Journal of the Neurological Sciences 180 (2000) 8693

www.elsevier.com / locate / jns

Development of a patient-specific dyspnoea questionnaire in motor neurone

disease (MND): the MND dyspnoea rating scale (MDRS)
a
b
b
a,
C.F. Dougan , Claire O. Connell , E. Thornton , C.A. Young *
a

Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley, Liverpool L9 7 LJ, UK
b
Department of Psychology, University of Liverpool, Liverpool, UK

Abstract
Motor neurone disease (MND) is a progressive, unremitting and fatal disease. Respiratory dysfunction is common and a significant
cause of morbidity. The relationship between subjective dyspnoea and objective measures of lung function have been unexplored in
MND. Increasing interest in the specific treatment of respiratory symptoms in MND has highlighted the need for simple, reliable and valid
measures to quantify the degree of dyspnoea in this condition. Several generic questionnaires have been developed to rate subjective
breathlessness but are inappropriate for use in MND patients as they often assess dyspnoea by exercise-limitation. As yet, there are no
published disease-specific measures to assess dyspnoea in MND. In order to accurately and reproducibly measure the subjective
experience of dyspnoea in this patient group, we have developed and validated a novel patient-specific dyspnoea questionnaire, the MND
dyspnoea rating scale (MDRS). It comprises three domains covering dyspnoea, emotion and mastery and is valid for use in MND patients
at all stages of disease progression. In our cohort of 40 unselected patients with MND we have shown that the patients subjective
experience of dyspnoea is closely related to emotion and psychological control over the disease. Dyspnoea is not related to objective
measures of lung function such as vital capacity, irrespective of limb or bulbar presentation. In conclusion, vital capacity, although useful
prognostically, is only one aspect of respiratory function in MND. The MDRS is a reliable and valid tool to rate subjective dyspnoea in
MND. 2000 Elsevier Science B.V. All rights reserved.
Keywords: Motor neurone disease; Amyotrophic lateral sclerosis; Dyspnoea; Questionnaire; Vital capacity

1. Introduction
Dyspnoea, the unpleasant sensation of breathlessness, is
a distressing symptom which may adversely affect quality
of life of people with motor neurone disease (MND). The
mechanism by which the individual experiences breathlessness is complex with integration of both physical and
psychological components. Despite the frequent involvement of the respiratory system in MND [1,2] the
relationship between dyspnoea, physiological measures
and functional state remain unexplored.
Dyspnoea is significant in contributing to poor quality of
life in other disease states and several questionnaires have

*Corresponding author.
E-mail address: young-c@wcnn.co.uk (C.A. Young).

been developed to assess the degree of dyspnoea [35].

However, existing measures such as the Medical Research
Council (MRC) dyspnoea scale [5] are inappropriate for
MND as the dyspnoea is graded on exercise tolerance
which cannot be applied to a patient with exercise-limiting
limb weakness. There are no published disease-specific
measures for dyspnoea in MND.
The primary aim of this study was to develop a
dyspnoea questionnaire for MND patients which would be
easy to easy to administer and applicable to patients at all
stages of disease progression and all levels of disability.
Psychometric properties of the questionnaire were tested to
establish validity, internal consistency and reliability.
Secondary outcomes were to examine the relationship
between subjective experience of dyspnoea and a more
objective measure of lung function and to investigate the
relationships between dyspnoea, fatigue, emotion and
mastery.

0022-510X / 00 / $ see front matter 2000 Elsevier Science B.V. All rights reserved.
PII: S0022-510X( 00 )00415-9

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

2. Methods

2.1. Development of the MND dyspnoea rating scale

( MDRS)
After review of the literature, we modelled the dyspnoea
domain of our questionnaire on the chronic respiratory
disease questionnaire (CRDQ) by Guyatt et al. [3]. The
dyspnoea domain is generated by the patient identifying
five activities of daily living which may make them feel
breathless. The patient then rates the degree of dyspnoea
on a five-point Likert scale which quantifies the severity.
In this way the patient can personalise the questionnaire
and it can be applied to all patients irrespective of their
physical limitations.
A pilot study was conducted and included open interview with patients, their carers and MND experts to
generate the remaining items important to this group of
individuals. The questionnaire was extensively modified
and reworded for clarity. Reflecting the importance of both
the physical and psychological contributors to dyspnoea,
the remainder of the questionnaire examined emotion,
fatigue and mastery.
The final questionnaire that emerged consisted of 16
questions each rated on a five-point likert scale 04, the
MDRS (see Appendix A). Low scores indicated minimal
symptoms; high scores indicated maximal symptoms. The
questionnaire was worded so it could be self-administered
or answered with the assistance of a carer or health
professional and requires approximately 10 min to administer.
To examine the testretest reliability of the MDRS, 10
stable patients completed the MDRS and had their vital
capacity measured 7 days after the initial data collection by
the same researcher.

2.2. Recruitment of patients and data collected

2.2.1. Patient group
Patients were recruited from the Walton Centre MND
database, and clinics at three teaching hospitals in the
NorthWest of England. All patients had a diagnosis of
MND confirmed by their consultant neurologist. After
consultation with the patients general practitioner, patients
in the community were approached initially by explanatory
letter or at their usual clinic visit.
Ethical approval was obtained for each centre and
informed consent from each participant. Data was collected in specialist clinics or at home in those patients too
unwell to visit hospital.
2.2.2. Demographics
Data collected included age, duration of disease as
defined by date of onset of first symptom, mode of onset
and presence of bulbar symptoms at the time of study.

87

2.2.3. Lung function

Vital capacity was used to measure lung function as it is
the only test of lung function that has been shown to have
prognostic value in MND [2,6]. Sitting slow vital capacity
was measured using a hand held spirometer with a
modified mouthpiece for those patients with orofacial
weakness. The best of three attempts was recorded. Height
was measured and used to predict expected vital capacity
using standardised tables [7].
2.2.4. Subjective measures
To test the validity of the MDRS compared to existing
dyspnoea scales, each patient completed two further scales;
the Borg scale and a visual analogue scale. Borg [4]
developed an interval scale which has been used widely in
clinical and research practice to measure dyspnoea in
patients with chronic lung disease. The Borg scale grades
the severity of dyspnoea between one and 10 with written
cues beside each number. The visual analogue scale [8,9]
is a widely used psychometric measure which is reliable
and responsive [10] and has been used to measure breathlessness both in normal controls and patients with lung
disease. The patient simply marks on a horizontal line their
symptom severity between two extremes of no breathlessness and worst breathlessness ever. The point at which
respondents mark represents their perception of breathlessness on this continuum.
2.2.5. Functional status
The ALSFRS [11,12] was administered to assess the
level of functional impairment. It is a valid, reliable and
sensitive disease-specific measure comprising 10 questions
enquiring about how swallowing, breathing, and limb
function affects activities of daily living. It as an ordered
categorical scale with each question graded between 0 and
4. High score indicate good function; low scores indicate
poor function.
2.2.6. Anxiety and depression
The Hospital Anxiety and Depression scale [13] was
completed. It is a screening questionnaire for assessing the
presence or absence of anxiety and depression. The scale
consists of 14 items divided equally into depression and
anxiety subscales. Ratings by patients are made on fourpoint scales which represent degree of symptoms. Items
are summed; high scores indicate presence of clinically
significant anxiety or depression.
2.3. Statistical analysis
SPSS for Windows version 9.0 was used for statistical
analysis [14]. Non-parametric tests were used as the data
did not conform to a normal distribution.
Principal components factor analysis [15] was performed to examine the underlying structure of the MDRS.
This is a statistical model rearranges a large set of

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

88

variables (or items) into smaller groups (or factors) of

related items.
Internal consistency was examined using Cronbachs a.
Testretest reliability was assessed by comparing the
average and mean difference in paired scores [16,17].
Relationships between variables were explored using
Spearmans correlation co-efficient. MannWhitney U-test
was used to explore differences between limb and bulbar
patients.

3. Results

3.1. Descriptives of the MND patients (see Table 1)

Forty patients were recruited (25 male, 15 female).
Median age of onset of MND was 60 years (range, 3480)
with the median duration of the disease being 31 months
(range, 799). The median percent predicted vital capacity
was 56.9 (range, 496%). There was no difference between males and females for any variable so they were
treated as a group. This was a representative group of all
stages of MND with a wide range of functional abilities
demonstrated by the ALSFRS (median, 24 of 40; range,
537).

3.2. The MDRS evaluation

3.2.1. Validity by factor analysis ( Table 2)
Three factors (domains) were extracted to explain a total
of 68% of the variance. The factor loading or correlation of
each item on the three extracted factors enabled us to
define which items belonged to each domain. The three
domains were best described as emotion, dyspnoea and
mastery. Fatigue had only a weak factor loading onto the
emotion domain and did not form a separate dimension.
The final questionnaire that emerged was named the
MDRS and is shown in Appendix A with scoring.
Table 1
Descriptives and baseline measures
Measure

Number of
patients

Median

Range

Age (years)

40

60

3480

Gender
Male
Female

25
15

Type of onset
Limb
Bulbar

28
12

Bulbar symptoms
at time of study
Duration (months)
Percent predicted vital
capacity
ALSFRS

Table 2
Rotated factor loadings
Item number a

Factor 1
emotion

Factor 2
dyspnoea

Factor 3
mastery

14, satisfied
11, relaxed
13, depressed
9, upset, worried
6, frustration
8, confident
16, tense
12, fatigue
2, dyspnoea
3, dyspnoea
4, dyspnoea
1, dyspnoea
5, dyspnoea
15, fear
7, panic
10, control

0.89
0.78
0.77
0.72
0.67
0.60
0.58
0.45
0.24
0.00
0.26
0.15
0.25
0.27
0.31
0.33

0.00
0.19
0.32
0.24
0.35
0.22
0.34
0.41
0.81
0.67
0.67
0.66
0.60
0.43
0.37
0.27

0.22
0.41
0.39
0.22
0.21
0.52
0.44
20.21
0.18
0.30
0.23
0.23
0.34
0.80
0.77
0.67

a
The word next to the item number is a brief cue. The full question
corresponding to each item number is given in full in Appendix A. Bold
type indicates the strongest factor loadings for each domain.

3.2.2. Internal consistency

Cronbachs a was 0.84 showing a high degree of
internal consistency. This increased to 0.93 when the
dyspnoea domain was extracted, suggesting that the
dyspnoea domain is measuring a separate sensation to the
remaining items.
3.2.3. Testretest reliability (see Fig. 1 a,b)
There was no significant change in respiratory symptoms or vital capacity over 7 days confirming the stability
of this group. The average difference in the total MDRS
score over this time was 21 (S.D. 5.9) confirming a high
degree of stability. The dyspnoea domain scores were more
variable but still reliable with an average difference of 3.22
(S.D. 3.01) between the two scores.
3.2.4. Validity
Validity of the dyspnoea domain was confirmed by
strong correlation with both measures of subjective breathlessness. There was a highly significant correlation with
both the Borg scale (Spearman correlation co-efficient,
r50.66, P50.0001) and the visual analogue scale (r5
0.72, P50.0001).
3.3. Relationships between dyspnoea and other
psychological measures

24
40
40

34
57

799
496

40

24

537

3.3.1. Subject dyspnoea rating versus lung function

There was no correlation between percent predicted vital
capacity and any of the three measures of subjective
dyspnoea although vital capacity did correlate weakly with
the total ALSFRS (r50.41, P50.01) and the bulbar
domain of the ALSFRS (r50.36, P50.03).

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

89

Fig. 1. (a) Testretest reliability: difference in MDRS scores over 7 days plotted against their average. (b) Reliability: difference between MDRS dyspnoea
domain scores plotted against their average.

3.3.2. Dyspnoea rating versus anxiety and depression

The dyspnoea domain was significantly correlated with
the hospital anxiety and depression scale (r50.41, P5
0.01). Looking at anxiety and depression separately,
anxiety was highly significantly correlated with the
dyspnoea domain (r50.42, P50.009); in contrast depression was not correlated with the dyspnoea domain (r5
0.27, P50.19). The Borg and VAS scales were also
significantly associated with anxiety although the relation-

ships were weaker (Borg: r50.33, P50.05; VAS: r50.37,

P50.03).

3.3.3. Dyspnoea rating versus fatigue, emotion and

mastery
Subjective dyspnoea rated by the MDRS dyspnoea
domain was highly significantly associated with emotion
(r50.47, P50.003) and mastery (r50.5, P50.002) but

90

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

was not correlated with fatigue question of the emotion

domain (r50.28, P50.09).

3.3.4. Dyspnoea rating versus functional status

There was no relationship between subjective dyspnoea
and functional status (r520.22, P50.18).
3.4. Comparison of limb and bulbar groups
Patients were grouped into limb or bulbar onset by their
first symptom. There was a significant difference in the
duration of illness between the two groups with limb
patients having a median duration of 20 months and bulbar
patients a shorter duration of 13 months (MannWhitney
U-test, two-tailed significance, P50.04). When corrected
for differences in duration of MND, the vital capacity was
significantly lower in the bulbar group compared to the
limb group (median vital capacity, 42 and 63% predicted,
respectively, P50.03). Interestingly, despite this marked
difference in vital capacity in each group, there was no
significant difference between the two groups with respect
to their perception of breathlessness.

4. Discussion
This study shows that dyspnoea is an important and
common symptom in MND patients regardless of whether
they have bulbar involvement or not. We have developed a
novel disease-specific questionnaire which appears to be a
valid, reliable measure to quantify dyspnoea in MND. The
perception of breathlessness in MND is not related to
disease duration, level of disability or vital capacity. This
study also demonstrates that mastery of breathlessness and
anxiety are important psychological components to the
patients perception of dyspnoea.
Previous measures to quantify breathlessness have been
developed for use in lung and cardiac disease [35] but
these often rate breathlessness according to exercise
capacity which is clearly not applicable in many MND
patients. Guyatt et al. [3] developed the chronic respiratory
disease questionnaire to assess the impact of dyspnoea on
the quality of life. The dimensions of this questionnaire
were dyspnoea, fatigue, emotion and mastery. It appears
that fatigue is not so important to MND patients as patients
with chronic lung disease. This may be because MND
patients are more disabled physically and are likely to have
reduced levels of activity compared to patients with pure
lung disease.
The Borg [4] and visual analogue scales [8,9] are global
rating scales and only give limited information about the
factors contributing to breathlessness. In contrast, the
MDRS dyspnoea domain is patient-specific and allows the
patient to quantify how dyspnoea affects their daily life.

Dyspnoea is a complex sensation resulting from the

integration of physical and psychological input under
higher cortical control [18] so it is not surprising that this
study has demonstrated no association between awareness
of breathlessness and objective measures of vital capacity
or fatigue or physical functioning. This lack of association
between dyspnoea ratings and pulmonary function has
been found by several studies in lung disease [1921] and
in MND [22]. The BDNF study group found no strong
association between the respiratory subscale of the
ALSFR-R and percent predicted forced vital capacity
(Spearmans correlation co-efficient 0.33).
This supports the assertion that subjective reports of
dyspnoea are not just a reflection of lung function but a
result of the integration of numerous neuroanatomical
pathways. These include input from peripheral sensory
receptors (mechanoreceptors, irritant, stretch and C-fibre
receptors), chemoreceptors, effort, work load, psychological input and higher cortical control [18].
Recognition of the multifactorial nature of breathlessness has important implications clinically in that it emphasises that assessment of respiratory system by vital capacity alone is inadequate and may overlook more sensitive
markers of respiratory function.
The finding that there is no difference in perception of
dyspnoea between limb and bulbar groups is initially
surprising as intuitively one would expect bulbar patients
to perceive more breathlessness due to dys-coordination of
bulbar muscles which is important to breathing during
speech and eating. Again this reflects the complex pathways involved in the perception of dyspnoea.
Fallat et al. [2] observed that reduction in vital capacity
to as low as 50% in patients with neuromuscular disease
was commonly overlooked by clinicians so there is a clear
need for early markers of respiratory system involvement.
The development of breathlessness is an important phase
in the natural history of MND. Specific treatments of
breathlessness by have been explored in lung disease
[2325] and are being developed to manage respiratory
failure in MND [26,27]. However, studies on simple
treatment of this potentially distressing symptom are
lacking in MND. Future research will establish the ability
of the MDRS to demonstrate responsiveness to change and
its role as an outcome measure evaluating response to
specific treatment of dyspnoea in this condition.

Acknowledgements
We are indebted to Mary O. Brien and Professor D.
Mitchell, Royal Preston Hospital and Dr. Schady, Manchester Royal Infirmary for helping with data collection.
Sister Dott Marshall and Dave Watling, Walton Centre for
Neurology and Neurosurgery who assisted with clinics and
vital capacity measurement.

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

Appendix A
A.1. The MND dyspnoea rating scale ( MDRS)
We would be grateful if you could take time to complete
this questionnaire. Please attempt to answer all of the
questions. We are interested in your answers even if you do
not have a problem with breathlessness.
If you have any problems with completing the questionnaire we can help you complete it at the clinic visit.
(A) I would like you to think of the activities that you
have done during the last 2 weeks that may have made you
feel short of breath. These should be activities which you
do frequently and which are important in your day to day
life. Please list as many activities as you can that you have
done during the last 2 weeks that have made you feel short
of breath.

chosen to be the most important to rate the amount of

breathlessness, if any, that you have experienced.
I would now like you to describe how much shortness of
breath you have experienced during the last 2 weeks while
doing the five most important activities you have selected.
Please circle one number from 0 to 4 which best describes
the degree of breathlessness during the five selected
activities:

(1) Activity 1: . . .
Extremely
short
of breath

Very short
of
breath

Moderate
shortness
of breath
2

Slight shortness
of breath
1

Not at all
short
of breath
0

(2) Activity 2: . . .
Extremely
short
of breath

1.
2.
3.
4.
5.

91

Very short
of
breath

Moderate
shortness
of breath
2

Slight shortness
of breath
1

Not at all
short
of breath
0

(3) Activity 3: . . .

(B) Below is a checklist of activities which make some

people feel short of breath. Please read through the list and
circle any activities that may have made you short of
breath during the past two weeks. The activities are:
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.

Talking
Eating
Dressing
Preparing meals
Carrying such as carrying groceries
Playing with children / grandchildren
Walking
Moving around your own home (aided or unaided)
Having a bath or shower (aided or unaided)
Bending
Being angry or upset
Lying flat
While trying to sleep

(C) From your answers to questions A and B, please

choose the five most important to you in your day to day
life and list below.
1.
2.
3.
4.
5.

Extremely
short
of breath

Very short
of
breath

Moderate
shortness
of breath
2

Slight shortness
of breath
1

Not at all
short
of breath
0

(4) Activity 4: . . .
Extremely
short
of breath

Very short
of
breath

Moderate
shortness
of breath
2

Slight shortness
of breath
1

Not at all
short
of breath
0

(5) Activity 5: . . .
Extremely
short
of breath
4

Very short
of
breath
3

Moderate
shortness
of breath
2

Slight shortness
of breath
1

Not at all
short
of breath
0

(6) In general, how much of the time during the last 2 weeks have you
felt frustrated or impatient?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(7) How often during the past 2 weeks did you have a feeling of fear or
panic when you had difficulty getting your breath?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(8) In the last 2 weeks, how much of the time did you feel very confident
and sure that you could deal with your illness?
All of the
time

The next section uses the five activities which you have

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

C.F. Dougan et al. / Journal of the Neurological Sciences 180 (2000) 86 93

92

(9) In general, how much of the time did you feel upset, worried, or
depressed during the last 2 weeks?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(10) How often during the last 2 weeks did you feel you had complete
control of your breathing problems with shortness of breath and tiredness?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(11) How much of the time during the last 2 weeks did you feel relaxed
and free of tension?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(12) How often during the last 2 weeks have you felt low in energy?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(13) In general, how often during the last 2 weeks have you felt
discouraged or down in the dumps?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(14) How happy, satisfied, or pleased have you been with your personal
life during the last 2 weeks?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(15) How often during the last 2 weeks did you feel upset or scared when
you had difficulty getting your breath?
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

(16) In general, how often during the last 2 weeks have you felt restless,
tense or uptight:
All of the
time
4

Most of the
time

Some of the
time

Hardly any
of the
time

None of the
time

Scoring of the MDRS

MDRS-dyspnoea. Questions 15 quantify dyspnoea. Low
scores indicate less breathlessness, high scores indicate
more breathlessness. Possible range 020.
MDRS-emotion. Questions 6, 8, 9, 11, 12, 13, 14 and 16
assess emotional function. Low score indicates low per-

ceived emotional disturbance, high score indicates high

perceived detrimental emotional disturbance. Possible
scores range from 0 to 32.
MDRS-mastery. Questions 7, 10, and 15 assess mastery /
control over the disease. Low score indicates low perceived detrimental affect on mastery and control over the
disease, high score indicates high perceived detrimental
affect over mastery / control over the disease. Possible
scores range from 0 to 12.
Total MDRS score 064.

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