European Journal of Paediatric Neurology (2003) 7, 155–159

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ORIGINAL ARTICLE

The Hammersmith Functional Motor Scale for

Children with Spinal Muscular Atrophy: a Scale
to Test Ability and Monitor Progress in Children
with Limited Ambulation
Marion Maina, Harvey Kairona, Eugenio Mercurib,c, Francesco Muntonib,*

a
Department of Physiotherapy, Hammersmith Hospital, Du Cane Road, London W12 OHS, UK
b
Department of Paediatrics, Dubowitz Neuromuscular centre, Hammersmith Hospital, Imperial College
Du Cane Road, London W12 OHS, UK
c
Department of Paediatric Neurology, Catholic University, 02100 Rome, Italy

Received 17 April 2002; received in revised form 6 August 2002; accepted 16 April 2003

KEYWORDS
Functional motor scale;
Spinal muscular atrophy;
Non-ambulant patients
Summary A functional motor scale was devised for use in children with spinal muscular
atrophy type 2 and type 3, in particular those with limited mobility, to give objective
information on motor ability and clinical progression. The scale, which has 20 scored
activities, was designed to be self-explanatory, quick, easy to use, reproducible and
reliable. In this paper we describe the development of the scale, reporting the criteria
used to choose the items to be included, their application in a normal cohort and in a
cohort of children with SMA and how we arrived to a final version of the scale in which
the items are arranged in order of difficulty.
The analysis of 120 assessments over 2 years from 51 children with type 2 and type 3
SMA also led to a more accurate profile of functional achievements in both type 2 and 3
SMA and to a more detailed sub-classification of type 2 SMA.
Q 2003 European Paediatric Neurology Society. Published by Elsevier Science Ltd. All
rights reserved.

Introduction most common neuromuscular disease of childhood

SMA is a neuromuscular condition due to the
degeneration of the anterior horn cells, inherited
in an autosomal recessive way.1 It is the second
*Correspondence. Tel.: þ 44-181-383-3295; fax: þ44-208-740-
8281.
E-mail address: f.muntoni@imperial.ac.uk
affecting approximately 1:10,000 – 12,000 children.
There is a wide range of severity, the most severe
form being Werdnig –Hoffman disease (or SMA type
1), in which affected children are unable to sit
unsupported. The mildest form is Kugelberg –
Welander disease (SMA type 3) in which indepen-
dent ambulation is achieved but mild to moderate
proximal muscle weakness may be experienced,

1090-3798/03/$ - see front matter Q 2003 European Paediatric Neurology Society. Published by Elsevier Science Ltd. All rights
reserved.
doi:10.1016/S1090-3798(03)00060-6
156
causing difficulty with stairs and rising from the
floor. Children with the intermediate form
(SMA type 2) are, in contrast, able to sit but not
to walk independently.2,3
Prognosis is dependent on severity and almost
invariably children with Werdnig– Hoffman disease
will die of respiratory complications by the age of 2
years. Long term survival is expected for children
with the type 2 and type 3 and currently available
medical treatment is directed at secondary com-
plications such as respiratory problems or manage-
ment of deformities.
Although the weakness itself does not progress in
SMA, several studies have documented a deterio-
ration in functional abilities due to increased body
size without an increase in power to cope with
these extra demands. As a result a significant
proportion of children with SMA type 3 can lose
the ability to walk independently.4,5
There are numerous functional motor scales that
can be used for patients with neuromuscular
disorders, but these cannot always be used in non-
ambulant children or in those with limited
ambulation.
In our department, functional motor scoring was
based almost exclusively on the scale developed by
Scott, Hyde et al published in 19826 devised for
boys with Duchenne Muscular Dystrophy. The scale
helps to monitor disease progression and gives an
indication of when boys with Duchenne Muscular
Dystrophy are about to lose independent ambu-
lation. The scale is also useful when assessing
children with other neuromuscular disorders who
have deteriorating ambulation but it is not a
reliable indicator of change in ability for many of
the weaker non-ambulant children. There are also
other scales, such as the Vignos scale7 which
determines levels of mobility and others assessing
ability in function and ‘activities of daily liv-
ing’,8 – 10 but although they all undoubtedly have
their uses, none has been shown to be sufficiently
sensitive when assessing functional change in
weaker, non-ambulant children. In contrast, a
new scale has been recently proposed for assessing
function in patients with neuromuscular disorders
with more severe functional impairment but this
was designed to be used in non-ambulatory
patients only.11
The purpose of our study was to develop a useful
and reliable scale, and to use it in clinical practice.
The objectives used when developing the scale
were that it should be easy to use and of rapid
execution (for the therapist and the child), suitable
for very young children but relevant to adolescents
and if necessary, adults, self-explanatory, repro-
ducible and reliable.
M. Main et al.
The aims of the scale were to:
(i) Evaluate and illustrate the motor ability of
children with SMA with limited ambulation;
(ii) monitor the progression of function;
(iii) provide a tool for an accurate classification of
SMA and in particular to allow a graded scale
that takes into account the significant clinical
variability of children with this disorder.
In this paper we will describe the development of
the scale, reporting the criteria used to choose the
items to be included, their application in a normal
cohort and in a cohort of children with SMA and how
we arrived to a final version of the scale in which
the items are arranged in order of difficulty.
Methods
The Hammersmith SMA functional motor
scale: developing the scale
Choice of items
The intention of the scale was that it included both
upper and lower limb activities as well as trunk and
head control. It was not possible to add many
specific upper limb tasks without using additional
standardised equipment or making the instructions
to the assessor more complicated. It was felt that
the activities such as rolling fully prone/supine,
propping on arms and crawling, all gave indirect but
useful indicators of upper limb performance.
The activities included in the assessment were,
rolling, sitting, lifting the head from prone and
supine, getting to and from lying, propping on arms,
4 point kneeling, crawling and standing.
The ability to get into and out of standing is
complicated and the scoring would have to differ-
entiate the many ways this can be achieved. It was,
therefore, deliberately omitted.
Stepping was not initially included in the scale.
The decision to include stepping was made in order
to take into account the major difference between
type 2 and type 3 SMA, as it is possible to place and
balance some children in standing who are rela-
tively weak but they would not be able to move
into or out of the position. Half rolling to each side
was combined into one activity to allow for the
inclusion of stepping and still maintain twenty
activities.
In the initial version of the scale the activities
were ordered by starting position. Table 1 shows a
list of these activities.
The Hammersmith Functional Motor Scale for Children with Spinal Muscular Atrophy 157

Table 1 List of activities.

Lifts head from prone Frog/chair sitting

Lifts head from supine
1/2 roll from supine
Rolls prone to supine over R
Rolls prone to supine over L
Rolls supine to prone over R
Rolls supine to prone over L
Achieves prop on forearms
Achieves prop on extended arms
Gets to sitting from lying
Long sitting
Touches one hand to head
Touches two hands to head
Gets to lying from sitting
Achieves four point
kneeling
Crawls
Stands holding on
Stands independently
Takes .4 steps unaided

Scoring criteria
The scale consists of 20 items, each scored on a 3
point scoring system. Each activity scores 2 for
unaided, 1 for assistance and 0 for inability.
The only exceptions are the activities of lying
from sitting and lifting the head from prone, in
which 1 cannot be scored. A total score can be
achieved by summing the scores for all the
individual items. The total score can range from
0, if all the activities are failed, to 40, if all the
activities are achieved.
All items have to be tested without jacket or
orthoses. Time required for assessment depends on
the age, understanding and co-operation of the
children. In a fully co-operative child, the scale can
be completed in a maximum of 10 – 15 min.
Reliability
Inter-rater reliability was tested on 35 children
selected at random using two therapists to score
each patient. The same scores were obtained for all
items in 34 out of 35 tests with the remaining test
having a difference of only one point in one item.
The inter observer agreement was . 99%.
Graph 1 Distribution of scores in 30 normal controls.
Note that while the infants assessed before 30 months
had very variable scores, those assessed after 30 months
all had scores of 39 or 40.
varied depending on co-operation and understand-
ing. The scale was successfully completed in all the
children of 29 months or above and more than 90%
of them had a score $ 39 (Graph 1).
Application of the scale to patients with SMA
In order to establish the suitability of the scale in
patients with SMA type 2 and 3, the scale was also
applied to 58 patients affected by SMA older than 30
months. The age range was 30 months to 19 years
with a mean age of 7 years, 5 months. Thirty-nine of
the 58 had SMA type 2 and 19 type 3, but only 12 of
these 19 were still ambulant.
A total of 120 assessments were recorded in the
SMA cohort. Sixteen children were tested once, 28
were tested twice and 14 three times. Global scores
in the 120 assessments ranged from 0 to 40. Graph 2

Application of the scale to young infants

While all the activities chosen should be achieved
by the age of 2 years in normal children,12 it is
sometimes difficult to complete an examination in
young infants at this age. In order to verify the
suitability of the application of the whole scale to
very young children the scale was used on 30 control
infants, assessed between the ages of 10 months
and 3 12 years (mean age 27 months), with no known
disabilities.
Graph 1 shows the distributions of the scores
obtained in the young controls.
Infants below 30 months showed a quite wide Graph 2 The graph shows the results of 120 assessments
variability in their performance and although the performed in children with SMA, showing the number of
scale could be used in these children, the results children who passed individual activities.
158 M. Main et al.

Table 2 The Hammersmith SMA functional motor scale.

Name Date Age Hosp. no.

Score 2 points Score 1 point Score 0 Score

(1) Frog/chair sitting no hand support 1 hand support 2 hand support ()

(2) Long sitting, no hands 1 hand support 2 hand support ()
(3) 1/2 roll from supine, both ways One way (R/L?) Unable ()
(4) Touches one hand to head (R/L?) (in sitting) Flexes head to hand Unable ()
(5) Touches two hands to head (in sitting) Flexes head to hands Unable ()
(6) Rolls prone to supine over R Pushes on hand Unable ()
(7) Rolls prone to supine over L Pushes on hand Unable ()
(8) Rolls supine to prone over R Pulls on hand Unable ()
(9) Rolls supine to prone over L Pulls on hand Unable ()
(10) Gets to lying from sitting (safely, not accidentally) Unable ()
(11) Achieves prop on forearms-head up Holds position when placed Unable ()
(12) Lifts head from prone (arms down by sides) Unable ()
(13) Achieves four point kneeling-head up Holds position when placed Unable ()
(14) Achieves prop on extended arms-head up Hold position when placed Unable ()
(15) Gets to sitting from lying through side lying Through prone Unable ()
(16)Crawls Crawls, head up Unable ()
(17) Lifts head from supine Through side flexion Unable ()
(18) Stands holding on with one hand Stands with MINIMAL trunk support Knee/hip support needed ()
(19) Stands independently: count .3 Stands independently count of 3 Stands momentarily ()
(20) Takes .4 steps unaided Takes 2– 4 steps unaided Unable ()
Total ( )

All tests done without jackets/orthoses.

shows the frequency distribution of the scores for Discussion

each item in the SMA cohort. The final version of the
scale (Table 2) is based on these results, with the 20 In this paper we describe the use of a simple clinical
items ordered in terms of achievement. scale to assess functional ability in children with
SMA with limited mobility. The scale does not use
Development of a decimal scale for SMA any equipment and can, therefore, be used in any
clinical setting. It is also self-explanatory, indicat-
ing precisely how it is scored. While this scale has
As the total scores in our scale becomes progress-
been primarily devised for non-ambulant children
ively lower with reduced functional ability, we used
with SMA, it can be used for any child with
the distribution of the total scores in the scale to
neuromuscular disease causing a similar level of
identify the steps of progressive functional impair-
functional impairment.
ment, and to subdivide children within the type 2
All the activities included in the scale should be
range in 10 subgroups according to their level of
completed by the age of 2 years by children with
functional ability (Table 3).
normal developmental milestones.12 Very often,
however, very young infants will not fully collab-
Table 3 Decimal scale.
orate and part of the activities can be only
Maximum function achieved Score Grade assessed through play and observation. In our
experience the scale can be reliably used in
Sits 1–4 2.0 children above the age of 30 months. At this age
Half rolling supine to side 5–6 2.1
Touches 2 hands to head 9–10 2.2
more than 90% of the children with normal
Rolling fully prone $ supine 11–18 2.3 development will complete the scale achieving at
Achieves prop on forearms 19–22 2.4 least a score of 39/40. All the children who scored
Achieves 4 point kneeling 23–26 2.5 39 lost one point on the same item, being able to
Gets to sitting from lying 27–30 2.6 lift the head in supine only through side flexion.
Crawls 31–32 2.7
Lifts head in supine 33–34 2.8 These results, therefore, suggest that from the age
Stands independently 35–38 2.9 of 30 months onwards any score lower than 39
Walks .4 steps 39–40 3.0 should be considered suggestive of functional
motor impairment.
The Hammersmith Functional Motor Scale for Children with Spinal Muscular Atrophy
The scale was equally easy to apply to children
with SMA and allowed achievement of a more
detailed profile of the severity of functional
impairment. While we initially designed the scale
ordering the activities by starting position, follow-
ing the application of the scale to SMA children we
analysed the profile of their achievements and in
the final version of the scale the items were
arranged in order of difficulty. This has proved to
be more practical when assessing children. One,
however, should not forget that while the scale is
ordered according to the frequency distribution of
the items most easily achieved, this order may not
always apply to individual cases. Older children
with contractures, for example, might be able to
bring their hands to their head even if they have
failed long sitting. This suggest that, especially
when used for the first time in a patient, all items
should be assessed.
Having a total score and a detailed profile of the
items passed will not only allow a more precise idea
of the functional achievements of an individual
child but also to follow possible changes in func-
tional abilities over time. We are currently collect-
ing longitudinal data to establish the individual
changes over time in patients types 2 and 3 SMA.
These data will be particularly useful as a baseline
for longitudinal interventional studies or thera-
peutic trials.
The availability of a continuous range of func-
tional abilities allowed us to have a more accurate
classification of functional ability in patients with
SMA 2. The International Classification for SMA is
based on age of disease onset and maximum
function achieved, identifying three main groups
on the basis of functional achievement, clinical
severity and survival. There are, however, substan-
tial differences between children belonging to the
same category and this affects morbidity and
management. For example, children at the weaker
end of the spectrum of type 2 SMA can just sit
unsupported while stronger children are almost
able to stand. In order to provide a basis to take into
account the differences between children with
SMA, decimal classifications have been proposed.13
Prior to development of this scale, however, there
was no objective means of applying a decimal
classification to patients with type 2 SMA. Using the
distribution of the scores in our scale, we have been
able to propose a classification into decimals which
provides a more accurate description of functional
abilities at the time of the assessment within SMA 2
and 3, and can also be used as a guideline for
intervention.
The SMA scale has now been in use in this
department for 4 years and has become an integral
159
part of the SMA assessment. The decimal classifi-
cation has been very useful for therapists when
setting goals for SMA children, in our experience, a
child with a grade of 2.7 – 2.9 should walk well in
orthoses and be able to propel a manual wheelchair
on even surfaces. Children with grades of 2.8 – 2.9
should also be encouraged to perform their own
transfers. Children with grades of 2.1 – 2.3 can only
stand (when practical) in standing frames and will
need maximum assistance to perform all activities
of daily living.
As it has been recently reported in a longitudinal
study, the evaluation of maximal functional ability
is a reliable prognostic indicator5 and it will be
interesting to evaluate how the scores obtained
using our scale can predict functional outcome and
long term survival.
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