Cerebral Palsy: Defining the Problem
Michael I. Shevell, MD, CM, FRCPC, and John B. Bodensteiner, MD
The term “cerebral palsy” has had varied meanings over the past century. This article presents the current
consensus definition that best captures the core elements of this heterogeneous entity. Elements comprising the
components of the consensus definition are elaborated upon to provide clarity. Areas of remaining lack of
consensus, mostly reflecting the timing of diagnosis and the exclusion of various etiologic entities are highlighted.
© 2004 Elsevier Inc. All rights reserved.
A DEFINITION provides the statement of the
meaning of a word, term, or concept. It de-
marcates and limits the outline of something, iden-
tifying its distinctiveness, core features, and es-
sence. It strives to provide clarity and agreement in
communication.1 Although the term “cerebral
palsy” was first used in the latter part of the
nineteenth century, a consensus definition was not
proposed formally until 1958.2 Put forward by the
Little Club, the following definition for cerebral
palsy (CP) was offered: “a persisting qualitative
motor disorder appearing before the age of 3 years
due to non-progressive damage of the encephalon
occurring before the growth of the central nervous
system is complete.”3
This early consensus definition and those that
have followed emphasize the heterogeneous nature
of CP with respect to etiology, clinical features,
severity, and outcome. As has been noted, “cere-
bral palsy is a symptom complex rather than a
specific disease.”4 Indeed, CP may be conceptual-
ized as a “term of convenience” that provides a
shorthand way of communicating regarding a
group of commonly encountered individuals shar-
ing impairments, medical requirements, therapeu-
tic needs and challenges.5 CP is not simply a
diagnosis of exclusion; if the elements of the def-
inition are present, then the entity can be properly
and surely diagnosed.6
The most recent consensus definition states that
CP is “an umbrella term covering a group of
non-progressive, but often changing, motor impair-
From the Departments of Neurology-Neurosurgery and Pe-
diatrics, McGill University, Montreal, Quebec, Canada; and
the Division of Pediatric Neurology-Barrow Neurological
Institute, St. Joseph’s Children’s Health Center, Phoenix, AZ.
Address reprint requests to Michael Shevell, MD, CM,
FRCPC, Room A-514, Montreal Children’s Hospital, 2300
Tupper, Montreal, Quebec, Canada H3H 1P3.
© 2004 Elsevier Inc. All rights reserved.
1071-9091/04/1101-0000$30.00/0
doi:10.1016/j.spen.2004.01.001
2 Seminars in Pediatric Neurology, Vol 11, No 1 (March), 2004: pp 2-4
ment syndromes secondary to lesions or anomalies
of the brain arising in the early stages of its
development.”7 For the sake of clarity, we consider
and elaborate on each element of this consensus
definition.
The phrase “umbrella term” captures the well-
recognized heterogeneity of CP.8 This heterogene-
ity is particularly evident in terms of etiology,
pathogenesis, and clinical manifestations. Clearly,
CP is not a single entity, and a wide spectrum of
possibilities is evident. It is this spectrum that
challenges the ongoing efforts to capture the es-
sential core and unvarying nature of CP in the
remainder of this most recent consensus definition.
The second element of the definition is that the
pathological basis for a child’s CP be nonprogres-
sive; that is, the cerebral lesion, whatever it might
be, neither resolves nor progresses.9 There are
several challenges in attempting to satisfy this
element.10 Consensus does not yet exist regarding
how long should a child be observed to exclude the
prospect of either resolution or progression. Fur-
thermore, observation occurs against the backdrop
of an immature nervous system undergoing rapid
change that is evident across developmental, func-
tional, cognitive, and language domains. Thus the
clinical picture of the affected child is expected to
change as the child matures. A final challenge is
that despite substantial advances in imaging tech-
nology, the actual nature of the pathological lesion
is often beyond our direct observation.
“Nonprogressive” excludes degenerative disor-
ders of gray or white matter, as well as neoplastic
processes.11 Strictly speaking, metabolic disorders
that feature repetitive ongoing insults to the central
nervous system should also be excluded. Uncer-
tainty exists regarding the inclusion or exclusion
of vascular (eg, moya-moya, mitochondrial en-
cephalomyopathy with lactic acidosis and strokes
(MELAS)) or traumatic (eg, shaken baby syn-
drome) disorders potentially featuring repetitive
acquired cerebral insults. A systematic review of
CEREBRAL PALSY: DEFINING THE PROBLEM
various conditions included and excluded in one
longstanding CP registry (Western Australia) has
been published.11 As recognized by the authors,
the inclusion or exclusion of a particular entity
often may be locally idiosyncratic.
A third element of the present consensus defini-
tion highlights that CP has at its core clinical
evidence for motor impairment.12 Motor impair-
ment refers to the objective evidence of abnormal
neurologic signs.13 Most often these signs are re-
lated to spasticity, featuring increased tone, exag-
gerated stretch reflexes, reemergence or excessive
persistence of primitive reflexes, and altered resis-
tance to passive stretch. The limb-specific distri-
bution of the observed spasticity provides a sche-
mata for clinical subcategorization of spastic CP.12
At other times, dyskinesias of movement (ie, dys-
tonia, chorea, athetosis) are the predominant neu-
rologic signs observed either singly or in combi-
nation with underlying spasticity (typically
quadriparesis). Rarely, the abnormal neurologic
signs may be predominantly those of hypotonia
with ataxia. Increasingly recognized are patients
who are the survivors of extreme prematurity and
present with mixed motor deficits including spas-
ticity, dystonia, and ataxia.14 Clinically, the motor
impairment may be evident through the delayed
acquisition of motor milestones, clumsiness, and
gait/ambulatory disturbances. The severity of the
motor disability is highly variable, ranging from
barely perceptible to a complete absence of pur-
poseful intended movement.
Although the definition highlights as essential a
motor impairment, other neurologic disabilities
frequently co-occur in the setting of CP.15 These
include significant developmental delay in other
domains (global developmental delay), frank cog-
nitive impairment (mental retardation), primary
sensory impairments (visual and/or auditory),
learning disorders (learning disability, attention
deficit hyperactivity disorder) and epilepsy (of all
types). Indeed, these other neurologic disabilities,
not the motor impairment, may be the major chal-
lenge facing the child and family.16 The presence
of these other neurologic disabilities does not in-
fluence the assignment of the CP diagnostic cate-
gory. The severity of these associated disabilities,
like the motor impairment itself, is highly variable.
The frequency of these associated disabilities var-
ies according to the specific type of CP and the
responsible etiologic factor.
3
By definition, CP is restricted to motor impair-
ment syndromes resulting from lesions of the
brain. Processes uniquely affecting the spinal cord
(eg, spinal muscular atrophy, myelomeningocele),
peripheral nerve (eg, giant axonal neuropathy,
neuro-axonal dystrophy), and muscle (eg, congen-
ital myopathies, muscular dystrophies), though re-
sulting in motor impairment syndromes of early
onset, are not considered under the rubric of CP.10
A final aspect of the definition is that the lesion
must have occurred in the early stages of develop-
ment. This has implications for the time frame for
case ascertainment.10 It is well known that many
abnormal neurologic signs noted early in infancy
resolve over time.17 Such transitory abnormalities
do not qualify as CP. Thus many centers and
registries have a lower limit of initial case ascer-
tainment at age 2 years with a confirmatory second
later age (often 5 years) at which the preliminary
diagnosis of CP is reaffirmed and validated.18 Lo-
cal variation exists regarding the precise timing of
initial tentative diagnosis and the second assess-
ment for validation.18 It is well recognized that
establishing an initial age of ascertainment at 2
years may not capture children with especially
severe CP who do not survive to this age. Often in
such severely affected children, the diagnosis of
CP is not doubted based on the combination of
readily observed and documented neurologic signs
and a nonprogressive underlying etiology. Al-
though most cases of CP are the result of a prenatal
factor or etiology, a small minority of cases are
postnatal in origin. These postnatal cases often are
vascular, infectious, or traumatic in origin. Con-
sensus does not yet exist regarding the upper time
limit for onset of such processes to be included
within the CP symptom complex. Many centers
and registries have chosen age 5 years as an upper
limit, but given ongoing evidence that the central
nervous system is not yet fully mature until later, a
definitive biological rationale for this upper limit is
not yet evident. Although variability in the time
frames for case ascertainment exists geographi-
cally, internal consistency within a specific setting
is necessary to accurately document epidemiologic
trends over time.10
Knowledgeable, seasoned observers may not al-
ways agree on assignment of the label of CP to an
individual case; but regardless of this uncertainty,
cogent reasons exist for retaining this concept.10
Despite heterogeneity in all spheres, children and
4 SHEVELL AND BODENSTEINER
adults with CP share certain challenges and needs.
Increasingly, therapeutic efforts are driven not by
cause, but rather by the spectrum of disability
apparent with the twin conjoint goals of minimiz-
ing limitations and encouraging fuller participa-
tion. Service provision is also directed by the
disability spectrum, with the increasing adoption of
a programmatic approach targeting affected areas
that evolve over the lifespan. Therapy and service
provision for individuals with CP is not uniform,
and thus the flexibility apparent in the CP diagnos-
tic construct is indeed an inherent strength of the
construct itself. The problem in CP is not in agree-
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ACKNOWLEDGMENTS
The authors would like to acknowledge the secretarial assis-
tance of Alba Rinaldi in the preparation of this manuscript, and
the Montreal Children’s Hospital Foundation for support during
the writing of this manuscript. M.I.S. is a Chercheur Boursier
Clinicien (Clinical Research Scholar) of the Fonds de Recher-
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