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Develop Med Child Neuro - 2014 - Jenni - Stability of Cognitive Performance in Children With Mild Intellectual Disability
Develop Med Child Neuro - 2014 - Jenni - Stability of Cognitive Performance in Children With Mild Intellectual Disability
Develop Med Child Neuro - 2014 - Jenni - Stability of Cognitive Performance in Children With Mild Intellectual Disability
1 Child Development Center, University Children’s Hospital Z€urich, Z€urich; 2 Children’s Research Center, University Children’s Hospital Z€urich, Z€urich; 3 Centre
Hospitalier Universitaire Vaudois and University of Lausanne, Statistical Unit, Institute of Social and Preventive Medicine, Lausanne, Switzerland.
Correspondence to Oskar G Jenni at Department of Pediatrics, Child Development Center, University Children’s Hospital, Steinwiesstrasse 75, CH-8032 Z€urich, Switzerland.
E-mail: oskar.jenni@kispi.uzh.ch
PUBLICATION DATA AIM Longitudinal studies that have examined cognitive performance in children with
Accepted for publication 19th September intellectual disability more than twice over the course of their development are scarce. We
2014. assessed population and individual stability of cognitive performance in a clinical sample of
Published online 2nd November 2014. children with borderline to mild non-syndromic intellectual disability.
METHOD Thirty-six children (28 males, eight females; age range 3–19y) with borderline to
ABBREVIATIONS mild intellectual disability (Full-scale IQ [FSIQ] 50–85) of unknown origin were examined in a
FSIQ Full-scale IQ retrospective clinical case series using linear mixed models including at least three
SES Socio-economic status assessments with standardized intelligence tests.
RESULTS Average cognitive performance remained remarkably stable over time (high
population stability, drop of only 0.38 IQ points per year, standard error=0.39, p=0.325)
whereas individual stability was at best moderate (intraclass correlation of 0.58), indicating
that about 60% of the residual variation in FSIQ scores can be attributed to between-child
variability. Neither sex nor socio-economic status had a statistically significant impact on
FSIQ.
INTERPRETATION Although intellectual disability during childhood is a relatively stable
phenomenon, individual stability of IQ is only moderate, likely to be caused by test-to-test
reliability (e.g. level of child’s cooperation, motivation, and attention). Therefore, clinical
decisions and predictions should not rely on single IQ assessments, but should also consider
adaptive functioning and previous developmental history.
Cognitive performance tests are widely used in the clini- references see Conley1 and a recent twin study considering
cal evaluation of children with intellectual disabilities. genetic and environmental contributions2) and in children
Medical diagnostic and therapeutic procedures as well as with genetic syndromes such as Fragile X, Down syn-
the need for special educational services are often decided drome, or Williams syndrome,3–7 only few studies exist
on the magnitude of intellectual impairment of these indi- that include children with mild intellectual disability of
viduals. For example, special needs services are offered unknown origin (i.e. non-syndromic or unspecific intellec-
depending on individual IQ test scores (e.g. children with tual disability). For instance, Naglieri and Pfeiffer8 studied
an IQ<70 or 75 will receive support, while others will 54 children with a mean Full-scale IQ (FSIQ) of 75 at age
not). Moreover, physicians may initiate further diagnostic 9 years, re-tested them 3 years later using the Wechsler
procedures (such as genetic testing, magnetic resonance Intelligence Scale for Children (WISC-R) and found a cor-
imaging, and so on) if an IQ is below a certain cut-off relation coefficient of 0.56. Similarly, Friedman et al.9
value or if cognitive decline is occurring. In this context, assessed 44 individuals with intellectual disability with a
a critical question is whether cognitive functions remain mean FSIQ of 80 at age 9 years, examined them 1.5 years
stable over time and, thus, are valid measures of the true later and found a correlation coefficient of 0.68 on the
abilities of the individual and therefore support further WISC FSIQ. In 2008, Whitaker10 summarized all available
clinical decisions. One approach to address this question studies in a meta-analysis on the stability of IQ in patients
is to study children with intellectual disability several with mild intellectual disability of unknown origin. Includ-
times during their development. ing 17 studies in his analysis, he found a weighted mean
While there is an abundance of earlier studies about the correlation coefficient of 0.82 for FSIQ over a mean test–
stability of IQ test scores in children with typical develop- retest interval of 2.8 years. He concluded that IQ remains
ment (for an older, but comprehensive review and relatively stable over time in children and adolescents with
5 10 15 20 5 10 15 20 5 10 15 20
100
85
70
55
40
100
85
70
55
40
100
85
Full-scale IQ
70
55
40
100
85
70
55
40
100
85
70
55
40
100
85
70
55
40
5 10 15 20 5 10 15 20 5 10 15 20
Age (years)
Figure 1: Illustration of the individual longitudinal profiles of all 36 individuals. K-ABC, German version of the Kaufman-Assessment Battery for Children;
HAWIK-R or HAWIK-III, German version of the Wechsler Intelligence Test for Children; HAWIVA, German version of the Wechsler Preschool and Pri-
mary Scale of Intelligence; SON-R or SON-Y, Snijders-Oomen Nonverbal Intelligence test.
Bootstrap-t using 2000 replicates (resampling of individuals) were used to construct 95% CI. A variance stabilizing transformation was used
to compute the CI of the intraclass correlation. Note that a 95% CI that does not include zero indicates a statistically significant result
(p<0.050). FSIQ, Full-scale IQ; K-ABC, German version of the Kaufman-Assessment Battery for Children; HAWIK-R or HAWIK-III, German
version of the Wechsler Intelligence Test for Children WISC; HAWIVA, German version of the Wechsler Preschool and Primary Scale of
Intelligence; SON-R or SON-Y, Snijders-Oomen Nonverbal Intelligence test.
down the average FSIQ score at a given age thus generat- explained by the increasing weaknesses in abstract reason-
ing biased estimates. We note that neither year of birth, ing and higher symbolic language skills, which are part of
sex nor SES at study entry were found to have a statisti- the cognitive testing procedures of these individuals.4
cally significant impact on FSIQ scores, which is in line Regarding individual stability, the estimated intraclass
with earlier studies (e.g. SES was not associated with any correlation coefficient indicates that the average correlation
IQ stability measures in children with mild intellectual dis- between two FSIQ measurements made on the same indi-
ability20). On the other hand, the data did support the exis- vidual is 0.58 (CI 0.46–0.74). Thus, the correlations from
tence of a practice effect. test to test are rather moderate. In fact, the figure illustrates
In terms of population stability, we observed that after individual profiles, which reflect ‘bouncing and rebouncing
adjusting for tests, practice and cohort effects, SES at study trends’ rather than steady patterns of improvement or
entry, and sex, the FSIQ of the children tended to decrease decline (as expressed by Moffitt et al.21). We have recently
over time, although this result was not statistically signifi- investigated the intraindividual stability of neuromotor and
cant (0.38 points of IQ per year, p=0.325). In contrast, if IQ scores in a sample of 256 healthy children of the Zurich
one would select a subset of healthy children scoring below Longitudinal Studies and reported a correlation between
85 at their first visit, one would expect their average FSIQ two FSIQ measurements in these healthy individuals of
to improve over the next few visits. Some children with 0.72 (CI 0.68–0.77) for measurements taken 4 years apart.22
average or above-average individual performances may This finding may suggest that long-term individual stability
score below 85 at some point only because of random of FSIQ in children with borderline to mild intellectual dis-
measurement errors. At the next visits these children would ability is lower than that of children with typical develop-
likely score higher thus inducing a general improvement in ment. In fact, cooperation, motivation, fatigue, and
population IQ performances (regression to the mean). The attention may play a more important role in the clinical
magnitude of that regression to the mean depends on the population of children with intellectual disability during the
proportion of variance related to measurement errors; a test situation than in typically developing participants. Of
larger measurement error variance would induce a larger note, our estimated intraclass correlation is lower than the
potential improvement in the population mean. Although average correlation of 0.82 found in the meta-analysis
the confidence interval for b5 (Table II) cannot rule out a reported by Whitaker.10 This may be because the follow-
positive trend in the evolution of population IQ score over up was longer in our study with more changes of tests along
time, the fact that the regression to the mean was not the years. However, recall that even a correlation of 0.8 as
observed on our sample suggests that, on average, cogni- found by Whitaker10 cannot be considered as high when
tive performance of clinically referred children with bor- the focus lies in predicting future IQ levels of a child. In
derline to mild intellectual disability should not be fact, with such a correlation a 95% CI for the difference
expected to improve over time, but rather remain relatively between two IQ measurements performed on the same
pffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffi
stable. This high population stability in non-syndromic child would lie within 1:96 2 ð1 qÞ ¼ 1:24 standard
intellectual disability stands in contrast to the cognitive deviations. This means that despite such a ‘high’ correlation
decline of children with specific syndromes such as Fragile one may still observe a difference of up to 1.24915=18.6
X, Down syndrome, or Williams syndrome3–7 where a pro- IQ points between two repeated measurements (Whitaker
gressive IQ decline has been consistently reported. The IQ actually found that 14% of children had scores that chan-
drop in these clinical populations across age may be ged by more than 10 points10).
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Book Review: Central Nervous System Infections in Seventeen disease-/syndrome-specific chapters by a range
Childhood of experts form the bulk of the work, each providing a con-
Edited by Pratibha Singhi, Diane E Griffin and Charles R Newton cise, up-to-date summary of one or more important CNS
International Review of Child Neurology Series infections, with in each case appropriate attention paid to
London: Mac Keith Press, 2014
£95.00 (Hardback) pp 378
practical matters of diagnosis and specific management.
ISBN: 978-1-909962-44-6 Notable among many excellent, well-referenced contribu-
tions are Whitley’s presentation of a lifetime of experience
in his chapter on herpes simplex CNS infection; Kumar and
The burden of central nervous system (CNS) infections Singh’s, and Kanta’s chapters on Japanese B encephalitis and
in children – and particularly in children in resource poor rabies; the Singhis’ expositions of their wealth of experience
settings – is staggeringly high. Doctors caring directly for of the management of bacterial and fungal brain infections,
acutely ill children, and neurologists from whom special- neurocysticercosis and the life-threatening bacterial exo-
ized opinions are sought, need a practical resource to toxin-mediated syndromes; Schoeman and van Toorn’s
keep abreast of developments in this fast-moving field. authoritative summary of CNS pathology caused by tuber-
This was attested to in a poll of members of the Interna- culosis, with their starkly juxtaposed reminders that while
tional Child Neurology Association that identified the early diagnosis and treatment is usually completely curative,
topic of children’s CNS infections as high on their wish in most settings (resource poor and rich alike) diagnosis is
list for an up-to-date textbook. In Central Nervous System long delayed and disability or death are common outcomes;
Infections in Childhood, the team of Singhi, Griffin, and and Sladky and Willison’s comprehensive discussion of
Newton have, in my opinion, produced a valuable, acute disseminated encephalomyelitis, Guillain-Barre syn-
scholarly resource for paediatricians and paediatric neurol- drome, and other post-infectious neurological disorders.
ogists. All three editors are eminent in the field compris- The editors’ intention is that an electronic version of the
ing that small zone of trisection in the Venn diagram book, available online, will be periodically updated, when
that represents the bodies of knowledge making up paedi- no doubt – for example – the dramatic impact of Meningo-
atrics, infectious diseases, and neurology. As well as coccal A conjugate vaccine (MenAfriVac), developed for
bringing a wealth of practical clinical experience and use in sub-Saharan Africa’s meningitis belt, can be pre-
complementary research to their project, they have sented in some detail. In this way too perhaps one unac-
recruited an ‘A list’ of international contributors, each countable omission can soon be addressed: perhaps
with personal experience and expertise in the areas of representing the triumph of hope for the eradication of
their contributions, to produce a remarkably well-bal- measles by vaccination over the general experience of fal-
anced multi-author textbook. tering progress, there is at present no chapter on this terri-
Scene-setting chapters provide a background in epidemi- bly important viral infection of the brain in this otherwise
ology, bacterial and viral pathogenesis, general principles of comprehensive book.
the management of CNS infections and their acute compli- At close to half the length of Bell and McCormick’s
cations, and neuroimaging, with a further useful contribu- Neurologic Infections in Children (2nd edition, 1981; digi-
tion by Neville on the important topic of febrile seizures. tized, 2008), Central Nervous Systems Infections in Childhood
Khandelwal and Rumboldt’s chapter on neuroimaging, with nevertheless provides a valuable, well-referenced textbook
its emphasis on magnetic resonance imaging (MRI), is per- for paediatricians and neurologists everywhere, and, with
haps inevitably presented in highly technical language. It regular updates, has every chance of becoming an endur-
reminds the reader of the rapid, transformative impact on ing, standard text.
neurology of computed tomography and MRI, and fore-
shadows exciting technological developments to be antici- J Simon Kroll
pated with, for example, diagnostic magnetic resonance Department of Medicine, Imperial College London, London, UK.
spectroscopy. The chapter represents a valuable primer text
to assist discussion with neuroradiologist colleagues. doi: 10.1111/dmcn.12586