the 5 original response
levels were reduced to 3, always collapsing level ‘0’ with ‘1’,
and level ‘2’ with ‘3’. For item No 12 ‘respiratory insufficiency’,
the best solution was obtained collapsing the three central
levels, thus obtaining the following three response options:
2=no respiratory insufficiency; 1 = use of BiPAP; 0 = invasive
mechanical ventilation. By way of example, a typical graphical
presentation of these results is shown in figure 1. In figure 1A,
the graph shows that the probability of using response levels ‘1’
and ‘2’ in that item is never modal (ie, higher than that of the
other levels). In figure 1B (after combining original level ‘0’
with ‘1’, and ‘2’ with ‘3’), the probability of selecting each of
the three revised response levels (score 0–2) is a clear function
of performance ( patient functional ability minus item difficulty)
shown on the x axis. The ‘thresholds’ correspond to the inter-
sections (ie, the probabilistic midpoint) between two adjacent
response curves. Whether the responses to the items are consist-
ent with the metric estimate of the underlying construct is indi-
cated by the ordered set of ‘thresholds’ for each item.
Applying this collapsing procedure, the RA showed that all
items included in each of the three subscales (bulbar function:
item Nos 1–3; motor function: item Nos 4–9; and respiratory
function: item Nos 10–12) fitted the respective constructs to
measure, except for item No 9 ‘climbing stairs’ (infit
Mnsq=1.64; outfit Mnsq=1.53) and item No 12 ‘respiratory
insufficiency’ (outfit Mnsq=1.56), which showed an unexpect-
edly high variability in the observed data compared with the
Rasch model prediction.
The other main results of RA for each subscale are shown in
figure 2 and table 3; distribution of subject functional ability
and item difficulty, reliability indices and results regarding the
three PCAs of the standardised residuals (analysing the variance
explained by the Rasch factor and the first residual factor). The

Figure 1 Rating scale category

probability curves of the item ‘speech’
for (A) the original five response levels
(score 0–4) and (B) the revised levels
obtained collapsing scoring option ‘0’
(‘loss of useful speech’) with ‘1’
(‘speech combined with non-vocal
communication’), and ‘2’ (‘intelligible
with repeating’) with‘3’ (‘detectable
speech disturbance’) (recoding 00112).
The y axis represents the probability
(0–1) of responding to one of the
response levels and the x axis
represents the different performance
values ( patient functional ability minus
item difficulty) in logits (negative
values=less functional ability). Vertical
arrows indicate the location of the
respective thresholds. The ideal plot
should look—as in (B)—like an
ordered even succession of hills, with
an ‘emerging’ crest where each
category is modal over a certain range.

1342 Franchignoni F, et al. J Neurol Neurosurg Psychiatry 2013;84:1340–1345. doi:10.1136/jnnp-2012-304701

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Neuromuscular

Figure 2 Person ability and item

difficulty maps of the three ALSFRS-R
subscales. The central line represents
the common logit metric (Rasch
measure). Frequency distribution of
person functional ability, and average
item location are shown in each upper
and lower panel, respectively.
Functional ability and item difficulty
increase from left to right. Misfitting
items are in bold. By convention, the
average difficulty of items is set at 0
logits. ALSFRS-R, Amyotrophic Lateral
Sclerosis Functional Rating Scale-
revised.

three subscales demonstrated different levels of sample item logits in each subscale, whereas item difficulty span was more
matching (the best was for bulbar function, the worst for limited, ranging from 1.89 logits (bulbar function) to 3.84 logits
respiratory function). Subject ability span was more than 10 (respiratory function). Item separation reliability was high in the

Table 3 Subject functional ability and item difficulty levels, reliability indices and principal component analysis of the standardised residuals for
each of the three ALSFRS-R subscales
Subscale 1 (item 1–3) Subscale 2 (item 4–9) Subscale 3 (item 10–12)

Average subject functional ability levels (range) 0.39 (−5.91 to 5.91) −0.74 (−5.50 to 5.60) 0.90 (−6.17 to 6.08)
Item difficulty levels (range) −1.09 to 0.80 −1.24 to 1.80 −2.03 to 1.81
Person separation reliability 0.78 0.82 0.69
Cronbach’s α 0.88 0.91 0.82
Variance explained by the Rasch factor (%) 55.4 59.2 60.6
Eigenvalue of the first residual factor 1.6 2.4 1.5
ALSFRS-R, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised.

Franchignoni F, et al. J Neurol Neurosurg Psychiatry 2013;84:1340–1345. doi:10.1136/jnnp-2012-304701 1343

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Neuromuscular
three subscales, while the person separation reliability was suffi-
cient or good in the bulbar and motor subscales, and borderline
(0.69) in the respiratory subscale. No PCA of the standardised
residuals presented residual correlations >0.30, thus confirming
the local independence of the items in each subscale. DIF ana-
lysis showed no difference in responses due to gender, age or
disease duration.
DISCUSSION
In clinical trials, we measure constructs (ie, ‘latent’ variables,
such as functioning), perform statistical tests on the scales’ raw
scores and draw conclusions. The appropriateness of these con-
clusions strongly depends on the metric quality of the selected
measures, and it has a crucial influence on patient care, drug
efficacy and health policies.
Unambiguous interpretation requires that a score represent a
single attribute (dimension). Otherwise, one could not be sure if
two individuals with the same score are, in fact, comparable.
This problem hampers understanding of clinical trial outcomes,
which in turn has consequences for selecting interventions for
individual patients.22
Our main finding is that the ALSFRS-R presents a series of
drawbacks that corrupt its metric quality.
The ALSFRS-R items showed good internal consistency accord-
ing to CTT, with a Cronbach’s α even higher than in the original
paper,4 but our dimensionality analysis argues against the validity
of summing the ALSFRS-R items into a single score. Our data
clearly indicate the presence of three different domains (bulbar,
motor and respiratory function): in each domain an aspect of func-
tional status can be independently assessed but domain scores
cannot be simply summed to obtain an overall functional status
measure in ALS. These three functions are clinically meaningful
and correctly represent the underlying structure of the domains
investigated by ALSFRS-R.4 23 24
RA showed that some rating categories of ALSFRS-R did not
comply with the set criteria for category functioning. This may
be due to rater difficulty in discerning among the five levels of
functional ability. As an example, in item No 7 (‘turning in bed
and adjusting bed clothes’) the wording of categories ‘0’ (help-
less) and ‘1’(can initiate but not turn or adjust sheets alone)
does not allow a clearly distinct ranking of functional ability
and could introduce error variance rather than metric informa-
tion into the ratings. The same can be said, in the same item,
for the categories ‘2’ (can turn alone or adjust sheets, but with
great difficulty) and ‘3’ (somewhat slow and clumsy, but no help
needed).
Collapsing the scoring options into a 3 level rating for all
items improved the measurement quality of the scale, providing
a simpler and more distinct idea of the level of functioning
represented by each rating level, without loss of measurement
information.18 These findings show that there is space for a
refinement of ALSFRS-R by item rewording and/or reduction of
option number.
After collapsing the categories, fit statistics showed two
misfits: item Nos 9 and 12. The misfit of item 9 (‘climbing
stairs’) is in line with the clinical observation that different
environmental factors (ie, home architecture) and personal atti-
tudes can produce high variability in this response, unexpected
by the Rasch model. The high outfit value of item 12 ‘respira-
tory insufficiency’ is due to the presence of subjects without dys-
pnoea and orthopnoea (less difficult items) but on permanent
ventilation (most difficult item); although the finding is clinically
understandable, it demonstrates an additional serious bias of the
scale.
1344 Franchignoni F, et al. J Neurol Neurosurg Psychiatry 2013;84:1340–1345. doi:10.1136/jnnp-2012-304701
Thus our results suggest a rethinking of item ‘climbing stairs’
and a clarification of the conceptual framework and measure-
ment strategy of the whole subscale ‘respiratory function’,
including the provision of detailed guidelines for its compil-
ation. More generally, there is a lack of standardised method-
ology for ALSFRS-R administration25 and no formal interview
instructions.
Concern about the metric properties of ALSFRS-R has
already been expressed.1 Our findings confirm what clinicians
know: the interpretation of a total raw score of ALSFRS-R is
hampered by ambiguities due to its different metric meanings
for the different ALS forms. This problem is likely to be compli-
cated by the presence in ALSFRS-R of a typical phenomenon of
ordinal summed rating scales; the relationship between total raw
scores and linear Rasch transformed measures of global function
is not linear but ogival.26 As patients approach the bottom of
the scale, each raw score point represents an increasing metric
distance, yet it appears that patients are ‘slowing down’ in their
worsening because it becomes increasingly difficult for them to
lose further raw score points. This finding would imply a
reduced sensitivity of the raw scores to a change occurring in
high and low functioning ALS subjects,27 28 and on the other
hand it underlines the complex relationship between progression
of disease and modification of ALSFRS-R raw scores.1 29
Care should be taken in interpreting our data. First, our non-
probability sample might compromise the study’s external valid-
ity. Nevertheless, our sample was a large cross section of
patients with a broad spectrum of disease severity, drawn from
three different tertiary ALS clinics. Second, we cannot exclude
the fact that some specific (linguistic, cultural or technical) char-
acteristics of the Italian version of the ALSFRS-R could have
somewhat influenced our results, although our version was
checked using a thorough procedure of ‘forward/backward
translation’ followed by pilot testing and expert revision,
without any semantic difficulties being found.
Our findings suggest that ALSFRS-R fails to satisfy rigorous
measurement standards and should be, at least in part, revised.
Valid inferences on the efficacy of treatment trials require high
quality outcome measures. At present, we believe that
ALSFRS-R should be considered as a profile of mean scores
from three different domains (bulbar, motor and respiratory
functions) more than a global total score. Further studies on
ALSFRS-R using modern psychometric methods are warranted
to confirm our findings and refine the metric quality of this
scale, through a step by step process.
Acknowledgements We thank the patients and their families for their
collaboration in this study.
Contributors Study concept and design: FF, GM, AG, PV and AC. Acquisition of
the data: GM, PV and AC. Analysis and interpretation of the data: FF, GM, AG, PV
and AC. Drafting of the manuscript: FF, GM, AG, PV and AC. Critical revision of the
manuscript for important intellectual content: FF, GM, PV and AC. Obtained
funding: GM and AC. Administrative, technical and material support: FF, GM, AG,
PV and AC. Study supervision: FF, GM, PV and AC. AC had full access to all of the
data in the study and takes responsibility for the integrity of the data and the
accuracy of the data analysis. All authors have approved the submitted version of
the paper.
Funding This work was funded in part by Ministero della Salute (Ricerca Sanitaria
Finalizzata, RF-MAU-2007-643050) and Centro Nazionale per la Prevenzione e il
Controllo delle Malattie (grant 31, 2009). The research leading to these results has
received funding from the European Community’s Health Seventh Framework
Programme (FP7/2007–2013) (grant agreements Nos 259867 and 278611).
Competing interests FF has received research support from the Italian Ministry of
Health (Ricerca Finalizzata). GM has received research support from the Italian
Ministry of Health (Ricerca Finalizzata). AC serves on the editorial advisory board of
Amyotrophic Lateral Sclerosis and has received research support from the Italian
 Downloaded from jnnp.bmj.com on February 1, 2014 - Published by group.bmj.com
Ministry of Health (Ricerca Finalizzata), Regione Piemonte (Ricerca Finalizzata),
University of Torino, Federazione Italiana Giuoco Calcio, Fondazione Vialli e Mauro
onlus and European Commission (Health Seventh Framework Programme); he serves 14
on a scientific advisory board for Biogen Idec and Cytokinetics.
Ethics approval The study was approved by the local ethics committees of the
three clinical centres involved. 15
Provenance and peer review Not commissioned; externally peer reviewed. 16
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Evidence of multidimensionality in the

ALSFRS-R Scale: a critical appraisal on its
measurement properties using Rasch
analysis
Franco Franchignoni, Gabriele Mora, Andrea Giordano, et al.

J Neurol Neurosurg Psychiatry 2013 84: 1340-1345 originally published

online March 20, 2013
doi: 10.1136/jnnp-2012-304701

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