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Definition
and
Classification
of Cerebral
Palsy
The
UCP Research
and
Educational
Foundation
in
collaboration
with the
Castang
Foundation
(Great
Britain) and
the NINDS/NIH
organized
and
conducted a
symposium on
the
Definition
and
Classification
of Cerebral
Palsy.
Financial
assistance
was received
from the
Dana
Foundation
for this
endeavor.
The
definition
used at this
time is a
product of a
workshop
held in
1963; the
classification
systems
presently
used are the
product of a
wide variety
of meetings
and
activities.
In July
2004, 30
international
experts
discussed
the needs
for changes
in the
present
definition
and
classifications
in order to
provide a
basis for
improved
communication
between
clinicians,
investigators
and public
health
workers. As
a result of
the
deliberations
of the
panel, a
proposed new
definition
and
classification
system has
been
developed.
The proposed
new
definition
and
classification
has been
published in
an
international
journal (DMCN).
The final
meeting of
the panel
occurred in
UCP
Headquarters
in
Washington,
DC in
January
2006. The
final
document
will be
published
later this
year.
Proposed Definition
and Classification
of Cerebral Palsy,
Journal of
Developmental
Medicine and Child
Neurology 08/05
Because of the
availability of new
knowledge about the
neurobiology of
developmental brain
injury, information
that epidemiology
and modern brain
imaging is
providing, the
availability of more
precise measuring
instruments of
patient performance,
and the increase in
studies evaluating
the efficacy of
therapy for the
consequences of
injury, the need for
reconsideration of
the definition and
classification of
cerebral palsy (CP)
has become evident.
Pertinent material
was reviewed at an
international
symposium
participated in by
selected leaders in
the preclinical and
clinical sciences.
Suggestions were
made about the
content of a revised
definition and
classification of CP
that would meet the
needs of clinicians,
investigators, and
health officials,
and provide a common
language for
improved
communication. With
leadership and
direction from an
Executive Committee,
panels utilized this
information and have
generated a revised
Definition and
Classification of
Cerebral Palsy. The
Executive Committee
presents this
revision and
welcomes substantive
comments about it.
INTRODUCTION
Authors: |
|
Martin Bax, Murray
Goldstein,
Peter
Rosenbaum,
Alan Leviton,
Nigel Paneth |
Cerebral palsy (CP)
is a well-recognized
neurodevelopmental
condition beginning
in early childhood
and persisting
through the
lifespan. Originally
reported by Little
in 1861 (and
originally called
‘cerebral paresis’),
CP has been the
subject of books and
papers by some of
the most eminent
medical minds of the
past hundred years.
Beginning at the end
of the 19th century
Sigmund Freud1 and Sir William
Osler2 both
contributed
important
perspectives on the
condition. From the
mid-1940s the
founding fathers of
the American Academy
for Cerebral Palsy
and Developmental
Medicine (Carlson,
Crothers, Deaver,
Fay, Perlstein, and
Phelps) in the USA,
and Mac Keith,
Polani, Bax, and
Ingram of the Little
Club in the UK, were
among the leaders
who moved the
concepts and
descriptions of CP
forward, and caused
this condition to
become the focus of
treatment services,
advocacy, and
research efforts.
It has always been a
challenge to define
CP, as documented by
the number of
attempts that have
been made over the
years. For example,
Mac Keith and Polani3 defined CP as ‘a
persisting but not
unchanging disorder
of movement and
posture, appearing
in the early years
of life and due to a
non-progressive
disorder of the
brain, the result of
interference during
its development.’ In
1964, Bax4 reported and
annotated a
definition of CP,
suggested by an
international
working group, that
has become a classic
and is still widely
cited. It stated
that CP is ‘a
disorder of movement
and posture due to a
defect or lesion of
the immature brain.’
Although this brief
sentence is usually
all that is cited by
authors, additional
comments were added
by Bax: ‘For
practical purposes
it is usual to
exclude from
cerebral palsy those
disorders of posture
and movement which
are (1) of short
duration, (2) due to
progressive disease,
or (3) due solely to
mental deficiency.’
The group for which
Bax was the reporter
felt that this
simple sentence
could be readily
translated into
other languages and
hoped that it might
be universally
accepted. They felt
that it was wiser at
that time not to
define precisely
what they meant by
‘immature brain’, as
any such definition
might limit services
to those in need.
Like its
predecessors, this
formulation of the
CP concept placed an
exclusive focus on
motor aspects, and
also stressed the
specific
consequences of
early- as opposed to
late-acquired brain
damage. Sensory,
cognitive,
behavioural, and
other associated
impairments, though
very prevalent in
people with
disordered ‘movement
and posture due to a
defect or lesion of
the immature brain’,
and often
significantly
disabling, were not
formally included in
the concept.
The heterogeneity of
disorders covered by
the term CP, as well
as advances in the
understanding of
development in
infants with early
brain damage, led
Mutch and colleagues5 to modify the
definition of CP as
follows: ‘an
umbrella term
covering a group of
non-progressive, but
often changing,
motor impairment
syndromes secondary
to lesions or
anomalies of the
brain arising in the
early stages of
development.’ This
definition
emphasized the motor
impairment and
acknowledged its
variability,
previously
underscored in Mac
Keith’s and Polani’s
definition, and
excluded progressive
disease, a point
introduced in Bax’s
annotation.
An International
Workshop on
Definition and
Classification of
Cerebral Palsy was
held in Bethesda,
Maryland, July 11–13
2004, co-sponsored
by the United
Cerebral Palsy
Research and
Educational
Foundation in
Washington and the
Castang Foundation
in the UK, with
special support
provided by the
National Institute
of Health/National
Institute of
Neurological
Disorders and
Stroke. The task of
the attendees was to
revisit and, if
possible, update the
definition and
classification of CP
in the light of
emerging
understanding of
developmental
neurobiology and
changing concepts
about impairments,
functional status,
and ‘participation’.
Reassessment of the
definition of CP was
prompted by a host
of factors: changes
in delivery of care
to children with
disabilities;
recognition that
children with slowly
progressive inborn
errors of metabolism
can present with
motor difficulties
at times
indistinguishable
from those of
children with
non-progressive
disease; increased
availability of
high-quality brain
imaging to identify
impairments in brain
structure;
acknowledgment that
developmental motor
impairment is almost
invariably
associated with a
range of other
disabilities; and
increased
understanding about
associated
antecedents and
correlates of CP.
The group agreed
that CP as
conceptualized
previously had
proved to be a
useful nosologic
construct, but that
previous definitions
had become
unsatisfactory. They
underlined that CP
is not an etiologic
diagnosis, but a
clinical descriptive
term. Reservations
were expressed about
the exclusive focus
on motor deficit,
given that persons
with
neurodevelopmental
disabilities may
present with
impairments of a
wide range of
functions that may
or may not include
severe motor
manifestations,
thereby calling for
the need for an
individualized,
multidimensional
approach to each
affected person’s
functional status
and needs. However,
it was proposed that
the concept
‘cerebral palsy’
should be retained
to serve diagnostic,
management,
epidemiological,
public heath
services, and
research purposes.
It was felt that an
updated definition
of CP, taking into
account the advances
in the understanding
of physiological and
pathological brain
development as well
as changes in
terminology, should
be proposed for
international use to
meet the needs
associated with
these purposes, as
well as to enhance
communication among
clinicians and
scientists. As in
the original
concept, the motor
disorder is
emphasized, while it
is recognized that
other developmental
disorders can
accompany it. This
emphasis is
justified by
phenotypic
differences in motor
disorder according
to whether
pathological
processes occur
early or late with
respect to
development, with
different management
and outcome
implications. More
generally, it is
also justified in
the context of brain
developmental
conditions, given
the importance of
motor aspects in
child development.
Evidence of the
motor impairments of
CP is apparent in
the first 18 months
of life, but many
children who are
eventually formally
diagnosed with CP
have received
medical attention
for neonatal
difficulties such as
feeding problems
before their gross
motor function
difficulties become
apparent.
To underline the
idea that a
comprehensive
approach to CP needs
to be
multidimensional and
that management of
patients with CP
almost always
requires a
multidisciplinary
setting, disorders
commonly
accompanying the
motor aspects of CP
have been identified
in the refined
definition. This
addition reflects
the idea that CP is
one of a group of
neurodevelopmental
disorders which
involve numerous
developing
functions. As in
other
neurodevelopmental
disorders, various
manifestations of
disordered brain
function may appear
more significant in
different persons or
at different
periods, e.g. some
aspects of the motor
impairment,
intellectual
disability,
epilepsy,
attentional
difficulties, and
many others may be
more prominent, or
more problematic, at
different stages of
the life of a person
with CP.
What follows here is
an updated
definition and
classification of
cerebral palsy, an
annotated
explanation of the
terms used, and the
thinking behind the
choice of those
words. It is hoped
that this document
will spur
discussion, and lead
eventually to the
goal, first
envisioned by Bax 40
years ago, of
international
consensus and
adoption of a common
set of ideas about
this condition.
THE
DEFINITION OF
CEREBRAL PALSY
Authors: |
|
Peter
Rosenbaum,
Bernard Dan,
Reine
Fabiola,
Alan Leviton, |
|
|
Nigel Paneth,
Bo Jacobsson,
Murray
Goldstein
and Martin
Bax |
Cerebral palsy
(CP) describes a
group of disorders
of the development
of movement and
posture, causing
activity limitation,
that are attributed
to non-progressive
disturbances that
occurred in the
developing fetal or
infant brain. The
motor disorders of
cerebral palsy are
often accompanied by
disturbances of
sensation,
cognition,
communication,
perception, and/or
behaviour, and/or by
a seizure disorder.
ANNOTATION
Cerebral palsy (CP)1 describes a group2 of disorders3 of the development4 of movement and
posture5 causing6 activity limitation,7 that are attributed
to8 non-progressive
disturbances9 that occurred in the
developing fetal or
infant10 brain.11 The motor disorders
of cerebral palsy
are often
accompanied by12 disturbances of
sensation,13 cognition,14 communication,15 perception16 and/or behaviour,17 and/or by a seizure
disorder.18
COMMENTARY ON THE
TERMS AND CONCEPTS
-
‘Cerebral palsy’
(CP) – it was
generally agreed
that the CP
concept,
essentially a
clinical
formulation
based on
phenomenology,
remains useful
in the current
state of
nosology.
Although the
word ‘palsy’ has
become largely
obsolete in
medical
nosography and
it has no
univocal
connotation, the
term ‘cerebral
palsy’ is
entrenched in
the literature
and it is used
universally by
clinicians,
therapists,
epidemiologists,
researchers,
policy makers,
health care
funding
organizations,
and lay persons.
The term ‘CP’
has, however,
been variably
used, with poor
comparability
across different
places and
times,
indicating the
need for a
consensual
definition.
Epidemiologists
in particular
require
consistent
terminology and
concepts across
time and space
in order to
identify
changing
patterns of
diseases and
disorders. It
was proposed to
retain the term
to relate future
research in CP
to existing
published work,
but to clarify
several aspects
of the
definition in
this report.
-
‘a group’ –
there is general
agreement that
CP is a
heterogeneous
condition in
terms of
etiology as well
as in types and
severity of
impairments.
Several
groupings are
possible and
warranted to
serve different
purposes. These
groupings may
show overlap.
Therefore, the
singular form
‘CP’ is used (as
opposed to
‘cerebral
palsies’) as an
umbrella term.
-
‘disorders’ –
this refers to
conditions in
which there is
disruption of
the usual
orderly
processes of
child
biopsychosocial
development. The
disorders are
persistent.
-
‘development’ –
the notion of
alteration in
development is
essential to the
CP concept. It
distinguishes CP
from
phenotypically
similar
disorders in
children or
adults due to
late-acquired
lesions, at a
time when motor
development is
relatively well
developed. The
‘developmental’
aspect of CP is
also important
with regard to
management
strategies that
may include
interventions
that address the
developmental
consequences of
the functional
limitations
associated with
CP, and
interventions
that are
directed at the
underlying
neurobiological
processes. The
developmental
nature of CP
almost always
implies impacts
on the
developmental
trajectories of
the people who
have CP. The
motor
impairments of
CP manifest very
early in child
development,
usually before
18 months of
age, with
delayed or
aberrant motor
progress. The
clinical picture
of CP evolves
with time,
development,
learning,
training,
therapies, and
other factors.
-
‘movement and
posture’–
abnormal motor
behaviour
(reflecting
abnormal motor
control) is the
core feature of
CP. It is
characterized by
various abnormal
patterns of
movement and
posture related
to defective
coordination of
movements and/or
regulation of
muscle tone.
Patients with CP
may also have
other
neurodevelopmental
impairments that
can affect
adaptive
functioning,
sensory
function,
learning,
communication,
and behaviour,
as well as
seizures.
Abnormal motor
control may be
further impaired
by features that
are associated
with CP.
However,
patients with
neurodevelopmental
disabilities
that do not
primarily affect
movement and
posture are not
considered to
have CP.
-
‘causing’ –
activity
limitations are
presumed to be a
consequence of
the motor
disorder. Thus
disorders of
movement and
posture that are
not associated
with activity
limitations are
not considered
part of the CP
group.
-
‘activity
limitation’ –
the World Health
Organization’s
International
Classification
of Functioning,
Disability and
Health6 speaks of
‘activity’ as
‘…the execution
of a task or
action by an
individual’, and
identifies
‘activity
limitation’ as
‘…difficulties
an individual
may have in
executing
activities’.
This term
amplifies the
previous concept
of ‘disability’
to recognize
changing
international
concepts and
terminology.
-
‘attributed to’
– understanding
of developmental
neurobiology
(including the
effects of
genetic,
chemical, and
other influences
on brain
development) is
increasing
rapidly, such
that it is
becoming
possible to
identify
structural and
other evidence
of brain
maldevelopment
in people with
CP. As a
consequence,
structural-functional
connections and
correlations are
becoming more
clearly
delineated than
has previously
been possible.
It must,
however, be
acknowledged
that at the
present time a
full
understanding of
causal pathways
and mechanisms
leading to CP
remains elusive
in many cases.
-
‘disturbances’ –
this term refers
to processes or
events that in
some way
interrupt,
damage, or
otherwise
influence the
expected
patterns of
brain
maturation, and
result in
permanent (but
non-progressive)
impairment of
the brain. In a
proportion of
cases it is
currently not
possible to
identify a
specific
‘disturbance’ or
a specific
timing of the
events that
appear to impact
on maturation.
These
disturbances may
include cerebral
dysplasia.
-
‘fetal or
infant’– the
specification
‘fetal or
infant’ reflects
the idea that
disturbances
that occur very
early in human
biological
development
impact
differently on
motor function
than
disturbances
that occur
later, even
those that occur
in early
childhood. There
is no explicit
upper age limit
as, depending on
aspects of motor
functioning, the
first two or
three years of
life may be
concerned.
Therefore, the
notion of early
lesion would
appear more
useful
clinically than
arbitrarily
specified time
limits. In
practical terms,
disturbance
resulting in CP
is presumed to
occur before the
affected
function has
developed (e.g.
walking,
manipulation,
etc.).
-
‘brain’ – the
term ‘brain’
includes the
cerebrum, the
cerebellum, and
the brai stem.
It excludes
motor disorders
of spinal,
peripheral
nerve, muscular
or mechanical
origin. (Note,
however, that
alterations in
the
neuromuscular
and
musculoskeletal
systems may
occur in CP as a
consequence of
the chronic
motor
impairment.
These
alterations may
restrict further
motor function
of patients with
CP, and be
associated with
‘secondary’
changes in
skeletal
alignment and/or
functional
capacity.)
-
‘accompanied by’
– in addition to
the disorder of
movement and
posture, people
with CP often
show other
disorders or
impairments.
These may be
caused by the
same
disturbances as
those that
caused CP and/or
represent
indirect
consequences of
the motor
impairment
and/or be caused
by independent
factors (hence
the term
‘accompanied by’
as opposed to
‘associated
with’).
-
‘sensation’ –
vision, hearing,
and other
sensory
modalities may
be affected.
-
‘cognition’ –
both global and
specific
cognitive
processes may be
affected,
including
attention. Note,
however, that
when a child has
severely delayed
cognition and no
motor signs
(except perhaps
for some degree
of hypertonicity
or hypotonicity)
it is not usual
to include them
within the
concept of CP.
Review 573
-
‘communication’
– expressive
and/or receptive
communication
and/or social
interaction
skills may be
affected.
-
‘perception’ –
the capacity to
incorporate and
interpret
sensory and/or
cognitive
information may
be impaired both
as a function of
the ‘primary’
disturbance(s)
to which CP is
attributed, and
as a secondary
consequence of
activity
limitations that
restrict
learning and
perceptual
development
experiences.
-
‘behaviour’ –
this also
includes
behavioural
problems in the
context of
psychiatric
disorders, such
as features of
autism, ADHD,
mood disorders
and anxiety
disorders.
-
‘seizure
disorder’–
virtually every
seizure type and
many epileptic
syndromes may be
seen in patients
with CP. Rarely,
the seizure
disorder may be
the cause of CP
(e.g. as a
consequence of
prolonged
infantile status
epilepticus), or
it may result in
further motor
impairment. It
is hoped that
this definition
will clarify the
CP concept and
allow unified
use of the term
both within and
across the
concerned
fields. As it
relies
essentially on
clinical aspects
and does not
require
sophisticated
technology, it
should be
possible to
apply this
definition very
widely.
THE
CLASSIFICATION OF
CEREBRAL PALSY
Authors: |
|
Nigel Paneth,
Diane
Damiano,
Peter
Rosenbaum,
|
|
|
Alan Leviton,
Murray
Goldstein
and Martin
Bax |
CP describes a group
of disorders of the
development of
movement and
posture, causing
activity limitation,
that are attributed
to non-progressive
disturbances that
occurred in the
developing fetal or
infant brain. The
motor disorders of
CP may be
accompanied by
disturbances of
sensation,
cognition,
communication,
perception, and/or
behaviour, and/or by
a seizure disorder.
This proposed
definition of CP
covers a wide range
of clinical
presentations and
degrees of activity
limitation, and it
is, therefore,
useful to further
categorize
individuals with CP
into classes or
groups. The purposes
of classification
include the
following.
-
Description:
providing the
level of detail
about an
individual with
CP that will
clearly
delineate the
nature of the
problem and its
severity.
-
Prediction:
providing
information that
can inform
health care
professionals of
the current and
future service
needs of
individuals with
CP.
-
Comparison:
providing
sufficient
information to
permit
reasonable
comparison of
series of cases
of CP assembled
in different
places.
-
Evaluation of
change:
providing
information that
will allow
comparison of
the same
individual with
CP at different
points in time.
Traditional
classification
schemes have focused
principally on the
distributional
pattern of affected
limbs (for example
hemiplegia or
diplegia) with an
added modifier
describing the
predominant type of
tone or movement
abnormality (e.g.
spastic or
dyskinetic), but it
has become apparent
that additional
characteristics must
be taken into
account for a
classification
scheme to contribute
substantively to the
understanding and
management of this
disorder.
INFORMATION
REQUIRED FOR
CLASSIFICATION
The information
available for
providing an
adequate
classification of
the features of CP
in any individual
will vary over the
age span and across
geographic regions
and settings. The
role of aging in
changing the
clinical
phenomenology of CP
has been little
studied, and the
possibility of
classification
changes over time
cannot be completely
dismissed. Defining
the presence or
degree of associated
impairments, such as
cognitive deficits,
is age-dependent,
and in infants the
type of motor
disorder may be hard
to characterize.
Some young children
diagnosed as having
CP may in fact have
very slowly
progressive
disorders that have
not yet been
diagnosed.
Factors other than
age will affect
classification.
Historical data,
especially about the
course of pregnancy,
will vary in
reliability and
validity. Where
neuroimaging
facilities,
diagnostic
specialists, and
biochemical
laboratories are not
available, exclusion
of progressive
disorders cannot
always be ensured,
nor can underlying
pathology, as
described by
radiological
findings, be
incorporated into
classification. All
classification
results should,
therefore, indicate
the age of the
child, the nature of
the information
available from
clinical history
(e.g. whether from
clinical notes or
maternal recall),
and the extent to
which diagnostic
investigation
(metabolic or
radiological) has
been performed.
USES AND
LIMITATIONS OF A
CLASSIFICATION
SYSTEM
Classification often
requires making
difficult decisions
about where to draw
the boundaries
within ordinal or
quantitative
measures. Some
degree of
arbitrariness is
inevitable.
Assignment of
individuals with the
diagnosis of CP to
distinct clinical
groups is not
straightforward and
will differ
depending on the
characteristic( s)
chosen as the basis
for classification.
No one single
approach has emerged
as definitive;
depending on the
purpose of the
classification,
certain
characteristics or
combinations of
characteristics may
be more useful than
others. For example,
in assessing the
effectiveness of a
new treatment for a
specific type of
tone abnormality,
the nature of the
motor disorder and
the level of
functional motor
ability are likely
to be paramount,
whereas determining
service delivery
needs will require
the consideration of
associated
impairments.
No classification
system is useful
unless it is
reliable. It is,
therefore, not
enough to specify
the characteristics
to be used in
classification; they
must be
operationally
defined so that, in
general, competent
examiners will
classify the same
individual in the
same way given
identical
information.
However, providing
such definitions is
beyond the scope of
this document. For
example, the term
spastic diplegia is
problematic for
classification
because its existing
definitions are
variable and
imprecise, and
because we lack
evidence that the
term can be used
reliably. Some use
the term to describe
children with
spastic CP whose
only motor deficit
is in the legs,
whereas others
include children who
have arm involvement
of lesser severity
than leg
involvement.
However, determining
the relative
severity of arm and
leg involvement can
be challenging
because they perform
very different
functions.
DEVELOPMENT OF A
STANDARDIZED
CLASSIFICATION
SCHEME
The state of the
science underlying
the proposed
classification has
evolved in recent
years and continues
to progress at a
rapid pace,
particularly in the
area of quantitative
assessment of the
radiographic and
clinical features of
CP. These advances
will continue to
improve our ability
to classify children
and adults with CP
more accurately.
Table I indicates
the four major
dimensions of
classification we
propose, which are
elaborated upon
below.
Table I:
Components
of CP
classification
|
|
TABLE:
COMPONENTS
OF CP
CLASSIFICATION
1. |
MOTOR
ABNORMALITIES
A. Nature
and
typology
of
the
motor
disorder: the
observed
tonal
abnormalities
assessed
on
examination
(e.g.
hypertonia
or
hypotonia)
as
well
as
the
diagnosed
movement
disorders
present,
such
as
spasticity,
ataxia,
dystonia,
or
athetosis
B. Functional
motor
abilities: the
extent
to
which
the
individual
is
limited
in
his
or
her
motor
function
in
all
body
areas,
including
oromotor
and
speech
function
|
2. |
ASSOCIATED
IMPAIRMENTS
The
presence
or
absence
of
associated
non-motor
neurodevelopmental
or
sensory
problems,
such
as
seizures,
hearing
or
vision
impairments,
or
attentional,
behavioural,
communicative,
and/or
cognitive
deficits,
and
the
extent
to
which
impairments
interact
in
individuals
with
CP
|
3. |
ANATOMIC
AND
RADIOLOGICAL
FINDINGS
A. Anatomic
distribution: the
parts
of
the
body
(such
as
limbs,
trunk,
or
bulbar
region)
affected
by
motor
impairments
or
limitations
B. Radiological
findings: the
neuroanatomic
findings
on
computed
tomography
or
magnetic
resonance
imaging,
such
as
ventricular
enlargement,
white
matter
loss,
or
brain
anomaly
|
4. |
CAUSATION
AND
TIMING
Whether
there
is a
clearly
identified
cause,
as
is
usually
the
case
with
postnatal
CP
(e.g.
meningitis
or
head
injury)
or
when
brain
malformations
are
present,
and
the
presumed
time
frame
during
which
the
injury
occurred,
if
known
|
|
-
Motor
abnormalities
-
Nature and
typology of
the motor
disorder:
The type of
abnormal
resting
muscle tone
or
involuntary
movement 574
Developmental
Medicine &
Child
Neurology
2005, 47:
571–576
disorder
observed or
elicited is
usually
assumed to
be related
to the
underlying
pathophysiology
of the
disorder,
and may also
reflect
etiological
circumstances,
as in
kernicterus.
Individuals
with CP have
traditionally
been grouped
by the
predominant
type of
motor
disorder,
with a
‘mixed’
category
available in
those cases
when no one
type
dominates.
This
strategy has
been adopted
by the
classification
system
described in
the
Reference
and Training
Manual of
the
Surveillance
of Cerebral
Palsy in
Europe (SCPE),7 which
divides CP
into three
groupings
based on the
predominant
neuromotor
abnormality:
spastic,
dyskinetic,
or ataxic,
with
dyskinesia
further
differentiated
into
dystonia and
choreoathetosis.
However, an
argument can
be made that
many
children
have mixed
presentations,
and that
identifying
the presence
of each of
the tone and
or movement
abnormalities
may be of
greater
clinical and
etiological
utility, as
recommended
by the 2001
NINDS
workshop on
childhood
hypertonia. 8We
take a
compromise
stance here
and
recommend
that cases
continue to
be
classified
by the
dominant
type of tone
or movement
abnormality,
categorized
as
spasticity,
dystonia,
choreoathetosis,
or ataxia,
but that any
additional
tone or
movement
abnormalities
present
should be
listed as
secondary
types. The
term ‘mixed’
should not
be used
without
elaboration
of the
component
motor
disorders.
-
Functional
motor
abilities:
The World
Health
Organization
International
Classification
of
Functioning,
Disability
and Health,6 along with
several
other recent
publications,
has
sensitized
health
professionals
to the
importance
of
evaluating
the
functional
consequences
of different
health
states. The
functional
consequences
of
involvement
of the upper
and lower
extremities
should,
therefore,
be
separately
classified
by using
objective
functional
scales. For
the key
function of
ambulation,
the Gross
Motor
Function
Classification
System (GMFCS)
has been
widely
employed
internationally
to group
individuals
with CP into
one of five
levels based
on
functional
mobility or
activity
limitation.9 A parallel
classification
scale, the
Bimanual
Fine Motor
Function (BFMF)
Scale, has
been
developed
for
assessing
upper
extremity
function in
CP but has
not been as
extensively
studied as
the GMFCS.10 A newer
instrument
for
assessing
hand and arm
function –
the Manual
Ability
Classification
System
(MACS) – has
been shown
to have good
interrater
reliability
between
parents and
professionals,
and will
shortly be
published.11 We follow
SCPE in
recommending
that a
functional
classification
system be
applied to
hand and arm
function in
children
with CP.
Bulbar and
oromotor
difficulties
are common
in CP and
can produce
important
activity
limitation,
but there is
as yet no
activity
limitation
scale for
such
functions. A
high
priority in
research is
to develop a
scale for
speech and
pharyngeal
activity
limitation
in CP. In
the
meantime,
the presence
and severity
of bulbar
and oromotor
involvement
should be
recorded.
Although
activity
limitation
is
important,
the extent
to which
motor
disorders
affect the
ability to
participate
in desired
societal
roles is
also an
essential
consideration.
However, at
present the
evaluation
of
participation
restriction
(formerly
termed
‘handicap’)
in CP is not
well
developed,
and reliable
categorization
of children
on the basis
of this
aspect of
daily life
is,
therefore,
not yet
possible.
-
Associated
impairments In
many individuals
with CP, other
impairments
interfere with
the ability to
function in
daily life and
may at times
produce even
greater activity
limitation than
the motor
impairments that
are the hallmark
of CP. These
impairments may
have resulted
from the same or
similar
pathophysiological
processes that
led to the motor
disorder, but
they nonetheless
require separate
enumeration.
Examples include
seizure
disorders,
hearing and
visual problems,
cognitive and
attentional
deficits, and
emotional and
behavioral
issues. These
impairments
should be
classified as
present or
absent; if
present, the
extent to which
they interfere
with the
individual’s
ability to
function or
participate in
desired
activities and
roles should be
described. SCPE
recommends, and
we agree, that
the presence or
absence of
epilepsy
(defined as two
or more afebrile,
non-neonatal
seizures) be
recorded, and
that IQ,
hearing, and
vision be
assessed.
Although SCPE
provides
terminology for
describing
different
degrees of
cognitive,
hearing, and
visual
impairment, we
recommend
recording IQ
score, corrected
vision in each
eye, and decibel
loss (if any) in
each ear
whenever this
information is
available.
Standardized
instruments are
available to
measure IQ,
vision, and
hearing, and
categories
describing
specific levels
of dysfunction
(e.g. visual
impair- ment,
profound hearing
loss, and mild
mental
retardation*)
have come to be
generally
accepted.
-
Anatomic and
radiological
findings
-
Anatomic
distribution:
The pattern
and extent
of the motor
disorder in
CP with
regard to
different
anatomic
areas should
be
specified.
Previous
classification
schemes
included
only the
extremities
and required
a subjective
comparison
of severity
in the arms
and the
legs.
Notably
missing from
current
anatomic
classification
schemes is a
description
of truncal
and bulbar
involvement.
We recommend
that all
body regions
– trunk,
each limb,
and
oropharyx –
be described
in terms of
any
impairments
of movement
or posture.
A scale for
describing
truncal
posture in
CP has
recently
been
developed.12 It is
clearly
important to
distinguish
unilateral
from
bilateral
motor
involvement,
and
categorization
based on
this
distinction
has good
reliability.7 However,
even this
distinction
can be
blurred
because many
children
with
primarily
unilateral
CP may also
have some
degree of
motor
involvement
on the
opposite
side and
some
children
with
primarily
bilateral
involvement
may have
appreciable
asymmetry
across
sides.
Although the
terms
‘diplegia’
and
‘quadriplegia’
have been
extensively
used in
research and
clinical
practice, we
propose that
these terms
not be used
in
classification.
Gorter et
al.13 have
documented
the
imprecise
use of these
terms in
clinical
practice. We
advise that
the anatomic
distinction
between
unilateral
and
bilateral CP
be coupled
with a
description
of the motor
disorder and
functional
motor
classification
in both
upper and
lower
extremities.
-
Radiological
findings:
Until
recently,
correlations
between
radiographic
findings and
clinical
presentation
in CP were
weak.
However,
advances
both in
imaging
technology
and in
quantitative
motor
assessments
are changing
this
picture. The
goal of
categorizing
all patients
on the basis
of specific
radiographic
findings
will require
more
development
before
implementation,
but we
concur with
the
recommendation
of the
American
Academy of
Neurology to
obtain
neuroimaging
findings on
all children
with CP
whenever
feasible.14 At present,
information
is
insufficient
to recommend
any specific
classification
scheme for
neuroimaging
findings.
-
Causation and
timing It is
increasingly
apparent that CP
can result from
the interaction
of multiple risk
factors, and in
many cases no
identifiable
cause can be
found.
Therefore,
although every
reasonable
effort should be
undertaken to
investigate
causes or causal
pathways,
clear-cut
categorization
by cause is
unrealistic at
the present
time. It is
possible that by
looking further
downstream from
putative cause
to common
mechanisms of
injury, and by
grouping cases
on that basis,
we may
ultimately have
a more salient
method of
classification.
Timing of insult
should be noted
only when
reasonably firm
evidence
indicates that
the causative
agent, or a
major component
of the cause,
was operative in
a specific time
window, as, for
example, with
postnatal
meningitis in a
previously well
infant. Although
recording
adverse events
in the prenatal,
perinatal, and
postnatal life
of a child with
CP is
recommended,
clinicians
should avoid
making the
assumption that
the presence of
such events is
sufficient to
permit an
etiological
classification
that implies a
causal role for
these events in
the genesis of
CP in the
affected
individual.1
1Note:
See editorial and
commentaries at the
beginning of this
journal which deal
with issues raised
in this paper. In
addition the
editorial in the
July issue of DMCN
by Ingeborg Krägeloh-Mann
discusses areas of
interest raised
here.
Comments are also
invited on the Castang Foundation
website (where some comments
are already posted).
DOI:
10.1017/S001216220500112X
Accepted for
publication 25th
April 2005.
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*UK
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List of
Abbreviations
GMFCS |
|
Gross Motor
Function
Classification
System |
SCPE |
|
Surveillance
of Cerebral
Palsy in
Europe |
© UCP Research &
Educational
Foundation, August
2005
|