Вы находитесь на странице: 1из 7


RESEARCH REVIEWS 15: 28 34 (2009)


Lianne H. English,1 Marcia A. Barnes,2* Heather B. Taylor,2 and Susan H. Landry2
Department of Psychology, University of Guelph, Ontario, Canada
Childrens Learning Institute, Department of Pediatrics, University of Texas Health Science Center at Houston, Houston, Texas

Spina bifida (SB) is a neural tube defect diagnosed before or at lus, a buildup of cerebral spinal fluid in the brain that impedes
birth that is associated with a high incidence of math disability often myelination and damages gray matter, particularly in posterior
without co-occurring difficulties in reading. SB provides an interesting
population within which to examine the development of mathematical
brain regions. Shunting for hydrocephalus is required in many
abilities and disability across the lifespan and in relation to the deficits in children with SB. The most severe form of this disorder is
visual-spatial processing that are also associated with the disorder. An called SB myelomeningocele. It is characterized by a lesion
overview of math and its cognitive correlates in preschoolers, school-age though which the spinal cord protrudes, which can vary in
children and adults with SB is presented including the findings from a location along the spine. The severity and nature of ambula-
longitudinal study linking early executive functions in infancy to the de-
velopment of later preschool and school age math skills. These findings tory and urinary complications vary depending on the level of
are discussed in relation to socio-historical perspectives on math educa- this lesion. Higher level lesions are related to poorer out-
tion and implications for intervention and directions for further research comes, often resulting in difficulties with self-generated loco-
are presented. ' 2009 Wiley-Liss, Inc.
motion and bladder control, and also worse outcomes in some,
Dev Disabil Res Rev 2009;15:2834.
but not all, aspects of cognition [Fletcher et al., 2004].
Neurodevelopmental outcomes across a number of
Key Words: spina bifida; math across life span; longitudinal research domains for individuals with SBM have recently been linked
to a small number of core deficits tied to the primary brain
dysmorphologies of SBM that are evident from birth, persist
throughout the lifespan, and result in a combination of spared
and deficient processing within domains as diverse as motor

pina bifida (SB) is a congenital neurodevelopmental dis-
order identified during gestation or at birth that is asso- function, perception, language, reading, and mathematics
ciated with high rates of math difficulties by school-age [Dennis et al., 2006]. Dennis et al. make a distinction between
often in the context of adequate development of general cog- stipulated processing, which involves performance that is auto-
nitive abilities and word reading. As such, SB is a useful disor- matically activated and established through associations and
der for investigating how and why children develop problems repetition, and constructed processing, which relies on the
with math and for studying some of the early developmental integration of information from various sources and on-line
precursors of later emerging disabilities in mathematics. This adjustments of performance. The former type of processing is
article begins with an overview of SB: its epidemiology, relatively intact in individuals with SBM, who show strengths
pathophysiology, and a model of neurocognitive functioning in activation of stipulated representations including the ability
that serves to organize findings across diverse cognitive out- to recognize faces, perceive objects from degraded visual cues
comes including math. We then discuss ways in which this [Dennis et al., 2002], retrieve small math facts (e.g., 2 3 5
disorder has been used to understand mathematical ability and 5) from memory [Barnes et al., 2006], and read words and
disability by using a life span approach that: (1) considers the access word meanings [Barnes and Dennis, 1992; Barnes et al.,
natural course of mathematical abilities and their cognitive 2004b]. The latter type of processing is consistently deficient
correlates in preschoolers, school-age children, and adults with as seen in shifting between perceptual representations [Dennis
SB; and (2) investigates potential developmental precursors of et al., 2002], performance on larger sum computations whose
later developing mathematical skills using longitudinal meth- answers are not reliably retrieved from semantic memory (e.g.,
ods. Possible implications for intervention and directions for
future research are outlined.

SB: AN OVERVIEW Grant sponsor: National Institute for Child Health and Development; Grant num-
SB is a neural tube defect that results from complex bers: P01HD35946, R01HD046609-04; Grant sponsor: Canadian Institutes of
gene-environment interactions. It is the most common dis- Health Research.
*Correspondence to: Marcia Barnes, The University of Texas Health Science Cen-
abling birth defect in North America, affecting the develop- ter, Department of Pediatrics, Childrens Learning Institute, 7000 Fannin, Suite
ment of both spine and brain. SB occurs in 0.30.5 of every 2431, Houston, TX 77030. E-mail: Marcia.barnes@uth.tmc.edu
1,000 live births [Williams et al., 2005]. Atypical neuroem- Received 7 November 2008; Accepted 17 December 2008
Published online in Wiley InterScience (www.interscience.wiley.com).
bryogenesis affects the corpus callosum, midbrain and tectum, DOI: 10.1002/ddrr.48
and cerebellum. Further complications arise from hydrocepha-
' 2009 Wiley -Liss, Inc.
8 7) [Barnes et al., 2006], and inte- and touching items may support the Longitudinal Study of Working
grating information within text to spec- development of one-to-one correspon- Memory/Inhibitory Control in
ify meaning [Barnes et al., 2007]. dence knowledge [Alibali and DiRusso, Infants and Mathematical
In the next section we present 1999]. Deficits in fine motor skills and Outcomes in Preschoolers and
studies on math in children with SB finger function and precision in upper School-Age Children
that illustrate the ways in which SB has limb control that are evident in infants Longitudinal investigations pro-
been used to: investigate the cognitive and toddlers with SB [Lomax-Bream vide a unique opportunity for examin-
correlates and consequences of math et al., 2007] could place early con- ing the cognitive underpinnings of later
difficulties from a life span perspective; straints on those aspects of counting and developing math abilities and impair-
test models of mathematical learning simple arithmetic that are supported by ments. A few recent longitudinal studies
disability (MLD) subtypes; and study fluid and accurate finger and fine motor of math in typically developing children
whether growth in particular cognitive skills. Coupled with gross and fine have focused on prediction of school-
abilities is related to mathematical out- motor restrictions, difficulties in visual age math abilities from preschool cogni-
comes in the preschool and early ele- attention (specifically in attention orien- tive processes. For example, these stud-
mentary school years. tation involving attention to and disen- ies have investigated the relation of
gagement from salient environmental domain general abilities such as working
LIFE SPAN STUDIES OF stimuli) that are present from infancy memory and inhibitory processing [Blair
MATHEMATICAL PROCESSING [Taylor et al., in press] and that are and Razza, 2007; Mazzocco and Kover,
IN SB: COGNITIVE related to midbrain dysmorphology 2007; Bull et al., 2008] or phonological
CORRELATES, LONGITUDINAL [Dennis et al., 2005a,b] mean that very processes [Hecht et al., 2001] and do-
PERSPECTIVES, AND young children with SB do not effi- main-specific abilities such as number
LONG-TERM CONSEQUENCES ciently explore space during a time of sense [Locuniak and Jordan, 2008] in
preschoolers to later math outcomes in
Infants and Toddlers with SB kindergarten and the early primary
The mathematical processing dif- grades. One of the advantages afforded
ficulties of individuals with SB are evi- The mathematical by a neurodevelopmental disorder diag-
dent across the life span. As early as 36 nosed at or before birth and associated
months of age, preschoolers with SB processing difficulties of with a high rate of school-age math dis-
have less well developed understanding ability, is that there is an opportunity to
of one to one correspondence, are less
individuals with Spina investigate growth in particular cogni-
skilled at rote counting, and are less able bifida are evident across tive domains in infancy and relate them
to match on the basis of quantity than to later academic outcomes. The first
their typically developing peers [Barnes the life span. Math study, to our knowledge, to examine
et al., 2005]. Fine motor and visual- deficits in Spina bifida the cognitive precursors of later devel-
spatial skills uniquely contribute to oping math skills as early as infancy and
these emerging math abilities, with fine . . . are pervasive, to follow the same children through the
motor skill as measured by a task of fine persisting into adulthood preschool years and into the early
motor dexterity (placing small grooved school years, is a collaborative and mul-
pegs into a pegboard) related to count- and adversely impact tidisciplinary project involving the Uni-
ing concepts and visual-spatial skill
related to quantity comparison. These
quality of life. versity of Texas Health Science Center
at Houston and Toronto Hospital for
findings suggest that the difficulties in Sick Children. English et al. [in press]
mathematical processing can be dis- evaluated whether infant executive
cerned in very young preschoolers with functions predicted an array of pre-
SB, certainly before the onset of any school and school-age math skills.
formal schooling, which has implica- rapid development of visually guided Although there is no consensus regard-
tions for early intervention and preven- behavior. Considering the importance ing an accurate definition of executive
tion. Moreover, the data also suggest of the coordination of visually guided functions, many models highlight the
that distinct mathematical abilities, even motor skills such as reaching and explo- central importance of both working
in the preschool years, may be sup- ration of the environment for cognitive memory and inhibitory control. Work-
ported by somewhat different cognitive development, early restrictions in these ing memory is the ability to hold infor-
and motor competencies. However, the aspects of development could limit sub- mation on-line while engaging in si-
developmental mechanisms by which sequent problem-solving and visual multaneous mental processes. Inhibitory
deficits in fine motor skills and visual- spatial capabilities [Thelen and Smith, control is the ability to suppress prepo-
spatial abilities might come to affect 1994], skills that have been hypothe- tent responses in favor of subdominant
early math skills such as counting and sized to be important for mathematical responses. A delayed response task, pur-
quantity comparison have not been cognition [Rourke, 1993; Assel et al., ported to measure these two constructs
well-studied in this population or in the 2003]. These potential early develop- [Diamond and Doar, 1989], was admin-
typically developing population. It has mental pathways to the evolution of dif- istered to infants three times between
been proposed that in typically develop- ficulties in math in SB might be fruitful 12 and 26 months. The nature of this
ing children across cultures there is a avenues for future longitudinal research task makes it difficult to discern the
link between fingers and counting and investigations in both children with SB unique contributions of working mem-
simple arithmetic problem solving and in typical development. One exam- ory and inhibitory control. However,
[Fayol et al., 1998; Butterworth, 1999; ple of such an approach is presented failures of working memory/inhibitory
Noel, 2005], and also that pointing to below. control are inferred when the child
Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL. 29
searches under a cup that the reward (in working memory and inhibition, may explanations stipulate that a specific
this case, a Cheerio) was hidden under be instrumental for the development of number-processing deficit underscores
in the previous trial even though they math proficiencies. These findings are math difficulties [Butterworth, 1999].
subsequently saw the examiner place consistent with those from other recent According to this view, humans and
the reward under the other cup. studies and with some views on the nonhuman animals are endowed with
Latent growth curve modeling relation between executive skills and the ability to understand quantities and
(LGM) was used to assess change in math. It has been found that working relative numerosities; mathematical abil-
infant executive functions and to deter- memory is related to certain types of ities develop from the innate number
mine whether initial level and growth mathematical problem solving skills in module. This view is supported by
in these early executive functions pre- typically developing preschoolers research suggesting that young infants
dicted later developing preschool and [Bisanz et al., 2005; Klein and Bisanz, demonstrate an understanding of small
school-age math skills. LGM is a flexi- 2000]. Blair and Razza [2007] found a numerosities and simple arithmetic
ble technique that offers various benefits similarly strong relation between mea- [Starkey et al., 1990; Wynn, 1992] and
compared to more traditional statistical sures of inhibitory control, effortful that children with MLD are particularly
techniques such as repeated-measures control, and math knowledge in a error prone on tasks that tap the mental
ANOVA. LGM can retain missing data group of high-risk children attending representation of number. The fact that
by computing maximum likelihood kindergarten. Working memory and in- working memory deficits are not uni-
estimates. LGM can also simultaneously hibitory control have also been found formly found in samples of children
evaluate growth in a construct as a to predict performance on math word with MLD is also used to argue against
dependent variable and as a predictor problems [Espy et al., 2004]. Children the domain general view [Butterworth
of subsequent outcomes. Two change with severe MLD have difficulties with and Reigosa, 2007].
parameters are evaluated in a growth cognitive tasks that tap working mem- Results from our longitudinal
curve model: intercept and slope. Inter- investigation support hypotheses about
cept is the mean performance on a task the importance of certain cognitive sys-
and slope is the mean rate of change in tems such as working memory/inhibi-
a construct over time. Results from our tory control in the development of
In general, working memory/in- math abilities, particularly when con-
hibitory control predicted performance longitudinal investigation trasted with the development of read-
on a range of informal math outcomes
at 60 months of age such as counting
support hypotheses about ing, but they are unable to address the
question of whether domain-general
and object-based addition and subtrac- the importance of certain skills such as working memory and in-
tion [based on Jordan et al., 1992]. Per- cognitive systems such as hibitory control are primarily causal in
formance on standardized tests of single the development of mathematical ability
and multidigit arithmetic at 7.5 years of working memory/ and disability. From a developmental
age was also predicted by level and perspective, there are different possible
growth in infant working memory/in-
inhibitory control in the pathways to math development that
hibitory control. Interestingly, compara- development of math could explain how and when executive
ble trends were not as robustly observed functions are salient. According to
for reading outcomes, particularly not abilities, particularly Zelazo et al. [2003], the knowledge of
at school-age. These different patterns when contrasted with the basic rule structures and problem com-
suggest that there may be discrepant ponents allows for the development of
pathways to math and reading develop- development of reading. . . complex executive abilities. In other
ment. It is possible that reading places pathway models, inhibitory control
only developmentally limited demands facilitates academic skill consolidation
on the executive system. Unlike math, above the influence of basic knowledge
reading becomes less effortful and more ory and some aspects of their mathe- representations [Diamond et al., 2007].
automatic over time; although some matical performance are either fully or The combination of these two hypothe-
aspects of math (e.g., access to math partially mediated by working memory ses leads to a more circular model of
facts and procedures in multidigit arith- [e.g., Geary et al., 2007]. math development. The early matura-
metic) may become more fluent with Domain-general theories of math- tion of executive functions, promoted
experience, many aspects of math ematical development and disability by environmental and genetic influences
require new learning [LeFevre, 2000]. stipulate that certain core cognitive sys- [Friedman et al., 2008], could enhance
Therefore, reading may only engage the tems are foundationally involved in the the early learning of basic mathematical
executive system when knowledge is emergence of math competencies. skills (e.g., counting, small sum arith-
being acquired and may also figure in These systems include: the central exec- metic). This increasingly solidified
the performance of older children with utive, which involves attentional and in- knowledge of math rule structures
reading disabilities, who have often not hibitory capacities; the language system, could facilitate the development of
automatized basic sound symbol corre- which is important for representing and more advanced executive functions. In
spondences. In contrast, math becomes manipulating mathematical knowledge turn, these executive abilities may allow
increasingly complex as children get for storage and retrieval; and the visuo- children to understand more complex
older and places considerably higher spatial system, which is responsible for mathematical concepts. Breakdowns in
demands on executive processes. understanding visual and spatial infor- various stages along this circular path
The results from our longitudinal mation [Geary et al., 2007]. In contrast could lead to math deficits and perpetu-
study on SB and learning suggest that to more domain-general theories of ate a cycle of academic underachieve-
specific cognitive processes, namely math development, domain-specific ment. Alternatively, some integrative
30 Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL.
models suggest that a core number and 22q11 deletion syndrome (see other tic and used less mature strategies in
module interacts with domain general articles in this volume). solving these problems than their typi-
cognitive abilities to facilitate mathe- One subtyping model of MLD cally developing peers. Our data suggest
matical skill development [Spelke and [Geary, 1993; 2004] proposes possible that efficiency of math fact retrieval
Kinzler, 2007]; presumably, breakdowns different pathways for MLDs depending may be a marker of computational skill
in either the core number module or on the presence/absence of RD. Chil- across the general population, and not
domain general cognitive abilities or dren with both MLD and RD may simply a marker of MLD [see Barnes
their interaction could affect mathemat- have difficulties in phonological work- et al., 2006]. That is, math fact retrieval
ical learning. ing memory that make it difficult to as- skills may be normally distributed in the
An alternative explanation for the sociate number combinations (e.g., 2 population much like phonological
overlap between executive functions 5) with their answers (7) to facilitate awareness is for reading. However,
and math has its roots in recent socio- direct retrieval of math facts (2 5 5 unlike phonological awareness and RD,
historical trends. Historically, during the 7). The math problems of children with we make no claim about causal connec-
early 1900s in the US, mathematics was MLD-only have been variously tions between math fact retrieval and
not offered to children in the early pri- hypothesized [Geary, 1993; Rourke, MLD.
mary grades. At the turn of the 20th 1993] to be related to difficulties in the Although children with MLD and
century, mathematics instruction for the spatial representation and manipulation SB, regardless of reading status, were
upper elementary grades emphasized of quantities, similar to that of adults less accurate and slower on single digit
rote memorization and drills. Early ele- with acquired brain injuries with spatial arithmetic problems than were children
mentary school mathematics textbooks dyscalculia [Hartje, 1987] or to difficul- with SB and no learning disability and
consisted predominantly of written ties in learning procedures including typically developing age peers, there
prose, with little exposure to diagrams counting strategies in single digit arith- were a few differences between the
or symbols. However, by the 1960s, a metic and regrouping in multidigit groups related to reading status: the
comprehensive mathematics curriculum arithmetic. The fact that a large propor- MLD-only group used more direct re-
had been established for all primary tion of children with SB have MLD trieval and solved small sum problems
grades. Advanced cognitively-based and co-occurring deficits in visual- (sums less than 10) more quickly than
math areas, like geometry, started to be spatial processing make this disorder of the MLDRD group. These findings
introduced to younger children. Cur- some interest to such subtyping models are consistent with the hypothesis that
rently, math curricula continue to pro- of MLD. Studies on both single and children with MLDRD show less
gressively emphasize visual-spatial con- multidigit arithmetic in children with direct retrieval of math facts because
cepts and executive thinking abilities SB are presented below. deficits in phonological working mem-
[Blair et al., 2005]. These problems ory may disrupt the ability to learn the
(e.g., pattern completion) required mul- Single Digit Arithmetic associations between addends and
tiple operations and cognitive skills such The most well studied aspect of answers [Geary, 1993, 2004].
as working memory. This increased uti- math in children with and without
lization of cognitive executive abilities, MLD, including children with SB, is Multidigit Arithmetic
early in a childs life, likely leads to arithmetic. Children with SB who have Those children with SB and
enduring changes in both cognitive and MLD whether they have MLD-only or MLD-only who also have deficits in
academic functioning. It is therefore MLDRD are slower and less accurate visual-spatial skills related to their brain
possible that children are becoming on single digit arithmetic problems dysmorphology could be considered an
more dependent on processes such as (e.g., 4 3) even when they are ideal test of the hypothesis that there is
working memory and inhibitory control directly retrieving the answer from a neurodevelopmental variant of the
to consolidate mathematical knowledge memory (e.g., I just know that 4 3 type of visual-spatial dyscalculia that is
and to solve novel problems. 5 7) and they use less developmen- sometimes seen in adults with acquired
tally-mature counting strategies such as brain injuries. One of the ways in
School-Aged Children with SB the min strategy (e.g., 4 5 5 5, 6, 7, which visual-spatial dyscalculia has been
Using low-achievement cut-offs 8, 9) on more problems compared to studied in adults [Hartje, 1987] and
(less than the 25th percentile), 25% of other children their age [Barnes et al., anecdotally reported in some children
children with SB have MLD with no 2006]. These findings converge with with MLD-only [Rourke, 1993] is to
co-occurring RD (MLD-only), whereas those from other studies that identify see whether these individuals make vis-
only 3% have RD without a co-occur- difficulty in math fact retrieval as a core ual-spatial errors when solving written
ring MLD; just over 20% have both deficit in children with MLD [Jordan multidigit arithmetic problems. These
MLDRD and this combination of et al., 2003] and furthermore, suggest include errors related to misreading and
learning disabilities is often associated that difficulties in math fact retrieval miswriting of numbers, the crowding of
with socio-economic disadvantage occur in children with MLD regardless written work, and the like. However,
[Fletcher et al., 2004]. The high rate of of the presence/absence of frank neuro- on multidigit subtraction tasks, children
MLD-only is much higher than that in logical insult or reading status. Children with SB and MLD-only do not commit
the general population [Shalev, 2007], with SB and no learning disability had more visual-spatial or visual monitoring
which makes SB ideal for comparing arithmetic abilities that were average, errors; that is, they were not more
the consequences and characteristics of but significantly lower than those of prone than any other group to misread
MLD with and without co-occurring their typically developing peers, while or miswrite numbers, to crowd their
RD. Math difficulties in the absence of the pattern for word reading was just written work, or to make errors due to
reading deficits are also apparent in the opposite. Interestingly, these chil- misalignment of numbers in columns
other genetic disorders, including dren with SB and no learning disabil- [Ayr et al., 2005; Barnes et al., 2006].
Turner syndrome, Fragile X syndrome, ities were slower in single digit arithme- Nor do visual-spatial errors characterize
Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL. 31
the performance of children with SB related to visual-spatial abilities in this the intervention [Fletcher et al., 2007].
on other operations such as multidigit population as it is in typically develop- Effective programs that explicitly
multiplication and long division [Barnes ing children [Barnes et al., 2002]. instruct mathematical content have been
et al., 2002]. Their errors in multidigit reported for both preschool and school-
arithmetic are mainly procedural in na- Adults with SB age children [reviewed in Fletcher
ture reflecting less well developed con- Math concepts and numerosity et al., 2007; Griffin, 2007], and there is
ceptual and procedural arithmetic understanding are similarly compro- every reason to think that such pro-
knowledge (e.g., understanding of base mised in many adults with SB grams would also be effective for chil-
10 and difficulties borrowing). In all, [Hommet et al., 1999]. Research by dren with neurodevelopmental disorders
the findings provide no evidence for Dennis and Barnes [2002] found that that particularly affect the development
the idea that MLD without RD repre- young adults with SB had difficulties of mathematical skills. Given the high
sents a subtype of MLD that has it ori- with computation accuracy and speed, rates of MLD in SB and some of the
gins in impairments in the spatial repre- problem solving and functional numer- other neurodevelopmental disorders
sentation and manipulation of quantity acy involving the ability to apply nu- presented in this volume, a focus on
or number. merical information to everyday situa- early intervention and prevention may
Correlational studies tell a similar tions such as making price comparisons, hold some promise; early intervention
story, namely, that visual-spatial abilities dealing with the value of coins, banking seems particularly relevant in light of
are not significantly related to multidigit and budgeting, and time concepts. Fur- the findings on persistent deficits in
arithmetic performance in children with thermore, data on a subgroup of adults math that interfere with functional out-
SB on either standardized or experi- who had been tested as children showed comes in adults with SB. However, one
mental tests of either single or multidi- that the difficulties in math are persis- of the questions that could be posed by
git arithmetic [Ayr et al., 2005; Barnes tent across time; those individuals who domain general theories of mathemati-
et al., 2006]. In contrast, like counting scored low on an oral math problem cal development and disability and by
and counting knowledge in young solving task (the Arithmetic subtest of some of the findings reviewed above, is
preschoolers, multidigit arithmetic in the Wechsler Intelligence Scale for whether there is any role for training of
school-age children with SB is related Children) had considerable difficulty in executive functions and attention either
to fine motor skills [Barnes et al., similar types of math problem solving alone or in combination with explicit
2005]. These findings are consistent and functional numeracy as adults. The mathematical content for preventing
with neuropsychological studies of severity of these limitations was affected mathematical difficulties or for improv-
typically developing children showing by working memory for numbers and ing mathematical processing in children
that early fine motor skills predict later lifetime number of shunt-revisions. at risk for MLD.
achievement in mathematics [Fayol Functional numeracy impairments lim- Some studies suggest that regula-
et al. 1998]. Although such findings are ited these adults ability to successfully tory abilities and executive skills such as
interesting, causal connections between complete everyday tasks related to gro- inhibitory control, working memory,
the development of finger skills and cery shopping, telling time, cooking, and behavioral aspects of attention may
math skills have not been established, and banking. Moreover, to a greater be systematically improved through
nor is there solid evidence for the hy- extent than functional literacy, which training in preschoolers [Dowsett and
pothesis that fine motor and math skills was intact in these adults [Barnes et al., Livesey, 2000; Diamond et al., 2007],
come to share common representational 2004a], functional numeracy was related and in school-age children with atten-
systems and neural substrates in parietal to self-reported levels of social and per- tion deficit/hyperactivity disorder
lobe through their developmental links sonal autonomy. These results suggest [Klingberg et al., 2005]. Although cor-
[Butterworth, 1999]. Nonetheless, these that math deficits in SB are not the relations between performance on some
findings point to ways in which theories result of a developmental lag that inde- executive function tasks and math were
from across the broad spectrum of psy- pendently resolves. Rather, these deficits shown in the Diamond et al., study, the
chologyin this case, neuropsycholo- are pervasive, persisting into adulthood study design did not permit one to cau-
gymay be relevant to understanding and adversely impact quality of life. sally link the intervention with aca-
MLD. That the pathophysiology of SB Considering the importance of mathe- demic achievement. These findings sug-
includes thinning of parietal lobes matical competence for quality of life, gest that specific training programs can
points to potential avenues for brain- adaptive functioning, and employment result in generalized improvements in
behavior research on mathematical de- success [Hetherington et al., 2005], it is cognitive domains such as executive
velopment in this disorder. clear than early and persistent math skills and attention, which are related to
Although children with SB have impairments can have significant long- academic achievement, particularly in
difficulties with arithmetic, within the term consequences for individuals with math. However, it is unknown whether
broad domain of mathematics, their SB. such programs: (1) result in training
arithmetic abilities and basic number effects that persist over time; (2) when
knowledge are better developed than IMPLICATIONS FOR used alone could lead to improved math
other aspects of mathematics such as ge- INTERVENTION AND AVENUES outcomes, and if so, at what ages and
ometry, estimation, and word problem FOR FUTURE RESEARCH for what types of children; (3) when
solving. These aspects of mathematics One of the general rules of used in combination with explicit
draw to a greater extent on the neuro- research on interventions for children mathematical content could lead to
cognitive weaknesses of individuals with with academic disabilities is that gains improved math outcomes over and
SB including difficulties in manipulating are specific to what is taught; in other above what is reported for those math
visual-spatial representations [Barnes words, math and reading will not programs alone. Conversely, one might
et al., 2002], and performance in these improve unless explicit teaching of math ask whether effective preschool and
domains of mathematics is, in fact, and reading content are a large part of school-age math interventions contain
32 Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL.
components that, in effect, train exec- ematical cognition, but also to broader Blair C, Razza RP. 2007. Relating effortful con-
utive functions and attention in addition questions of plasticity and neural reor- trol, executive function, and false belief
understanding to emerging math and literacy
to providing mathematical content. For ganization. Finally, SB represents a ability in kindergarten. Child Dev 78:
example, several successful math pro- potentially interesting population in 647663.
grams for children with MLD incorpo- which to study the behavioral and neu- Bull R, Espy KA, Wiebe SA. 2008. Short-term
rate motivators to help students regulate ral consequences of early intervention memory, working memory, and executive
their attention and behavior [reviewed for mathematical difficulties. n functioning in preschoolers: longitudinal
predictors of mathematical achievement at 7
in Barnes et al., in press]. years. Dev Neuropsychol 33:205228.
As mentioned previously, SB is Butterworth B. 1999. What counts: how every
associated with brain dysmorphologies brain is hardwired for math. New York: The
that may be particularly implicated in REFERENCES Free Press.
mathematical processing. Thinning Alibali MW, DiRusso AA. 1999. The function of Butterworth B, Reigosa V. 2007. Information
gesture in learning to count: more than processing deficits in dyscalculia. In: Berch
often occurs in the posterior regions of keeping track. Cogn Dev 14:3756. DB, Mazzocco MM, editors. Why is math
the brain, namely the parietal and occi- Assel MA, Landry SH, Swank P, et al. 2003. Pre- so hard for some children? Baltimore:
pital cortices, of individuals with SB cursors to mathematical skills: examining the Brooks Publishing Company, p 6581.
[Fletcher et al., 1996]. The parietal cor- roles of visual-spatial skills, executive pro- Dehaene S, Piazza M, Pinel P, et al. 2003. Three
tex, in particular, is considered to be cesses, and parenting factors. Appl Dev Sci parietal circuits for number processing.
7:2738. Cogn Neuropsychol 20:487506.
important for several aspects of mathe- Ayr LK, Yeates KO, Enrile BG. 2005. Arithmetic Dehaene S, Spelke E, Pinel P, et al. 1999. Sources
matical processing. For example, circuits skills and their cognitive correlates in chil- of mathematical thinking: behavioral and
in parietal lobe are implicated in mathe- dren with acquired and congenital brain dis- brain-imaging evidence. Science 284:
matical function and dysfunctiona order. J Int Neuropsychol Soc 11:249262. 970974.
bilateral intraparietal system for core Barnes MA, Dennis M. 1992. Reading in chil- Dennis M, Barnes MA. 2002. Math and numer-
dren and adolescents after early onset hydro- acy skills in young adults with spina bifida
quantitative processing, a region of the cephalus and in their normally developing and hydrocephalus. Dev Neuropsychol 21:
left angular gyrus for verbal processing age-peers: phonological analysis, word rec- 141155.
of numbers, and a posterior superior ognition, word comprehension, and passage Dennis M, Edelstein K, Copeland K, et al. 2005a.
parietal system for mathematical proc- comprehension skill. J Pediatr Psychol 17: Space-based inhibition of return in children
essing such as estimation that may 445456. with spina bifida. Neuropsychol 19:456465.
Barnes MA, Dennis M, Hetherington R. 2004a. Dennis M, Edelstein K, Copeland, K et al. 2005b.
require spatial attention [Dehaene et al., Reading and writing skills in young adults Covert orienting to exogenous and endoge-
1999; 2003]. It is unknown whether with spina bifida and hydrocephalus. J Int nous cues in children with spina bifida.
similar relations between damage to pa- Neuropsychol Soc 10:680688. Neuropsychologia 43:976987.
rietal lobe and math hold for individuals Barnes MA, Faulkner H, Wilkinson M, Dennis Dennis M, Fletcher JM, Rogers S, et al. 2002.
with a neurodevelopmental disorder like M. 2004b. Meaning construction and inte- Object-based and action-based visual per-
gration in children with hydrocephalus. ception in children with spina bifida and
SB in which the neural substrate is Brain Lang 89:4756. hydrocephalus. J Int Neuropsychol Soc 8:
damaged very early in life before the Barnes MA, Fuchs LS, Ewing-Cobbs L. Neuro- 95106.
development of mathematical abilities. psychological and cognitive approaches to Dennis M, Landry SH, Barnes M, et al. 2006. A
As mentioned earlier, dysmorphology in mathematical disabilities. In: Yeates K, Taylor model of neurocognitive function in spina
structures such as the midbrain or pari- G, Ris D, et al., editors. Pediatric neuropsy- bifida over the life span. J Int Neuropsychol
chology: research, theory, and practice, 2nd Soc 12:285296.
etal lobe might also affect the acquisi- ed. New York: Guilford Publications (in Diamond A, Barnett S, Thomas J, et al. 2007.
tion of mathematical skills through their press). Preschool program improves cognitive con-
effects on the development of attention Barnes MA, Huber J, Johnston AM, et al. 2007. trol. Science 317:14871388.
and visual exploration, fine motor skills, A model of comprehension in spina bifida Diamond A, Doar B. 1989. The performance of
reaching and pointing and the like. meningomyelocele: meaning activation, inte- human infants on a measure of frontal cortex
gration, and revision. J Int Neuropsychol function, the delayed response task. Dev
How some of these core motor and Soc 13:854864. Psychobiol 22:271294.
cognitive deficits in SB that are related Barnes MA, Smith-Chant B, Landry SH. 2005. Dowsett SM, Livesey DJ. 2000. The development
to the primary dysmorphologies of the Number processing in neurodevelopmental of inhibitory control in preschool children:
disorder and that are present from birth disorders: spina bifida myelomeningocele. In: effects of executive skills training. Dev
affect the development of mathematical Cambell JD, editor. Handbook of mathemat- Psychobiol 36:161174.
ical development. New York: Psychology English L, Barnes MA, Landry SH. Executive
abilities from infancy and across the life- Press. p 299313. function development in spina bifida and its
span would be of interest. Barnes MA, Pengelly S, Dennis M, et al. 2002. influence on preschool and school age aca-
Functional imaging studies are Mathematics skills in good readers with hy- demic outcomes. J Int Neuropsychol Soc (in
currently underway in individuals with drocephalus. J Int Neuropsychol Soc 8: press) (abstract).
SB that should help identify particular 7282. Espy KA, McDiarmid MM, Cwik MF et al.
Barnes MA, Wilkinson M, Khemani E, et al. 2004. The contribution of executive func-
neural networks that are implicated in 2006. Arithmetic processing in children with tions to emergent mathematic skills in pre-
different aspects of math functioning spina bifida: calculation accuracy, strategy school children. Dev Neuropsychol 26:
such as calculation and estimation in use, and face retrieval fluency. J Learn Disa- 465486.
this neurodevelopmental disorder. bil 39:174187. Fayol M, Barrouillet P, Marinthe, C. 1998. Pre-
Comparisons with other neurodevelop- Bisanz J, Sherman JL, Rasmussen C, et al. 2005. dicting arithmetical achievement from
Development of arithmetic skills and knowl- neuro-psychological performance: a longitu-
mental disorders at high risk for MLD edge in preschool children. In: Campbell JID, dinal study. Cognition 68:B63B70.
and in children with MLD without editor. Handbook of mathematical cognition. Fletcher JM, Bohan TP, Brant ME, et al. 1996.
neurodevelopmental disorder would New York: Psychology Press. p 143162. Morphometric evaluation of the hydroce-
provide knowledge about whether the Blair C, Gamson D, Thorne S, et al. 2005. Rising phalic brain: relationships with cognitive
neural signature of MLD varies across mean IQ: changing cognitive demand of development. Childs Nerv Syst 12:
mathematics education for young children, 192199.
populations even when the behavioral population exposure to formal schooling and Fletcher JM, Dennis M, Northrup H, et al. 2004.
phenotype looks remarkably similar. the neurobiology of the prefrontal cortex. Spina bifida: genes, brain, and development.
Such studies would be relevant to math- Intelligence 33:93106. In: Glidden LM, editor. International review

Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL. 33

of research in mental retardation. San Diego: Hetherington R, Dennis M, Barnes MA, et al. Mazzocco MM, Kover ST. 2007. A longitudinal
Elsevier Academic Press. p 63117. 2005. Functional outcomes in young adults assessment of executive function skills and
Fletcher JM, Lyon GR, Fuchs LS, et al. Learn- with spina bifida and hydrocephalus. Childs their association with math performance.
ing disabilities: from identification to Nerv Syst 22:117124. Child Neuropsychol 13:1845.
intervention. New York: Guilford Press, Hommet C, Billard C, Gillet P, et al. 1999. Neu- Noel M. 2005. Finger gnosia: a predictor of nu-
2007. ropsychologic and adaptive functioning in merical abilities in children? Child Neuro-
Friedman NP, Miyake A, Young SE, et al. 2008. adolescents and young adults shunted for psychol 11:413430.
Individual differences in executive functions congenital hydrocephalus. J Child Neurol Rourke BP. 1993. Arithmetic disabilities, specific
are almost entirely genetic in origin. J Exp 14:144150. and otherwise: a neuropsychological per-
Psychol Gen 137:201225. Jordan NC, Hanich LB, Kaplan D. 2003. A longitu- spective. J Learn Disabil 26:214226.
Geary DC. 1993. Mathematical disabilities: cogni- dinal study of mathematical competencies in Shalev RS. 2007. Prevalence of developmental dys-
tive, neuropsychological, and genetic com- children with specific math difficulties versus calculia. In: Berch DB, Mazzocco MM, edi-
ponents. Psychol Bull 114:345362. children with comorbid mathematics and read- tors. Why is math so hard for some children?
Geary DC. 2004. Mathematics and learning dis- ing difficulties. Child Dev 74: 834850. The nature and origins of mathematical
abilities. J Learn Disabil 37:415. Jordan NC, Huttenlocher J, Levine SC. 1992. learning difficulties and disabilities. Baltimore:
Geary DC, Hoard MK, Byrd-Craven J, et al. Differential calculation abilities in young Paul H Brooks Publishing. p 4960.
2007. Cognitive mechanisms underlying children from middle- and lower-income Spelke ES, Kinzler KD. 2007. Core knowledge.
achievement deficits in children with mathe- families. Dev Psychol 28:644653. Dev Neuropsychol 21:241253.
matical learning disabilities. Child Dev 78: Klein JS, Bisanz J. 2000. Preschoolers doing arith- Starkey P, Spelke ES, Gelman R. 1990. Numerical
13431359. metic: the concepts are willing but the abstraction by human infants. Cognition
Griffin S. 2007. Early intervention for children at working memory is weak. Can J Exp Psy- 36:97127.
risk of developing mathematical learning dif- chol 54:105115. Taylor HB, Landry SH, English L, Banes MA.
ficulties. In: Berch DB, Mazzocco MMM, Klingberg T, Fernell E, Olesen PJ, et al. 2005. Infants and children with spina bifida. In:
editors. Why is math so hard for some chil- Computerized training of working memory Donders J, Hunt S, editors. Principles and
dren? the nature and origins of mathematical in children with ADHDa randomized, practice of lifespan developmental neuropsy-
learning difficulties and disabilities. Balti- controlled study. J Am Acad Child Adolesc cology (in press).
more, MD: Paul H. Brookes Publishing. Psychiatry 44:177186. Thelen E, Smith LB. A dynamic systems approach
p 373395. LeFevre J-A. 2000. Research on the development to the development of perception and
Hartje W. 1987. The effect of spatial disorders on of academic skills: introduction to the special action. Cambridge: MIT Press, 1994.
arithmetical skills. In: Deloche G, Seron X, issue on early literacy and early numeracy. Williams LJ, Rasmussen SA, Flores A, et al. 2005.
editors. Mathematical disabilities: a cognitive Can J Exp Psychol 54:5760. Decline in the prevalence of spina bifida and
neuropsychological perspective. Hillsdale, Locuniak MN, Jordan NC. 2008. Using kinder- anencephaly by race/ethnicity: 19952002.
NJ: Erlbaum. p 121135. garten number sense to predict calculation Pediatrics 116:580586.
Hecht SA, Torgesen JK, Wagner RK, et al. 2001. fluency in second grade. J Learn Disabil Wynn K. 1992. Addition and subtraction by
The relations between phonological process- 41:451459. human infants. Nature 358:749750.
ing abilities and emerging individual differ- Lomax-Bream, LE, Barnes M, Copeland K, et al. Zelazo PD, Muller U, Frye D, et al. 2003. The
ences in mathematical computational skills: a 2007. The impact of spina bifida on devel- development of executive function in early
longitudinal study from second to fifth opment across the first 3 years. Dev Neuro- childhood. Monogr Soc Res Child Dev 68:
grades. J Exp Child Psychol 79:192227. psychol 31:120. 1127.

34 Dev Disabil Res Rev  Math in Spina Bifida  ENGLISH ET AL.