A historical perspective on cerebral palsy as a concept and a diagnosis

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European Journal of Neurology 2005, 12: 582–587

REVIEW ARTICLE

A historical perspective on cerebral palsy as a concept and a diagnosis
A. Kavčič and D. B. Vodušek
Division of Neurology, University Medical Centre, Ljubljana, Slovenia

Keywords:                            Sigmund Freud was the first to write about cerebral palsy as a nosographic category,
cerebral palsy, Little’s             uniting various infantile motor deficits of brain origin. He did not ascribe more than a
disease, Sigmund Freud               temporary value to the term (infantile) cerebral palsy, but it has become – and still is –
                                     an indispensable part of the nosographic system. Nevertheless, it is still easier to
Received 16 July 2004                explain what cerebral palsy is not than to define it precisely.
Accepted 4 August 2004

                                                                          children had to be labelled generally in the sense of a
Introduction
                                                                          medical diagnosis, for Freud in his monograph on
If a historical study of cerebral palsy (CP) is under-                    infantile CP wrote that the term infantile CP charac-
taken, it soon becomes apparent that not only are there                   terized Ô… what already has been applied over a long
several definitions, but also a variety of interpretations.                period of time to pathological condition in which
The fact that CP has been known as a clinical entity for                  paralysis is overshadowed or replaced by muscular
over a hundred years (Freud, 1968), and is still to some                  rigidity or spontaneous muscular twitching.Õ (Freud,
extent loosely defined (Badawi et al., 1998; Bax, 2001),                   1968).
is surprising and, we think, worth considering.                              Freud’s concept of infantile CP was broader than
                                                                          that formulated some decades later by other authors.
                                                                          He actually suggested that this term should be applied
Freud’s analysis
                                                                          even to neurological cases in which paralysis was
In clinical practice, the term CP was often used inter-                   completely absent, as, for example, in children with
changeably with the eponym Little’s disease [after the                    mental retardation, or where disorders did not manifest
British orthopaedic surgeon who described spastic                         themselves permanently, such as epilepsy (Freud, 1968)
diplegia, which he considered to be due to birth com-                     [what is very close to the concept of (early) brain
plications (Little, 1862)] for many years (Freud, 1968;                   damage formulated much later (Amiel-Tison et al.,
Russell and Kennedy, 1937; Fanconi and Wallgren,                          1994)].
1950). It was, however, Sigmund Freud [a promising                           Freud probably decided to unite the various motor
neuropathologist from Vienna who became a promin-                         deficits of children in one nosographic category because
ent psychiatrist (Ferris, 1998)] who first wrote about                     he did not find any other alternative to bring more
(infantile) CP1 as a nosographic category, uniting var-                   order to the field. This hypothesis is supported by the
ious infantile motor deficits of brain origin (Freud,                      fact that he had begun studying motor deficits of chil-
1968).                                                                    dren separately. First he researched cerebral hemiplegia
   In all previous publications on motor deficit of chil-                  in co-operation with Oskar Rie (Freud and Rie, 1891).
dren, the term CP, if it had already been used, had been                  Then, he considered the remaining motor deficits of
written either only in a plural form (Osler, 1987; Sachs                  children. He unified them into one clinical group,
and Peterson, 1890) or, if in the singular, only in specific               termed cerebral diplegias, defined as those manifesting
combination with other words as a synonym for a                           a physical handicap of the whole body (i.e. both sides of
particular motor deficit (i.e. infantile meningeal hae-                    the body). Among these, he differentiated four main
morrhage – cerebral birth palsy, infantile hemiplegia –                   types: (1) general cerebral stiffness, (2) paraplegic stiff-
acute cerebral infantile palsy) (Gowers, 1888). Never-                    ness, (3) bilateral hemiplegia, and (4) general chorea
theless, in the routine clinical practice of the second half              and bilateral athetosis (Freud, 1893). Finally, he cov-
of the 19th century, (apparently) static motor deficits of                 ered all these various motor deficits by one nosological
                                                                          entity, i.e. infantile CP (Freud, 1968).

Correspondence: Professor David B. Vodušek, Division of Neurology,
University Medical Centre, SI-1525 Ljubljana, Slovenia (tel.:             Little’s view
+386 1 5221501; fax: +386 1 5222293; e-mail: david.vodusek@kclj.si).
1
                                                                          In the mid-19th century William John Little earned the
 The German term Ôdie infantile CerebrallähmungÕ used in the original
                                                                          reputation of substantiating the causal relationship
publication by Freud was translated into English as Ôinfantile cerebral
paralysisÕ, although Ôcerebral palsyÕ was preferred at that time
                                                                          between birth complications and disorders of mental
(Bartram, 1964; Bobath, 1969).                                            and physical development after birth (Little, 1843). His

582                                                                                                                      2005 EFNS
A historical perspective on cerebral palsy   583

views on this topic were summarized in the article                        From a distance of more than a hundred years, it is
entitled ÔOn the influence of abnormal parturition, diffi-               obvious that both Little and Freud contributed signi-
cult labours, premature birth, and asphyxia neonatorum,                ficantly to the understanding of the natural history of
on the mental and physical condition of the child, espe-               CP. Because Freud studied motor deficits of children
cially in relation to deformitiesÕ, probably the most                  approximately 3–4 decades later than Little, the ques-
commonly cited article on CP, which he addressed to                    tion of priority is not contested. Undoubtedly, Little’s
the Obstetric Society. From the three responses pub-                   aetiological approach represents one of the great mile-
lished at the end of the article, it is clear that Little              stones of studying motor deficits of children. This was
logically drew attention to the, at that time, unknown                 recognized immediately. By contrast, the value of
field of injuries to which the nervous system was liable                Freud’s conceptual approach had been growing almost
during, and immediately after, birth (Little, 1862). In                imperceptibly until decades later it was realized that his
connection with birth complications, he actually des-                  concept of CP was a great milestone as well.
cribed one clinical picture of non-progressive motor
deficit of brain origin, namely spastic rigidity, which, at
                                                                       Attempts to solve the terminological
the end of the 19th century, constituted one of four
                                                                       confusion
main clinical forms of such motor deficits in children
(Freud, 1968). All the same, no other name has become,                 In the 20th century it became gradually evident that CP
and remains, so closely associated with CP than his.                   was a useful nosographic category, although its ter-
                                                                       minology was rather confused (Bax, 1964). In the mid-
                                                                       20th century, as at the end of the 19th century, CP
Little versus Freud
                                                                       represented a clinical entity for some, while for others it
Given that Little discussed one clinical form of motor                 was just a general denotation for similar clinical entities
deficits of children, which he never termed CP, nor                     (Phelps, 1947; Perlstein, 1952). This lack of consensus
linked it with other motor deficits of brain origin, his                became less and less tolerable, for it made scientific
comparatively great influence on the common under-                      study difficult.
standing of CP seems at first sight unwarranted. Little’s                  The American Academy for Cerebral Palsy (later the
clear and objective reply to the immediate response of                 American Academy for Cerebral Palsy and Develop-
his contemporaries showed that he did not claim a                      mental Medicine) coped with this problem in 1953 by
pioneering position in discovering neurological sequelae               inquiring extensively of its members about the nomen-
of abnormal and premature parturition. When he found                   clature and classification of CP (Minear, 1956).
no reference to this theme in the works of English                        Another initiative for re-thinking the terminology of
medical writers, he referred to Shakespeare, who, uni-                 CP in the mid-20th century was taken by two Eng-
versal as he was, did not disappoint him. In the physical              lishmen, Ronald MacKeith and Paul Polani. Adherents
character of Richard III2, he in fact recognized a kind                soon began to gather around them, and in 1957 they
of deformity which, in his opinion, originated at birth                formed the Little Club (Bax, 1964).
(Little, 1862).
   Freud, however, combined all infantile motor deficits
                                                                       Great contribution but little contemporary
of brain origin except rapidly progressive into one
                                                                       influence
nosological entity (Freud, 1968). Certainly, it can be
said that Freud’s decision to introduce the unifying                   The terminological confusion that continued to afflict
concept was prudent, with the concept surviving for                    the field of CP for the first half of the 20th century
over a century. All the same, Freud, who supposed that                 shows that, with his concept of infantile CP, Freud did
motor deficits of children would eventually be defined                   not significantly influence his contemporaries. From the
more precisely (preferably aetiologically) by new in-                  viewpoint of the current understanding of CP, which is
sights into the human nervous system, did not ascribe                  basically the same as Freud’s, this is surprising.
more than a temporary value to the entity he intro-                       If the publications on infantile motor deficits of brain
duced (Freud, 1968).                                                   origin of the 19th century are read comparatively (Lit-
                                                                       tle, 1862; Vogel, 1870; Bastian, 1875; Gowers, 1888;
2
 ÔI that am curtailed of this fair proportion,                         Osler, 1987; Sachs and Peterson, 1890), it seems that
Cheated of feature by dissembling Nature,                              Freud’s work on this topic (Freud and Rie, 1891;
Deform’d, unfinish’d, sent before my time
                                                                       Freud, 1893; Freud, 1968), being both analytic and
Into this breathing world, scarce half made up,
And that so lamely and unfashionable                                   descriptive, was too theoretical for his time. He based
That dogs bark at me as I halt by them.Õ (Shakespeare, 1985; Little,   his work much more on literature review than on his
1862)                                                                  own clinical experiences. If there had not been the

 2005 EFNS European Journal of Neurology 12, 582–587
584    A. Kavčič and D. B. Vodušek

precise descriptions of infantile motor deficits of Jac-       The question, as to whether CP is, to a large extent, the
ques Mathieu Delpech, William John Little, William            result of birth complications, seemed thus practically
Osler, William Richard Gowers and many others,                solved. Then, studies appeared that demonstrated that
Freud would not be able to develop his insightful             birth asphyxia could not be regarded as a rare cause of
concept of CP. He finished his extensive work in this          CP among term infants (Hagberg et al., 2001; Moster
field with his monograph on infantile CP (Freud, 1968),        et al., 2001), leading to the renaissance of a hypothesis
and then became renowned for psychoanalysis. It               thought obsolete.
overshadowed practically all his other works (Ferris,
1998), perhaps contributing to the lack of influence of
                                                              Cerebral palsy in the 21st century
his excellent work on CP. In retrospect, this contributed
to the persistence of terminological confusion.               The efforts of the American Academy for Cerebral
                                                              Palsy and the Little Club notwithstanding, the discus-
                                                              sion on terminology and classification of CP continued
Causal relationship between birth asphyxia
                                                              (Bax, 1964; Mutch et al., 1992). In epidemiological
and cerebral palsy
                                                              studies, the Swedish classification of CP (Hagberg,
In over 20 years of orthopaedic practice, Little came to      1989) became by far the commonest classification used.
the conclusion Ôthat the proportion of entire recoveries      This has not changed yet, although recently the usage of
from the effects of asphyxia neonatorum is smaller than        the European classification has been slowly increasing
has hitherto been supposed.Õ He based his opinion on          (Surveillance of Cerebral Palsy in Europe, 2000). This
approximately 200 well-documented personal cases of           happened without there being evidence that the Swedish
spasticity with birth complications in their history          classification is the most appropriate, and without any
(Little, 1862). Because he did not use any statistical        particular efforts to establish a consensus. In defining
methods, but only a descriptive comparison with the           CP, pragmatism has not yet prevailed. Several different
general opinion of that time, we cannot know how              definitions are still used, although the compatibility of
frequent such motor deficit of children were in the            epidemiological studies is strongly dependent on the
mid-19th century.                                             uniformity of the methodology used.
   Freud considered the relationship between birth               If several definitions of CP are read one after an-
asphyxia and infantile motor deficit of brain origin           other, significant differences between them are per-
critically because of the fact that the development of        haps not noticed (Table 1). If a study of CP is
children with birth asphyxia could be completely nor-         planned, however, the definition (no matter which has
mal (Freud, 1893). Little, who was an orthopaedic             been chosen) has to be defined precisely, if a study is
physician, did not see such cases in his practice, or at      to be firmly based. It is not possible to avoid the
least not frequently. But Freud with his analytical ap-       ambivalence of the issue of brain maturation (at what
proach could not fail to notice these cases. He allowed       age the brain ceases to be immature). In the defini-
the possibility that spasticity could be a result solely of   tions of CP, formulations such as: Ôin the period of
intrauterine factors or combined with birth complica-         early brain growthÕ or Ôin the early stages of its (i.e.
tions (Freud, 1968).                                          brain) developmentÕ can still be found. It is also not
   Freud’s analysis of birth asphyxia as a cause of CP        possible to avoid the question as to whether aetiolo-
elicited no lively discussion among his contemporaries,       gically and/or pathologically well-determined motor
although it was noticeably different to Little’s views. As     deficit beginning early in life although not being
the usage of the term CP gradually increased, Little’s        hereditary, as for instance kernicterus because of Rh
conclusion on the causal relationship between asphyxia        incompatibility, or schizencephaly, are justifiably
(and other complications) during birth and spasticity         termed CP.
was even generalized to all clinical types of CP (Scott,         The upper age limit of the onset of motor deficit
1976).                                                        being recognized as CP is (exactly) defined in only a few
   Since the mid-20th century, when CP has also been          definitions of CP (Wilson, 1916; Vining et al., 1976).
studied epidemiologically, it has been shown again and        None of these has been used widely, although chrono-
again that relatively few cases can be explained on the       logical specification of the early stages of development
basis of birth asphyxia (Scott, 1976; Paneth, 1986;           (the immature brain) would make the definition of CP
Nelson and Leviton, 1991), although besides spastic           clearer. It does not seem a logical decision, but per se it
diplegia, as spastic rigidity was termed at that time, CP     was wise, for, instead of defining an authoritative
also comprised other forms of non-progressive brain           agreement on what exactly CP is, it enabled the gradual
motor deficits (i.e. other spastic, ataxic and dyskinetic      evolution of the concept, which more easily lent itself
disorders) (Hagberg et al., 1975; Mutch et al., 1992).        to the inclusion of new discoveries about the human

                                                                         2005 EFNS European Journal of Neurology 12, 582–587
A historical perspective on cerebral palsy          585

Table 1 Some characteristic/frequently used definitions of CP

ÔInfantile cerebral palsy would thus be defined as the general concept of all cerebral diseases in infancy caused by a direct effect of accidental
etiology, occurring either in the fetal period or after birth, and affecting one or more neuron systems.Õ (Freud, 1968)

Infantile cerebral palsy Ôis a paralysis of cerebral origin occurring in the first six years of life. It is always a hemiplegia, and the cases which affect
both sides of the body will be found to be no exception to this rule, but are simultaneous lesions upon the hemispheres.Õ (Wilson, 1916)

ÔCerebral palsy may be defined as a condition characterized by paralysis, paresis, incoordination, dyskinesia, or any aberration of motor function
that is due to involvement of the motor control centres of the brain.Õ (Perlstein, 1952)

ÔCerebral palsy is a descriptive term applied to a group of motor disorders of young children, in whom full function of one or more limbs is
prevented by paresis, involuntary movement, or incoordination.Õ (Balf and Ingram, 1955)

ÔCerebral palsy is a persistent 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.Õ (The Little Club, 1958 (Bax, 1964))

ÔThe term cerebral palsy does not designate a disease in any usual medical sense. It is, however, a useful administrative term which covers
individuals who are handicapped by motor disorders which are due to nonprogressive abnormalities of the brain.Õ (Crothers and Paine, 1988)

The term cerebral palsy Ômay be defined as one component of a group of childhood neurologic disorders which reflect cerebral dysfunction rather
than damage per se and which may result from cerebral maldevelopment, infection, injury, or anoxia before or during birth or in the early years of
life.Õ (Denhoff and Robinault, 1960)

Cerebral palsy is Ôa disorder of movement and posture due to a defect or lesion of the immature brain.Õ (Bax, 1964)

ÔCerebral palsy is a group of disorders characterised by reduced ability to make voluntary use of the muscles, caused by a non-progressive and non-
hereditary brain disorder arising before or at delivery or during the first years of life.Õ (Christensen and Melchior, 1967)

ÔCerebral palsy is the result of a lesion or maldevelopment of the brain, non-progressive in character and existing from earliest childhood. The
motor deficit finds expression in abnormal patterns of posture and movement, in association with an abnormal postural tone.Õ (Bobath, 1969)

ÔCerebral palsy is defined as a nonprogressive disorder of motion and posture due to brain insult or injury occurring in the period of early brain
growth (generally under 3 years of age).Õ (The Kennedy Institute (Vining et al., 1976))

ÔCerebral palsy is a descriptive term for a collection of nonprogressive neuromotor disorders of central origin that become manifest early in life and
are not the result of a recognized cerebral malformation.Õ (Paneth, 1986)

Cerebral palsy is Ôan 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 its development.Õ (Mutch et al., 1992)

nervous system function than a rigid definition without                         syndromes (diseases) to cover by the term CP and
a firm biological base could. Because of complex and                            which not (Badawi et al., 1998). They took traditional
dynamic processes, taking place simultaneously and                             use into account and so they in fact widened the CP
successively, depending on heredity and environment,                           category into other nosographic spectra for several
the ontogenetic development of the human brain can be                          cases had been included in CP registers only because
chronologically divided in several different ways, each                         knowledge had been insufficient to recognize them as
defendable (Connolly and Forssberg, 1997; Kandel                               separate entities.
et al., 2000).                                                                    The foregoing proposal of Australian experts pub-
   Knowledge of the human nervous system is con-                               lished in 1998 has not elicited an intensive debate yet.
tinuously growing; therefore it would be unlikely to                           Given that it did not bring into question the concept of
expect that the concept of CP will not continue to                             CP but its internal structure, it seems that CP will re-
evolve.                                                                        main a part of our nosographic system, at least in the
   Nadia Badawi et al. from Australia suggested that                           near future.
the concept of CP as a separate entity was becoming
outdated because of the better understanding of the
                                                                               How to redefine cerebral palsy?
pathology underlying infantile motor impairment syn-
dromes. But on the other hand they stated that the term                        To delineate CP firmly and clearly, a consensus is re-
CP was well recognized and still useful for service                            quired. The history of the term CP (certainly not short)
provision and management and hence it should not be                            shows that there has not been a complete or long-last-
abandoned. To improve interobserver agreement about                            ing consensus about details of CP as yet. We believe
the application of the term CP in epidemiological                              that it will always be difficult to achieve, for
studies, they provided an extensive list of various                            (unchangeable) biological dimensions of CP are less
developmental syndromes or infantile diseases with                             significant than (changeable) nosographic, therapeutic
motor impairment and put forward which of these                                or social ditto.

 2005 EFNS European Journal of Neurology 12, 582–587
586      A. Kavčič and D. B. Vodušek

Table 2 An example of the diagnostic criteria for cerebral palsy (CP)

Possible CP
Mandatory inclusion criteria: disorder of movement and posture manifesting as spastic diplegia, spastic hemiplegia, spastic tetraplegia, ataxia,
dystonia, choreo-athetosis - alone or in any combination; onset early in life; no evidence of progression.
Mandatory exclusion criteria: active disease that could explain the foregoing features; chromosomal disorders.
Supportive features: other signs of brain dysfunction that could be caused by the same pathological process as the foregoing disorders of movement
and posture (epilepsy, learning disorders, disorders of speech, vision or hearing); born after multiple pregnancy; vanishing twin syndrome;
intrauterine growth retardation; major antenatal placental abruption; preterm birth; acute intrapartum hypoxia; reduced fetal heart rate variability
from the onset of labour; extensive chorioamnionitis; congenital coagulation disorders; autoimmune disease of the mother; no child with the same/
similar clinical picture in a family.

Probable CP
Mandatory inclusion criteria: disorder of movement and posture as in possible CP; onset early in life; no evidence of progression or other disease
that could explain the foregoing features at school age.

Definite CP
Mandatory inclusion criteria: disorder of movement and posture as in probable CP, plus still no evidence of progression unrelated to aging or other
disease that could explain the foregoing features at age 18 or older.

   In neurology, there has been a tendency in the last                      Balf CL, Ingram TTS (1955). Problems in the classification of
decades to delineate ambiguously defined disorders by                          cerebral palsy in childhood. Br Med J July 16:163–166.
                                                                            Bartram JB (1964). Cerebral palsy. In: Nelson WE, ed.
diagnostic criteria. Such an approach works well in
                                                                              Textbook of Pediatrics, 8th edn. W.B. Saunders Company,
both clinical practice and scientific studies. We propose                      Philadelphia, PA, USA, pp. 1244–1247.
that agreement about the diagnostic criteria for CP (an                     Bastian CH (1875). On Paralysis from Brain Disease in its
example of them – incomplete as is incomplete our                             Common Forms. Macmillan and Co., London, UK.
knowledge of CP – is presented in Table 2) would prove                      Bax MCO (1964). Terminology and classification of cerebral
                                                                              palsy. Dev Med Child Neurol 6:295–297.
fruitful.
                                                                            Bax M (2001). What’s in a name? Dev Med Child Neurol 43:75.
                                                                            Bobath K (1969). The Motor Deficit in Patients with Cerebral
                                                                              Palsy. Spastics International Medical Publications in
Conclusion
                                                                              Association with William Heinemann Medical Books Ltd,
The concept of CP arose as a temporary solution in an                         Lavenham, Suffolk (Clinics in Developmental Medicine –
                                                                              no. 23).
era, which is separated from the present by amazing
                                                                            Christensen E, Melchior JC (1967). Cerebral Palsy. A Clinical
progress in neural science. CP has, nevertheless, become                      and Neuropathological Study. Spastics Society Medical
an indispensable part of the nosographic system. Per-                         Education and Information Unit in Association with
haps the umbrella term CP has continued to be used in                         William Heinemann Medical Books Ltd, London, UK
spite of new discoveries of the human nervous system                          (Clinics in Developmental Medicine – no. 25).
                                                                            Connolly KJ, Forssberg H (eds) (1997) Neurophysiology and
function because, as it is not firmly defined, it did not
                                                                              Neuropsychology of Motor Development. Mac Keith Press,
obstruct scientific progress; at the same time, however,                       London, UK (Clinics in Developmental Medicine – no. 143/
it can fill the appointed nosographic emptiness. What-                         144).
ever the explanation of this phenomenon might be, CP                        Crothers B, Paine RS (1988). The Natural History of Cerebral
remains a handy term for scientific description and                            Palsy. Blackwell Scientific Publications Ltd, Oxford, UK
                                                                              (Classics in Developmental Medicine – no. 2), (Original
medical diagnosis, although it is still easier to explain
                                                                              work published in 1959).
what CP is not than to define it precisely.                                  Denhoff E, Robinault IP (1960). Cerebral Palsy and Related
                                                                              Disorders. A Developmental Approach to Dysfunction.
                                                                              McGraw-Hill Book Company, Inc., New York, NY, USA.
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