350 Archives of Disease in Childhood 1995; 72: 350-354

CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.72.4.350 on 1 April 1995. Downloaded from

Diagnosis and management of

Lewis Rosenbloom

The interest of clinicians in cerebral palsy has picture. Within the umbrella of this label it is fluctuated markedly over the years and has also important that the details of the motor been influenced serially by diagnostic con- impairments be described as precisely and siderations, classifications, epidemiological accurately as possible. studies, aetiological and pathogenetic contro- An increasing number of descriptive tools, versies, neonatal practice and follow up, and for example, the motor assessment inventory,2 by the variety of patterns of care, treatment, what has been described as the limb by limb and support available for children with neu- approach,3 and the gross motor function rodevelopmental disabilities and their families. measure4 are now available to do this. Their It is against this evolving background that this use in monitoring the progress of individual review summarises the availability and signifi- children is discussed presently. cance of information that can lead to a diag- nosis of cerebral palsy in young children and to its appropriate management. Classification From the perspective of early diagnosis classifi- cations are oflimited benefit as those which are Diagnostic considerations of most value are based on defined rather than Historically cerebral palsy has consistently evolving syndromes. Nevertheless it is advan- been described as an evolving disorder of tageous to appreciate the part played by an motor function secondary to there being non- agreed system of classification in correlating progressive pathology of the immature brain. epidemiological and clinical studies.5 The However, this begs important clinical, patho- classification used in Sweden and reviewed logical, and aetiological questions. recently by Hagberg and Hagberg is now

From the clinical viewpoint it is unsatisfac- widely accepted and is summarised as diplegia, http://adc.bmj.com/ tory to apply a label of cerebral palsy when the tetraplegia, hemiplegia, dyskinetic and ataxic movement disorder is either relatively subtle cerebral palsy.6 or alternatively is swamped. The former is Synonyms for tetraplegia include quadri- exemplified by children who are clumsy. This plegia and double hemiplegia. Children are is an important finding in its own right and in assigned to this grouping when they have severe some cases may well have an aetiological and spasticity of all four limbs, the upper limb dis-

clinical continuum with cerebral palsy. This is ability being the same or worse than that in the on September 30, 2021 by guest. Protected copyright. not true for all clumsy children, however, and lower limbs. In diplegia by contrast, there is it is much more to their advantage to have their lesser involvement of the upper limbs, although disabilities, including accompanying learning when present this may have an ataxic as well as difficulties, accurately delineated as a prelude a spastic component. In practice it may be diffi- to appropriate help being provided.1 cult to decide where diplegia ends and tetraple- Similarly it is not particularly useful to label gia begins and this difficulty is compounded by a child as having cerebral palsy if there is, for North American practice where many children example, some degree of or who are labelled quadriplegic would in Europe spasticity but with this overwhelmed by there be considered to have a diplegia. being a profound or severe degree of mental The dyskinetic group includes those with handicap. Within this context it needs to be fluctuating dystonia as well as involuntary kept in mind that severe hypotonia of central movements of choreoathetoid type. Where origin in infancy is a non-specific marker of there are mixed forms of motor disorder, the neurological dysfunction and that for older predominating one is used in classification. children it almost certainly requires a signifi- cant degree of cortical function to be acquired before persisting hypotonia can evolve into Epidemiology spasticity. Thus in practice the description of Studies from Sweden,7 Australia,8 and the hypotonic cerebral palsy, if it is to be used at UK9 have been very helpful in setting the Child Development all, will only be seen in association with very current scene with regard to the prevalence of Centre, Alder Hey severe degrees of mental handicap. the various cerebral palsy syndromes and how Children's Hospital, Eaton Road, Liverpool It is preferable therefore to reserve the diag- this is changing. What is particularly note- L12 2AP nosis of cerebral palsy to conditions where worthy is that there is an increasing proportion motor abnormalities dominate or at the very of children born very prematurely within the Correspondence to: Dr Rosenbloom. least are a prominent part of the clinical total cerebral palsy population and this is a Diagnosis and management ofcerebral palsy 351

direct consequence of there being increasing disabled of individuals with cerebral palsy, the numbers of preterm survivors, a constant pro- vast majority being severely mentally handi- portion ofwhom have impairments. Moreover, capped and epileptic with bulbar palsies, Arch Dis Child: first published as 10.1136/adc.72.4.350 on 1 April 1995. Downloaded from within that population are many children with microcephaly, growth failure, and visual severe motor disabilities and additional neuro- defects being common. The syndrome is seen developmental problems and this has very in both preterm and term infants and in the major implications for future resource and former is often accompanied by posthaemor- service provision. What is not yet known is rhagic hydrocephalus. A wide variety of path- whether the increased morbidity of preterm ologies including malformations, intrauterine infants is a consequence of their surviving infections, fetal , and peri- with prenatally determined abnormalities or natal hypoxic ischaemic can whether their problems are purely or primarily all manifest themselves ultimately in this way. a consequence of prematurity. So also can genetically determined disorders The epidemiological relationship between and particularly when a child presents with birth asphyxia and cerebral palsy also requires a symmetrical tetraplegia in the absence of elucidation. Case studies series commencing significant perinatal abnormalities, it is appro- with that of Little have linked perinatal events priate to estimate an empirical recurrence risk with cerebral palsy in a non-specific way and of one in eight to one in 10. often with systematic errors built into the Neuropathology and may research.'0 Cohort studies have also been used show a widespread multicystic encephalo- extensively in which markers of asphyxia, for pathy,17 often with cortical and subcortical example, birth acidosis have in general failed atrophy and gliosis,18 when there is a history of to yield increased numbers of children with perinatal adversity. cerebral palsy. This might well have been expected given that only a small increase could have been anticipated, although it is of interest HEMIPLEGIA that an Apgar score of 3 or less at 20 minutes is This is the second commonest syndrome seen associated with a 250-fold increased risk of in children born preterm and the commonest cerebral palsy. 1 l in term infants.7 Preterm hemiplegia has a Alternatively case-control studies have been non-specific association with birth problems.19 used in which children with cerebral palsy By contrast term hemiplegia most commonly are compared with controls for markers of results from events early in the third trimester asphyxia,12 13 and it is from these that the figure involving hypoperfusion,20 although malfor- of approximately 10% for an asphyxial cause for mations and late events leading to infarction cerebral palsy in children born at term has been within the distribution of a middle cerebral derived. It is relevant that a much smaller artery are not uncommonly seen. proportion than this can be demonstrated to

have received suboptimal perinatal care. http://adc.bmj.com/ The other epidemiological method used is DYSKINETIC CEREBRAL PALSY that of randomised controlled trials, for In current paediatric practice, dyskinetic cere- example, that of intrapartum monitoring used bral palsy occurs most often in term infants in Dublin.14 These hitherto have failed to show and has a close association with obvious peri- that differences in obstetric care are reflected natal adversities. Most frequently this is in reduced numbers of children with cerebral unforeseen acute circulatory failure with severe

palsy but again they have the disadvantage of but short lived birth asphyxia, although often on September 30, 2021 by guest. Protected copyright. not having been large enough for any measur- only a mild or moderate hypoxic ischaemic able change in outcome to be likely. encephalopathy.21 From the neuropathological and neuroradiological perspectives there is ample evidence of basal ganglia pathology as Aetiology and pathogenesis being the basis of the clinical abnormalities DIPLEGIA that result.22 23 The underlying mechanisms are those of These characteristically are of the dystonic periventricular leukomalacia and periventricu- type producing a very severe degree of motor lar haemorrhagic venous infarction.'5 The disability with preservation of primitive neo- diplegic syndromes are characteristically seen natal reflex patterns. In other survivors of in infants born prematurely with haemorrhagic severe and prolonged birth asphyxia, there can infarction of the periventricular areas being be both basal ganglia and cortical and subcor- seen especially in the increasing numbers of tical damage demonstrated with a resulting very preterm survivors. Radiological support mixed clinical picture.24 for this concept is available from magnetic The choreoathetoid form of dyskinetic form resonance imaging studies.'6 of cerebral palsy is, by contrast, now less com- Thus diplegia does not correlate with birth mon and this parallels the virtual eradication of asphyxia; rather its likely cause is hypoperfu- bilirubin encephalopathy. sion of periventricular structures at the time in gestation when these are most vulnerable. ATAXIC CEREBRAL PALSY As might be predicted there is often clinical TETRAPLEGIA difficulty in distinguishing congenital ataxic Within the terms of the classification detailed syndromes from the heredodegenerative atax- above, these are the most catastrophically ias in older children. Most congenital 352 Rosenbloom

have a prenatal origin that is often genetic, child and support for the parents to be offered. although acquired haemhorragic cerebellar Where the putative diagnosis is one of the lesions have been described.15 cerebral palsy syndromes the investigations to be performed are determined by the past Arch Dis Child: first published as 10.1136/adc.72.4.350 on 1 April 1995. Downloaded from history and no further tests at all might be indi- Diagnosis of cerebral palsy cated. In other circumstances genetic studies, Neurological abnormalities should always be neuroimaging, and biochemical tests may be routinely sought as part of neonatal care and relevant especially if there is no certainty that this applies especially when infants are signifi- the pathology is non-progressive. cantly premature or are recognised a' having Detailed chromosomal analysis is always been subject to other perinatal hazards. indicated when a malformation syndrome is Clinical changes that are found can often be suspected, when the child has dysmorphic correlated with concurrent neuropathological, features, or when there is a positive family neurophysiological, and radiological findings history. This may include the use of specific while the newer techniques of magnetic reso- techniques, for example, to identify fragile nance spectroscopy and near infrared spectro- X syndrome or Angelman's syndrome. Com- scopy25 offer the prospects of additional puted tomographic or magnetic resonance non-invasive assessment measures of cerebral brain imaging may be indicated if a progressive oxidative metabolism. However, currently neurological disorder is possible or to confirm available neonatal evaluation techniques have a to parents (or sometimes their legal advisers) less well established relationship with neuro- the nature and timing of acquired brain logical and developmental abnormalities seen lesions. The state of this art18 23 24 iS consider- in later infancy and childhood. ably in advance of conventional British and So far as specific clinical findings are con- European practice because of the limited avail- cerned, these have been well summarised by ability of magnetic resonance imaging and the Dubowitz who has detailed the common need for infants to be sedated or anaesthetised observations seen in infants who are small for for this procedure. their gestational age, those who survive signifi- Biochemical studies can be limited to cant intraventricular bleeding, the evolving thyroid function tests to exclude occult picture seen in periventricular leukomalacia, hypothyroidism and amino acid chromato- and the clinical stages of hypoxic ischaemic graphy unless children present with progressive encephalopathy.6 She rightly emphasises the or fluctuating neurological abnormalities. requirement to use age appropriate techniques and for examinations to be performed repeatedly with nevertheless there being con- Assessment of motor function tinuing difficulty in correlating focal lesions Historically the parameters for judging the with localising signs. Nevertheless, persistent severity of cerebral palsy have been subjective

generalised disturbances of tone, seizures, but a number of more objective measures have http://adc.bmj.com/ continued irritability or decreased alertness, become available relatively recently including persistent asymmetries of posture and move- the motor assessment inventory3 and the limb ment, and delay in establishing efficient by limb approach.4 Hallam and her col- feeding are all in favour of affected infants con- leagues27 have recently compared both these tinuing to be neurologically abnormal beyond measures with one another and with the the neonatal period. Griffiths's locomotor development quotient28 in respect of a population of vulnerable A combination of clinical and investigative on September 30, 2021 by guest. Protected copyright. features in the neonatal period should deter- children. They concluded that the limb by mine the details of follow up. Crucially the limb approach, which provides information on aims of this are to ensure optimal progress and the type, distribution, and severity of motor health of infants, to detect adverse sequelae involvement but not on a child's movement including all types of neurodevelopmental dis- abilities had the greatest sensitivity and was orders, and to initiate appropriate help. The easy to perform. It may very well be, however, details of relevant professional involvement that two other novel assessment methods, the require to be determined locally. gross motor function measure5 and the gross motor performance measure,29 will provide significant advantages. The role ofinvestigations The gross motor function measure looks at Irrespective ofneonatal findings a low threshold five functional areas of lying, sitting, crawling, of suspicion is required for infants who show standing, and walking on a four point scale for evidence of developmental delay (corrected each while the gross motor performance when necessary for prematurity), persistent measure, currently a research tool, is endeav- hypotonia, evolving dystonia or spasticity, focal ouring to examine the quality of movement abnormalities of movement, posture or tone, a demonstrated by children with disabilities. decreased rate of head growth, abnormal visual Motor function assessment for children with or auditory behaviour, and seizures. cerebral palsy has also to be set within a wider Under these circumstances and against a developmental context and appropriate background wherever possible of clear expla- measures for looking at cognitive, social, and nations to the parents, it is possible for a linguistic skills and sensory functioning require provisional diagnosis of a neurodevelopmental to be applied when the abilities of children disorder to be made, for appropriate investiga- with cerebral palsy are examined. However, tions to be initiated, and for treatment for the few standardised tests for these measures are Diagnosis and management ofcerebral palsy 353

available. Clinical pointers such as eye pointing recognition and treatment of drooling, and for and the rapidity and appropriateness of social there to be an awareness of neurological the increas- bladder involvement in cerebral responses may be important while palsy. Arch Dis Child: first published as 10.1136/adc.72.4.350 on 1 April 1995. Downloaded from ing availability and use of computers with It should also follow that more relevant specialised switching is likely to become par- targets for cerebral palsy research and service ticularly advantageous in the near future. provision can be set. Appreciable outcomes for services should include evaluation of motor developmental achievements, the prevention Physical treatment of avoidable deformities, achieving satisfactory The provision of appropriate physiotherapy nutrition and growth for children with cerebral programmes is a mainstay of treatment for palsy, provision of relevant appliances includ- young children with cerebral palsy and sooner ing communication aids, and the availability of or later it is then usual for physiotherapy to be suitable curricula and teaching methods. incorporated into the broader curricular Proceeding through childhood and appreci- requirements of children with physical disabili- ating that the majority of those with cerebral ties. It follows in logic, if not in established palsy become adults38 it could reasonably be fact, that appropriate physical treatment will claimed that it is effective adaptation to adult lessen the effects of these. However, the life, with the provision of appropriate resources methodology required to demonstrate this has for this, that is the ultimate outcome measure proved difficult to develop and given also the for cerebral palsy that requires satisfaction. emotional investment of parent in physiother- It is only in this way that the major advances apy availability for their affected children it is in knowledge concerning the aetiology and not surprising that objective measures of its pathogenesis of the cerebral palsy described at effectiveness have proved elusive. the beginning of this review, can serve as a Available methodologies were described by foundation for alleviating the long term dis- Scrutton30 and limited studies of the effective- abilities that arise as a consequence of their ness of varieties of neurodevelopmental treat- occurrence. ment have been presented by a number of 1 Gordon N, McKinlay I, eds. Helping the clumsy child. groups of workers.31-35 Results in all of these Edinburgh: Churchill Livingstone, 1980. studies have been indeterminate because ofthe 2 Chandler LS, Andrews MS, Swanson MW. A movement assessment ofinfants screening test manual. Rolling Bay, WA: small groups of children involved and limited University of Washington, 1987. duration of follow up. Alternative methods of 3 Evans P, Johnson A, Mutch L, et al. A standard form for recording clinical findings in children with a motor deficit physical treatment have some support often as of central origin. Dev Med Child Neurol 1989; 31: 119-20. a consequence of media involvement. In the 4 Russell DJ, Rosenbaum PL, Cadman DT, et al. The gross motor function measure: a means to evaluate the effects of UK; conductive education36 is one such physical therapy. Dev Med Child Neurol 1989; 31: 341-52. approach but in the only published outcome 5 Mutch L, Alberman E, Hagberg B, et al. Cerebral palsy epidemiology: where are we now and where are we going? study its effectiveness was not established.37 Dev Med Child Neurol 1992; 34: 547-55.

However, it is within the twin contexts of 6 Hagberg B, Hagberg G. The origins of cerebral palsy. In: http://adc.bmj.com/ David TJ, ed. Recent advances in paediatrics XI. cerebral palsy as an evolving clinical disorder Edinburgh: Churchill Livingstone, 1993: 67-83. and appropriate multidisciplinary practice, 7 Hagberg B, Hagberg G. The changing panorama ofinfantile hydrocephalus and cerebral palsy over forty years: a that promoting motor function and limiting Swedish survey. Brain Dev 1989; 11: 368-73. motor impairments as a consequence of 8 Stanley FJ. An epidemiological study of cerebral palsy in Western Australia 1956-1975. 1. Changes in total inci- physiotherapy require to be placed, rather than dence of cerebral palsy and associated factors. Dev Med that physiotherapy should be regarded as a Child Neurol 1979; 21: 701-13. 9 Pharoah POD, Cooke T, Rosenbloom L, Cooke RWI.

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