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Cognitive and Neuropsychological Functioning

in Children With Cerebral Palsy


Eileen B. Fennell, PhD, Thomas N. Dikel, PhD

ABSTRACT

This article reviews the extant literature on intellectual functioning in different subtypes of cerebral palsy. Following a
definition of the characteristics of each of three major cerebral palsy groups, typical neurologic and magnetic resonance
imaging findings are reported. More recent studies that examine the intellectual and neuropsychological functioning of
children within these classification groups are also reviewed. This review concludes that there remains a significant lack
of precise information about the impact of cerebral palsy on the intellectual, motor, and neuropsychological functioning
of children and that neuropsychological assessment can provide the necessary tools for such studies. (J Child Neurol
2001;16:58-63).

Baxl defined cerebral palsy as a disorder of movement and cerebral palsy As Menkes and Sarnat note,’ with the decline
posture resulting from a lesion of the immature brain. One in neonatal mortality, there has been an increase in the inci-
of a group of so-called &dquo;static encephalopathies,&dquo;z the brain dence of cerebral palsy ranging from 1 to 2 per 1000 births.22
lesions producing the clinical symptoms are nonprogres- The majority of these children are preterm infants with birth
sive. Clinically, however, the manifestations of cerebral palsy weights of 2500 g or less: the lower the birth weight, the
may evolve over time, and secondary complications may greater the risk of cerebral palsy.
arise as the child ages from the motor and movement dis-
orders and the associated sensory impairments that are pre- CLASSIFICATION OF THE CEREBRAL PALSIES
sent.3 Originally defined by Little in 1843 as arising from
difficult deliveries, cerebral palsy is now recognized to result Cerebral palsy is most often classified according to the
from a variety of prenatal, perinatal, and postnatal causes. clinical presentation of primary motor deficits, although
These include hypoxia-ischemia lesions, periventricular and some children present with a mixed picture of clinical
intraventricular hemorrhagic lesions, migrational defects deficits. The most commonly used classifications are
occurring early in fetal development, cerebrovascular mal- (1) spastic cerebral palsies, (2) extrapyramidal cerebral
formations, intrauterine infections, and central nervous sys- palsies, and (3) hypotonic cerebral palsies.
tem infections.’ Birth asphyxia accounts for between 10% and
14% of cases of cerebral palsy, whereas congenital factors SPASTIC CEREBRAL PALSY
account for about 50% of cases. 2,5 With the increased survival
of premature infants and the advances in neuroimaging, Spastic cerebral palsy results from dysfunction in the cor-
more precise definitions of the cerebral lesions have led to ticospinal tracts leading to increased muscle tone, increased
a better characterization of the differences in types and or hyperreflexia, and the persistence of primitive reflexes.’
locations of lesions in preterm and term infants who develop As a result of increased tone, contractures may develop in
the joints of the wrist, arm, ankles, hip, and knees. This group
Received Sept 15, 2000. Accepted for publication Sept 15, 2000. of cerebral palsies accounts for between 66% and 82% of
cases reported in several surveys.3 Spastic cerebral palsies
From the Department of Clinical and Health Psychology, University of
Florida, Gainesville, FL. are further subdivided into three groupings: spastic diple-
Presented at the University of Florida Consensus Conference in Child gia, spastic quadriplegia, and spastic hemiplegia.
Neurology: Management of Spasticity, May 4-6, 2000. Spastic diplegia is characterized by bilateral spasticity,
Address correspondence to Dr Eileen B. Fennell, Department of Clinical
and Health Psychology, University of Florida, PO Box 100165, Gainesville,
typically greater for the legs than the arms. The typical
FL 32610-0165. Tel: 352-265-0680, ext. 46893; fax: 352-265-0468; e-mail: magnetic resonance imaging (MRI) finding in spastic diple-
efennell@hp.ufl.edu. gia is periventricular leukomalacia with infarction in the

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59

vascular border zone of the internal capsule and thalamus.44 muscle or peripheral nerve diseases. Sometimes called
The lesion is thought to result from hypotensive perfusion of ataxic cerebral palsy, many children develop frank cerebellar
these regions and occurs more commonly in preterm than in symptoms including incoordination, gait disturbances, and
term infants.’ Term infants with spastic diplegia manifest a impairments in rapid coordinated successive movements.’
wider variety of MRI lesions, including periventricular leuko- The etiology of this form of cerebral palsy is unclear, with
malacia, micropolygyria, and porencephaly, as well as nor- some suggestion that it is the result of delayed development

mal MRIs.~ Seizures are fairly common in children with of the cerebellum or in the maturation of type-1 and type-2
spastic diplegia and are often generalized or focal seizures with muscle fibers.3 Rarely is perinatal asphyxia identified as
secondary generalization. Approximately 16% to 27% of chil- causally related to the disorder. MRI findings, when present,
dren with spastic diplegia will experience seizures.66 include a small cerebellum, enlarged ventricles, and gyral
Spastic quadriplegia is the most severe form of bilateral atrophy. Children with this disorder often develop learning
cerebral palsy with involvement of both upper and lower disabilities.’ Although specific subtypes of learning dis-
extremities. MRI findings in preterm infants with spastic abilities have not been described, in our experience, these
quadriplegia typically involve severe periventricular leuko- often involve deficits in motor programming for handwrit-
malacia. In term infants, cortical lesions are more common ing, attentional dysfunction, and slowed motor output,
and typically involve the parasagittal watershed distribution which affects the child’s abilities on timed tests. Intellectual
of cerebral vessels.’ Cystic lesions and polymicrogyria have functioning is rarely impaired among these children except
also been identified in the MRIs of full-term infants, sug- for lower scores on intelligence tests that are timed, such
gesting that earlier adverse events affected normal prolifer- as on the Performance Scale of the Wechsler Intelligence

ation and migrational processes in the developing brain. Scale for Children (WISC-III).7 Finally, there are a smaller
Seizure disorders are common among these children, and men- number of children who manifest a mixed clinical picture
tal retardation is frequent among the more severely affected. of both spasticity and extrapyramidal signs. The clinical
Spastic hemiplegia typically involves unilateral spasticity manifestations of these so-called &dquo;mixed cerebral palsies&dquo;
of the arm and leg contralateral to the lesion with greater include increased reflexes, dystonia, and/or other extrapyra-
involvement of the arm. Flexion contractures of the affected midal signs. Surveys estimate that approximately 13% of chil-
limbs are often present. Many different pathologies have dren with cerebral palsy will present with this mixture of
been identified in spastic hemiplegia, although the etiology clinical signs. ~ As a result, the MRIs of these children reflect
is unknown in about one third of the cases.’ The most com- the varying etiologies and structural lesions that give rise
mon etiologic factors identified include various intrauter- to the clinical picture.
ine insults including maternal hemodynamic disturbances,
placental emboli, anomalous fetal circulation, and maternal COGNITIVE FUNCTIONING IN CEREBRAL PALSY
infection.4 A variety of postnatal etiologies have also been
identified including bacterial meningitis, viral meningoen- Given the heterogeneous nature of the clinical picture in cere-
cephalitis, and vascular accidents. 3,4 Focal epilepsies and sen- bral palsy, it is difficult, if not impossible, to make satisfactory
sory deficits may be present in the affected limb(s).6 generalizations about the relationship of cerebral palsy and
cognitive functioning. The literature representative of stud-
EXTRAPYRAMIDAL CEREBRAL PALSY ies meant to pursue this issue is sparse and often does not
differentiate between types of cerebral palsy. Earlier
This group of cerebral palsies, which accounts for between reviews8~9 have given broad estimates of the ranges of IQs
5% and 22% of cases surveyed, is characterized by a variety among these children. Estimates among these children of
of abnormal motor patterns and postures that arise from the frequency of mental retardation, defined as IQ scores of
defective regulation of muscle tone and coordinated move- 69 or below, range from 50% to 70%. We know, however, that
ments.3 The pathology involves basal ganglia and extrapyra- rates vary significantly between levels of cerebral palsy
midal pathways. Sometimes referred to as dyskinetic cerebral and that levels of impairment vary within cerebral palsy sub-
palsy, manifestations of the disorder include involuntary type. Individuals with spastic quadriparesis are, in most
athetoid movements of the limbs or dystonic posturing of the cases, severely intellectually impaired, whereas half of
trunk and limbs MRI findings may include bilateral basal hemiparetic children have IQs in the average range, and 18%
ganglia lesions arising from kernicterus or hypoxic-ischemic achieve scores over 100.1° In children with spastic diplegia,
damage. Epilepsy is not common among these children. A there tends to be a general correlation between severity of
wide range of intellectual abilities have been reported, with motor deficit and level of retardation.:3 In cases of extrapyra-
4
many patients scoring within the normal ranges.4 midal cerebral palsy, delayed or deficient language skills due
to dysarthric incoordination of muscles of language and
HYPOTONIC CEREBRAL PALSY speech and significant gross motor handicaps can lead to
false underestimation of intelligence. In these cases, motor
This form of cerebral palsy is characterized by generalized function tends to be more impaired than cognition.3 Current
decreased muscle tone that persists from infancy beyond classification systems for cerebral palsy emphasize physical
age 3 years. The hypotonia is not the result of primary impairment and classification, and definition of retardation

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60

considers functional, or intellectual, limitations, disability, Ito et all&dquo; assessed 23 children with spastic diplegia
and societal limitations without a clear understanding of prior to and 2 years following entrance into school. After two
pathophysiology or the details of impairment.ll years of school, Wechsler Verbal IQs increased significantly,
but Wechsler Performance IQs did not change, thereby
INTELLIGENCE TESTING increasing the Verbal-Performance split. Wechsler Verbal IQs
of children studying in standard classrooms increased sig-
A measured level of intelligence is generally assumed to pro- nificantly more than those of children in special classes; how-
vide an indication of the extent to which one has developed ever, mean Wechsler Performance IQ increases were greater
the basic cognitive and academic skills required for success for children in special classes. Therefore, the increased
in our culture. 12 In addition, a child’s intelligence can influ- Verbal-Performance split was most pronounced for chil-
ence both general adaptation and the effectiveness of treat- dren studying in ordinary classrooms. Interestingly, six chil-
ment for cerebral palsy 13 ; data from intelligence testing can dren with spastic quadriplegia were assessed for Wechsler
be used to assess the efficacy of educational or intervention Verbal IQ. Although their Wechsler Verbal IQs were lower
programs and to provide indications of a child’s cognitive than those of diplegic children prior to entry into school,
progression or regression. there were no differences after 2 years of schooling. 18
However, the use of standard measures of intelligence Laterality of lesion may also impact performance on intel-
for assessing children with cerebral palsy poses significant ligence measures. On a battery of neuropsychological tests,
challenges. Test results must be interpreted in the context despite similar Wechsler Verbal IQs, right-hemiplegic children
of the motor, speech, visual, and auditory difficulties that performed significantly poorer than left-hemiplegic children
may be present in children with cerebral palsy. Wechsler Per- and sibling controls on measures of syntactic awareness
formance Scale subtests demand adequate vision and visual and repetition of semantically coherent material. 19 Another
discrimination, visual-motor coordination, gross and fine study found that mild to moderate right hemiplegics (left
motor skills, the ability to work quickly and efficiently, and hemisphere impaired) demonstrated impaired acquisition for
the ability to communicate responses to the examiner. Opto- drawings relative to control subjects.2° When comparing
metric examinations of children with cerebral palsy and intel- children with right- and left-sided hemiplegia with age-
ligence within the average range (standard IQ scores matched controls for verbal and nonverbal function, both
between 85 and 115) have demonstrated increased inci- hemiplegic groups were relatively impaired in nonverbal
dence of strabismus, amblyopia, nystagmus, optic atrophy, function.21 Although the right-hemiplegic group was more
and significant refractive errors. 14 Visual impairment may also impaired in verbal function than the left-hemiplegic or con-
be exacerbated by visual-perceptual difficulties, produc- trol groups, this impairment was evident only in the girls.
ing greater visual handicap than might be expected by visual When compared to sibling controls, children with right- and
acuity and strabismus alone. 15 Articulatory impairment and left-infantile hemiplegia demonstrated correlation between
speech intelligibility were examined in subjects with cere- left-hand impairment and poor arithmetic computational
bral palsy who were deemed to have adequate intelligence, skills.l9 The performance of right-hemiplegic children on
hearing, and ability to perform the required tasks. Results measures of syntactic awareness and repetition of seman-
identified characteristic dysarthric phonemic features such tically coherent material was, despite similar Wechsler Ver-
as anterior lingual place inaccuracy, reduced precision of bal IQ, significantly poorer than that of left-hemiplegic
fricative and affricate manners, and inability to achieve children.
extreme positions in vowel articulatory spaces Upper-
extremity motor impairment may interfere with the hand- SEIZURES AND CEREBRAL PALSY
eye coordination and fine and gross motor skills that become
sequentially increasingly demanding within the Perfor- The presence of seizure disorder may be related to increased
mance Scale subtests. risk for cognitive deficits. Vargha-Khadem and colleagues22
Another major issue affecting consideration of cogni- found that the scores of hemiplegic children on measures
tion and cerebral palsy pertains to the split between Verbal of memory and IQ were not correlated with hemispheric lat-
IQ and Performance IQ on Wechsler intelligence tests that eralization of lesion, and early cerebral damage to either
is often found in these children. In any assessment of intel- hemisphere tended to result in few and mild deficits. In
ligence, the use of summary scores assumes measure of a children with comorbid epilepsy, however, high incidence
unitary ability. If this is not the case, the reported IQ serves and degree of deficit, unrelated to hemisphere of lesion, were
in a limited capacity, much as a midpoint or forced average observed. An investigation of incidence of epilepsy in cere-
among a diverse array of strengths and weaknesses, that may bral palsy found epilepsy in over 50% of patients, with
misrepresent the child’s level of functioning.’z~’7 Moreover, seizure onset generally less than 4 years post cerebral palsy
intelligence testing generally presumes that individuals have diagnosis.&dquo; Epilepsy was associated with increased intel-
adequate coordination to accurately convey their nonver- lectual deterioration. In a more detailed review of the preva-
bal thinking abilities; violation of this assumption may lence, nature, and prognosis of epilepsy in cerebral palsy,
reduce the accuracy of the Wechsler Performance IQ as an 38% of the 85 reviewed cerebral palsy cases had epilepsy.24
estimate of nonverbal thinking However, the patients most commonly affected had spastic

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61

tetraplegia and diminished intellectual capacity. Zafeiriou abnormality or sulcal prominence. However, Melhem et
et al25 found overall prevalence rates of 36%, with increasing a1,~2 in a chart review of children with spastic cerebral palsy
incidence of epilepsy in hemiplegic (42%), tetraplegic (57%), and MRI-documented periventricular leukomalacia, found
dystonic (81%), and atonic-diplegic (88%) patients. Seizure larger mean lateral ventricular volumes in cognitively
onset occurred prior to the child’s first birthday in 70% of impaired groups relative to control groups and groups with-
children with cerebral palsy and epilepsy, and epilepsy was out cognitive impairment. They also found significantly
found to be a major prognostic factor for mental retardation larger lateral ventricular volumes in moderate and markedly
and poor motor development. Onset in the first year was also motor-impaired groups than in control or mild motor deficit
found in a majority of epileptic cerebral palsy patients, con- groups. A retrospective analysis of computed tomographic
stituting 42% of 323 cerebral palsy patients.26 Nearly 50% of (CT) examinations of 76 cerebral palsy children with no
patients with spastic tetraplegia and hemiplegia had comor- severe intellectual deficit showed abnormalities in 63%; in

bid epilepsy, with lower incidence in patients with spastic former preterm infants, that number rose to 88%.33 The CT
diplegia. abnormalities were generally posterior, bilateral, and sym-
In some cases, electroencephalographic abnormalities metric, often involving ventriculomegaly and affecting the
may be present in the absence of clinical signs. In a study contours of the lateral ventricles.
of 51 children with hemiparetic cerebral palsy, 80% had Single photon emission computed tomography (SPECT)
electroencephalographic abnormalities; however, less than was employed to evaluate cerebral perfusion impairment in
50% showed clinical signs.2’ Children with congenital anom- 51 children, ages 6 months to 6 years, 11 months with man-
alies were found to have higher incidence and earlier onset ifestations of cerebral palSy.31 Thalamic hypoperfusion was
of epilepsy than children with perinatal insult. 28 Children with detected in 50 children. Temporal lobe hypoperfusion was
term-type injuries were more likely to show these results found in 53%, basal ganglia in 41%, cerebellum in 39%, and
than children with preterm injuries. A study looking at extratemporal cortices in 22%. On the basis of MRI, however,
potential prognostic indicators of intelligence and seizures only 7 patients showed thalamic abnormality (2 in basal gan-
in hemiparetic cerebral palsy found that anatomic abnor- glia and 5 in extratemporal cortices) and 1 showed cerebellar
malities of commissural or association pathways or of cere- atrophy. Rather, the major MRI findings were white-matter
bral cortex were associated with higher incidence of seizures changes and thinning of corpus callosum.
and abnormal intelligence.6
NEUROPSYCHOLOGICAL ASSESSMENT
BRAIN IMAGING
Neuropsychological norm-reference testing provides a
Cranial MRI has been used to help determine etiologic fac- valuable objective index of evaluation of both baseline
tors in children with clinically documented motor delay. MRI function and functional change over time. However, a
abnormalities were found in 77% of children diagnosed with review of the literature demonstrates a paucity in neu-
cerebral palSy.21 Etiologic association was established solely ropsychologically based investigations of cognitive func-
from MRI in 23%, with 32% supporting suspected etiology. tion and functioning in general relative to cerebral palsy.
In no case was myelination delay the sole abnormality, and The following studies provide examples of the utility of neu-
only 17% of the children with minor motor delay had abnor-

ropsychology in better understanding the functional impact
mal scans. of cerebral palsy.
Brain imaging studies can be instructive in the devel- Kiessling and colleaguesl9 investigated differential hemi-
opment of our understanding of the etiology of cerebral spheric function in hemiplegic cerebral palsy in a study
palsy; however, imaging abnormalities may be more related population of 8 children with right hemiplegia, 8 with left
to visual-motor than cognitive abilities. Fedrizzi et al3° inves- hemiplegia, and 13 nearest-age normal siblings as a control
tigated correlation between Wechsler Full Scale, Verbal, group. Their study involved neuropsychological measures
and Performance IQs and features of periventricular leuko- of semantic comprehension, expressive language function
malacia on MRI. The investigators found a significant Ver- (confrontation naming), syntactic awareness, ability to
bal-Performance split, and periventricular leukomalacia repeat semantically coherent information, short-term mem-
was detected in all of the 30 children (age 6 years, 8 months ory, nonverbal reasoning, and academic skills (reading
to 14 years, 7 months). Severity of ventricular dilation, recognition, spelling, and arithmetic problem solving).
degree and extent of white-matter reduction, involvement Results indicated impaired function in right-hemiplegic (left
of optic radiation, and thinning of posterior corpus callosum lateralized brain lesion) children on measures of syntactic
were significantly correlated with Wechsler Full Scale and awareness and sentence repetition (high-level language
Performance IQs. No correlation was observed, however, function), providing evidence for a lateralized neural sub-
between Wechsler Verbal IQ and any analyzed MRI fea- strate of language ability, present at birth. The relationship
tures. In addition, Feldman et a131 found correlation between between extent and type of disability and measures of
degree of motor disability and the extent of T 2-weighted MRI degree of fine-motor (hand involvement) impairment demon-
white-matter signal abnormality, with no relation demon- strated significant correlation between hand impairment
strated between cognitive outcome and degree of signal and scores on syntactic awareness and sentence repetition

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62

tasks. Although syntactic comprehension was impaired in CONCLUSION _

the right-hemiplegic group, receptive vocabulary was gen-


erally intact. Left-hand function (right-brain lesion) Clinicians and researchers are faced with complex issues
correlated with math achievement scores, supporting the regarding the disposition of children with cerebral palsy.
idea that mathematical ability-math-estimating skills and Families, policy makers, and corporate interests want to
concepts of number and set-requires good visual-spatial know what sort of life to expect for children with cerebral
skills. palsy. Undoubtedly, a primary element in that equation is the
More recently, Craft and colleagues&dquo; studied alter- child’s cognitive ability. This review has indicated a general
ation in cognitive performance following selective dorsal lack of empirical research addressing these issues.
rhizotomy for treatment of spastic diplegic cerebral palsy. Most studies of cognitive effects of cerebral palsy have
Sixteen prematurely born, surgically treated children with reported either global IQ scores or vocational outcomes.
IQs over 80 were compared to 9 prematurely born children There is also a lack of studies attempting to examine the spe-
who had not been treated with selective dorsal rhizotomy cific site of brain lesions in relation to associated neu-
and 24 healthy age- and sex-matched children recruited from ropsychological functioning. Although some recent studies
a local school. Cognitive ability was assessed 1 day pre- have described a variety of etiologic factors in the diagno-
operatively and 6 months postoperatively. Visual attention sis of cerebral palsy, most have focused on premature and
was assessed by Posner et al’s covert orienting task low-birthweight infants with pre- and perinatal neurologic
which has a valid-cue, simple orienting response condi- lesions. Here, too, most report global estimates of IQ rather
tion and an invalid cue condition that involves inhibition, than provide detailed information on memory, visual-spatial,
disengagement, and redirection. Six additional measures of and language functioning, which would provide the basis for
cognitive function were taken from the Woodcock-Johnson developing specific remediation or accommodation strate-
Psychoeducational Battery.37 Results showed significant gies for the child.
between-group differences in changes in visual attention Future studies should attempt to more clearly delineate
performance over time. Both cerebral palsy groups were the relationships between MRI of brain lesions and the
slower than the healthy children in both conditions at base- functional effects of those lesions on early, middle, and late
line and follow-up assessments. No difference was shown childhood neuropsychological functioning. Although it is
between the cerebral palsy groups at baseline for either con- clear that different lesions are seen in different types of cere-
dition. At follow-up, there was no difference in perfor- bral palsy, there remains a relative dearth of knowledge on
mance between the cerebral palsy groups on the valid cue the developmental impact and functional expression of
condition. On the invalid cue condition, however, the chil- these lesions beyond motor symptoms and IQ scores. Bet-
dren in the selective dorsal rhizotomy group were signifi- ter definition of these functional brain-behavior relationships
cantly faster than the children in the nonoperated group, would allow us to better understand how to develop more
indicating an improvement in significant aspects of atten- specific educational, psychological, and vocational inter-
tional functioning. On the Woodcock-Johnson subtests, ventions to enhance or maximize outcome. Attempts to
both cerebral palsy groups performed more poorly than the understand the cognitive correlates of cerebral palsy must
healthy children on all measures, except memory for sen- investigate and present results by subtype rather than as gen-
tences, on both test administrations. All children generally eralizations regarding cerebral palsy as a whole. We suggest
showed improvement from the first to the second assess- that neuropsychological assessment provides a rich his-
ment, with degree of improvement similar for all groups, tory and solid empirical basis for such investigation.
with one exception. Children in the selective dorsal rhizot-
omy group demonstrated substantially greater improve- Acknowledgment
ment than children in the other groups on a measure of This research was supported, in part, by the National Institutes of Health,
National Research Service Award T32HD027524 from the National Institute of
visual-auditory associative learning. Through use of neu-
Child Health and Human Development.
ropsychological testing, the investigators were able to
demonstrate distinct improvements in attentional and cog-
nitive (visual-auditory learning) functioning following selec- References
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