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J Clin Exp Neuropsychol. Author manuscript; available in PMC 2011 March 15.
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Published in final edited form as:
J Clin Exp Neuropsychol. 2010 April ; 32(4): 417–432.
Language disorders in children with central nervous system
injury
Maureen Dennis
Program in Neurosciences and Mental Health, The Hospital for Sick Children, Toronto, Ontario,
Canada, and Departments of Surgery & Psychology, University of Toronto, Toronto, Ontario,
Canada
Abstract
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Children with injury to the central nervous system (CNS) exhibit a variety of language disorders
that have been described by members of different disciplines, in different journals, using different
descriptors and taxonomies. This paper is an overview of language deficits in children with CNS
injury, whether congenital or acquired after a period of normal development. It first reviews the
principal CNS conditions associated with language disorders in childhood. It then describes a
functional taxonomy of language, with examples of the phenomenology and neurobiology of
clinical deficits in children with CNS insults. Finally, it attempts to situate language in the broader
realm of cognition and in current theoretical accounts of embodied cognition.
Keywords
Language disorders; Pragmatic; Semantic; Syntactic; Motor speech; Central nervous system injury
INTRODUCTION
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Children and adolescents identified with injury to the central nervous system (CNS) exhibit
a variety of language disorders that have been described by members of different disciplines,
in different journals, using different descriptors and taxonomies. This paper is an overview
of language deficits in children with CNS insults, whether congenital or acquired after a
period of normal development. It first reviews the principal CNS conditions associated with
language disorders in childhood. It then describes a functional taxonomy of language, with
examples of the phenomenology and neurobiology of clinical deficits in children with CNS
insults. Finally, it attempts to situate language in the broader realm of cognition and in
current theoretical accounts of embodied cognition.
The scope of the paper is limited to language after CNS injury. It does not cover primary
language acquisition failure (specific language impairment, SLI), language in conditions
defined by abnormal behavior (e.g., autism; attention deficit hyperactivity disorder, ADHD),
language associated with basic sensory loss (e.g., deafness), or language in children with
mental retardation of genetic origin (e.g., Down syndrome, Fragile X syndrome, Williams–
Beuren syndrome). Although these conditions are often correlated with abnormal brain
development, they are not identified by CNS injury, and their language characteristics and
intervention issues have been covered extensively in journal articles and in recent
handbooks (e.g., Fletcher & Miller, 2005; Schwartz, 2008).
Address correspondence to Maureen Dennis, Program in Neurosciences and Mental Health, Department of Psychology, The Hospital
for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada (
[email protected]).
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CONDITIONS ASSOCIATED WITH CHILDHOOD LANGUAGE DISORDERS
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In children, language can be disrupted by congenital malformations originating at various
points during gestation, by birth trauma, or by brain injury acquired at a later point in
development. The broadest distinction is that between congenital and acquired language
disorders.
Congenital conditions
Unlike adults, children may be born with a significant compromise of the brain mechanisms
responsible for language. A large number of congenital conditions affect language.
Spina bifida meningomyelocele—Spina bifida meningomyelocele (SBM) is a neural
tube defect associated with malformations of spine and brain. It occurs at a rate of 0.3–0.5
per 1,000 live births (from postdietary fortification data), a decline over the past 20 years
widely attributed to the emphasis on dietary supplementations of folate acid and vitamin B
(Williams, Rasmussen, Flores, Kirby, & Edmonds, 2005).
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The neurobiology of SBM involves structural and microstructural abnormalities of the
cerebellum, midbrain, and posterior cortex; and hypoplasia and microstructural
abnormalities of white matter tracts, including the corpus callosum (Del Bigio, 1993; Dennis
et al., 1981; Dennis et al., 2005; Fletcher et al., 1996; Fletcher et al., 2005; Fletcher, Dennis,
& Northrup, 2000; Hannay, 2000; Hasan et al., 2008).
Inborn errors of metabolism—Systemic metabolic disorders that result in the
accumulation of metabolites in the bloodstream cause brain disruption that may include
speech and language deficits. Some of the inborn errors of metabolism that have been shown
to affect speech and language include phenylketonuria (an absence of the enzyme
phenylalanine), galactosemia (an inability to utilize the sugars galactose and lactose because
of disordered carbohydrate metabolism), Wilson disease (a progressive degenerative
disorder of the brain and liver resulting from inability to process dietary copper), Sanfilippo
syndrome (an autosomal recessive enzyme deficiency; Valstar, Ruijter, van Diggelen,
Poorthuis, & Wijburg, 2008), and congenital hypothyroidism (Ozanne, Murdoch, &
Krimmer, 1990).
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Unilateral congenital pathology—Because of the common association of adult
language disorders with left hemisphere brain insult, children with congenital damage to one
side of the brain are of interest to theories of language development, language lateralization,
and functional age-based plasticity. Although much theoretically interesting information
about language has emerged from the study of early focal lesions (e.g., Bates, Thal, &
Janowsky, 1992; Bates, Vicari, & Trauner, 1999), children with complete removal of one
hemisphere have been of particular interest: They represent an extreme form of unilateral
brain injury; cases have similar volumes of lateralized brain damage; and the congenital
malformations in some series are identical on left and right sides (e.g., Hoffman, Hendrick,
Dennis, & Armstrong, 1979), allowing exploration of differences in laterality unconfounded
by differences in pathology.
Childhood-acquired conditions
Like adults, children can exhibit language disorders from injury to the central nervous
system after a period of normal development. Childhood-acquired language disorder, or
childhood-acquired aphasia, refers to language impairment evident after a period of normal
language acquisition that is precipitated by, or associated with, an identified form of brain
insult.
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Seizure disorders—Seizure disorders may be associated with language deficits (Williams
& Sharp, 2000), and language symptoms may be part of clinical seizures or part of ictal
speech automatisms. The most studied seizure disorder is the Landau–Kleffner syndrome
(Denes, 2008; Landau & Kleffner, 1957), which involves acquired aphasia with convulsive
disorder and agnosia for sounds in children who acutely or progressively lose previously
acquired language (Appleton, 1995; Majerus, Van der Linden, Poncelet, & Metz-Lutz,
2004).
Vascular disorders—Vascular disorders involve interruptions to the blood supply within
the brain as a result of occlusion (ischemic stroke) or rupture (hemorrhagic stroke).
Degenerative disorders like atherosclerosis are rare in children, while vascular strokes
associated with congenital heart disease occur in childhood (Ozanne & Murdoch, 1990),
from an embolism from the heart, complications of heart surgery, or hypoperfusion from
prolonged hypotension, or from sickle cell disease (Ris & Grueneich, 2000). The
neurobiology of childhood strokes is variable. Many childhood strokes are secondary to
intracranial occlusive disease in the basal ganglia, although cortical vascular lesions in the
left temporoparietal lobe that produce aphasia occur from cerebral arteritis (Dennis, 1980b)
or ruptured arteriovenous malformations (Hynd, Leathem, Semrud-Clikeman, Hern, &
Wenner, 1995).
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Traumatic brain injury—Children exhibit a range of language disturbances in the acute
phase after head injury (Guttmann, 1942; Jordan, 1990; Loonen & Van Dongen, 1990; Van
Dongen & Loonen, 1977), a common cause of childhood-acquired language disorders. The
neurobiology of childhood traumatic brain injury (TBI) includes immediate impact injury
(contusions, diffuse axonal damage), secondary intracranial events (hematomas, brain
swelling, infections, subarachnoid hemorrhages, hydrocephalus), and extracranial factors
(hypoxia, hypotension). TBI results in focal brain damage (vascular injury involving
contusions and hemorrhage, especially in the frontal and temporal lobes) and diffuse brain
damage (to axons in the white matter), as well as in secondary damage such as raised
intracranial pressure, hypoxia, and ischemia (Gennarelli & Graham, 1998).
Brain tumors—Brain tumors in children are often associated with language disturbances
(Hudson, 1990; Van Dongen & Paquier, 1991). The neurobiology of brain tumors depends
on the tumor etiology, size, and location. The most studied form of childhood brain tumors
are posterior fossa tumors, such as malignant medulloblastomas and benign astrocytomas,
which occur with relatively high frequency in children, and which are associated with
speech and language deficits (Dennis, Spiegler, Riva, & MacGregor, 2004).
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Cancer treatments—Radiotherapy and chemotherapy, which are often part of the
treatment for childhood cancers such as acute lymphoblastic leukemia or malignant brain
tumors, cause structural and functional damage to the brain (Withers, 1992). Long-term
survivors of acute lymphoblastic leukemia treated with radiation and chemotherapy show
cognitive deficits that include language impairments (Hudson, Buttsworth, & Murdoch,
1990; Moleski, 2000).
Infectious conditions—Infectious diseases of the brain, which may involve viral,
bacterial, spirochetal, and other microorganisms that infect the meninges or the brain, may
affect language (Anderson & Taylor, 2000; Smyth, Ozanne, & Woodhouse, 1990), either as
a primary effect of brain involvement in conditions like herpes simplex encephalitis, or as a
secondary effect of sensorineural hearing loss in conditions such as bacterial meningitis or
toxoplasmosis.
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Hypoxic disorders—Anoxia, a state in which the oxygen levels in the body fall below
physiologic levels because of depleted oxygen supply, can come about from various causes
(including severe hypotension, cardiac arrest, carbon monoxide poisoning, near-drowning,
and suffocation) that involve a drop in the level of cerebral blood flow or the oxygen content
of the blood. A prolonged period of cerebral anoxia will produce permanent brain damage or
anoxic encephalopathy, which is associated with a range of language deficits (Murdoch &
Ozanne, 1990).
LANGUAGE DISORDERS
Broadly, language is a code that links linguistic representations with various levels of
meaning (Caplan, 1992): of words, sentences, texts, and social–affective communication.
The code can be instantiated in different modalities (auditory, visual, tactile) and described
at different levels of analysis (word, sentence, text). Multiple codes may coexist.
This section reviews the characteristics of language disorders associated with congenital
brain defects or acquired brain conditions. The information is organized in a taxonomy
(Figure 1) that (reading from left to right) shows language representation level, functional
domain, example of functional domain, and representative clinical deficit.
Pragmatics
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Pragmatics is concerned with how speakers use language to effect successful functional
communication (Kempson, 1975). Pragmatic linguistic representations are activated,
typically in social contexts, to serve intentional purposes (e.g., to give instructions, to mask
and/or communicate thought), and to make affective judgments (e.g., to praise, blame,
criticize, or empathize). A key issue in pragmatic comprehension, as Stemmer (2008)
suggests, is how to compute meaning from something that is not stated, a task that requires
distinguishing sentence meaning (the semantic properties of a sentence) and speaker
meaning (what the speaker intended to communicate; Noveck & Reboul, 2008).
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Intentions—As originally used (Brentano, 1874/1973), intentionality referred to a property
of mental states: that of being directed towards something. More recently, intentionality is
used as a form of representation for mental categories such as beliefs and desires (Malle,
Moses, & Baldwin, 2001), with intentionality being a quality of purposeful actions, and
intentions being mental states that represent those actions. Theory of mind, a component of
social cognition (Yeates et al., 2007), involves the ability to think about mental states in
oneself and others and to use them to understand and predict what other people know and
how they will act (Bibby & McDonald, 2005). The term, theory of mind, emphasizes that
individuals see themselves and others in terms of mental states—desires, emotions, beliefs,
intentions, and other inner experiences—that result in (and from) human action (Wellman,
Cross, & Watson, 2001).
The primary meaning of some verbs is not an action but an internal state. Mental state verbs
(Hall & Nagy, 1986) are a class of words that include know, remember, forget, think,
believe, and pretend (Hall & Nagy, 1986; Karttunen, 1971). These words assume some
information (presuppositions) and suggest other information (implications; Karttunen, 1971;
Kiparsky & Kiparsky, 1970). Speech acts (Searle, 1969) are intentional acts performed by a
dialogue participant to express the mutual intentions of a speaker and a listener and to
influence the mental state of a participant (Beun, 1994).
Children with TBI have deficits in comprehension of mental state verbs (Dennis & Barnes,
2000) and in producing appropriate speech acts (e.g., when asked to produce a statement
using the words “pie either have” while looking at a picture of adolescents in a school
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cafeteria staring at a selection of desserts; Dennis & Barnes, 2000). Adolescents with TBI
who perform poorly on a test of theory of mind express few mental state terms in their
conversations (Stronach & Turkstra, 2008). Children with early unilateral brain damage
have impairments in making pragmatic inferences, with different profiles of deficit
occurring depending on lesion laterality (Eisele, Lust, & Aram, 1998).
Affect—Pragmatics concerns feelings and judgments, as well as information. The ability to
be emotionally deceptive is an important component of pragmatics because it requires the
recognition that inner emotional states need not be congruent with emotional expressions
(Saarni, 1999). Expressing experienced emotion has been termed emotional expression, in
contrast to communicating a socially appropriate emotion, emotive communication (Buck,
1994).
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Children with TBI have more difficulty with emotive than with emotional communication.
They can identify basic emotions felt by characters in narratives (e.g., they know that a
character will feel sad when she is sick), and they can understand why a deceptive emotion
might be communicated (e.g., they know that a child’s mother will not allow her to play if
aware that she is sick); nevertheless, they are unable to communicate the socially deceptive
emotion (e.g., looking happy; Dennis, Barnes, Wilkinson, & Humphreys, 1998). When
performing the same task, children with autism, a profound disorder of language and
communication, have deficits in both emotional and emotive communication (Dennis,
Lockyer, & Lazenby, 2000).
Intentional–affective language—Intentions and affect often operate together, as in
irony and empathy. Irony has complex social functions. Through the use of the rhetorical
functions of praise and blame, irony conveys social messages that include formulating a
judgment while muting its evaluative force (Dews & Winner, 1997), muting criticism
(Harris & Pexman, 2003), or establishing social distance through a negative assessment of
the actions of the hearer (Haverkate, 1990). Empathy is a means of giving comfort or
maintaining social connectedness in complex or difficult situations. One might use an
empathic lie, for instance, to make someone feel better about a bad haircut (e.g., “Your hair
looks really nice like that!”).
Children with TBI have difficulty understanding irony and empathy. They do not distinguish
between the second-order intentions of irony (to make the hearer feel bad about himself or
herself) and empathy (to make the hearer feel good about himself or herself; Dennis, Purvis,
Barnes, Wilkinson, & Winner, 2001).
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Syntax
Syntactic structures assign important aspects of sentence meaning (Caplan & Hildebrandt,
1988), especially of functional roles (e.g., who is acting, who is being acted on). Failure to
assign and interpret syntactic structure signals a syntactic comprehension disorder
(Caramazza & Zurif, 1976), the hallmark of which is an inability to assign aspects of
sentence meaning correctly in sentences that are logically and pragmatically semantically
reversible except for syntactic structure (e.g., the dog chased the cat/the cat chased the dog).
Of patients with syntactic comprehension deficits, some are able to construct relevant
syntactic structures even when they fail to map the products to establish meaning (e.g.,
Linebarger, 1990).
Syntactic arguments—The adult neurobiology of syntax suggests left lateralization. The
adult left hemisphere has a strong association with syntax; functionally, it constructs syntax
in real time (Swinney, Zurif, Prather, & Love, 1996) and assigns syntactic structure during
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language comprehension (Caplan, 1992; Caplan & Hildebrandt, 1988; Stromswold, Caplan,
Alpert, & Rauch, 1996). Damage to perisylvian areas of the left hemisphere disrupts syntax;
conversely, left hemisphere damage that spares this region and disrupts lexical–semantic
function preserves sensitivity to syntax (Dogil, Haider, Husmann, & Schaner-Wolles, 1995).
The immature left hemisphere also has a strong association with syntax. Compared to those
with early right hemisphere damage and hemispherectomy, individuals with congenital
damage to and removal of the left hemisphere are slower and less accurate in understanding
sentences with noncanonical word orders (e.g., reversible passive sentences such as the dog
is chased by the cat) in which meaning is provided by syntactic structure but not semantic
plausibility, whether comparisons are made between hemidecorticate groups with early
lateralized hemispheric damage from varying pathologies (Dennis & Kohn, 1975) or from a
single pathology (Dennis & Whitaker, 1976). The syntactic comprehension deficit after left
hemisphere damage is evident whether comparisons are made to chronological age (Aram,
Ekelman, Rose, & Whitaker, 1985; Dennis, 1980a; Dennis & Kohn, 1975; Dennis &
Whitaker, 1976; Paquier & Van Dongen, 1993), mental age (Stark, Bleile, Brandt, Freeman,
& Vining, 1995), or brain-intact cotwins (Feldman, Holland, & Keefe, 1989; Hetherington
& Dennis, 2004).
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Syntactic comprehension deficits for noncanonical word orders are also evident when only
part of the left hemisphere is removed but speech control has shifted to the right hemisphere
(Kohn, 1980). These data are in agreement with functional magnetic resonance imaging
(fMRI) demonstrations that language reorganization after hemispherectomy in childhood
involves right hemisphere homologues of the left hemisphere language areas (Liégeois,
Connelly, Baldeweg, & Vargha-Khadem, 2008) and also with data showing that congenital
left hemisphere focal lesions prompt a mirror-image reorganization of the entire
cerebrocerebellar network engaged in speech production (Lidzba, Wilke, Staudt, KrägelohMann, & Grodd, 2008).
Semantics
Broadly, semantics is concerned with meaning. Semantic disorders in children with brain
injury range from severe verbal auditory agnosia for common sounds, such as a dog barking
or a doorbell ringing (Cooper & Ferry, 1978), to problems understanding word meaning
(Dennis, 1992) or oral or written texts (Barnes, Faulkner, Wilkinson, & Dennis, 2004).
Lexicon—The lexicon consists of a word store, most of it concerned with literal meanings
of individual words. The lexicon also involves phrasally stipulated meanings of overlearned
figurative expressions, such as common idioms.
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Literal meaning: According to standard models of speech production (Levelt, 1989),
producing semantically appropriate words requires accessing word forms as well as word–
sound planning so that the representation of the sound of a word may be converted into a
form suitable for production. Disorders of lexis are common in both adults (Libben, 2008)
and children with brain injury.
Impairments in accessing word forms from concepts are exhibited by failure to produce a
word in response to a picture, definition, or context, despite intact semantic and
phonological processing evidenced by the ability to describe, categorize, or repeat the target
word. A variety of forms of childhood brain injury are associated with word-finding
difficulties in which children can produce a word in response to a picture, although not to
semantic information such as “What lives in the jungle, has big floppy ears and a trunk?”
(Dennis, 1992). Indications of a disorder in accessing word forms include pauses, sequences
of sounds that do not form words (neologisms), words related in meaning to the target
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(semantic paraphasias), and the use of circumlocutions. In the fluent form of childhood
aphasia, associated with lesions to the posterior left hemisphere cortical language areas
(Klein, Masur, Farber, Shinnar, & Rapin, 1992), anomia, word-finding deficits, semantic
paraphasias, and circumlocutions occur (Dennis, 1980b; Hynd et al., 1995). Children with
aphasia from TBI exhibit a variety of language symptoms in the acute stage, which show
some resolution over time (Loonen & Van Dongen, 1990; Van Dongen & Loonen, 1977),
even though anomia and reduced verbal fluency are consistent long-term deficits (Hécaen,
1983; Jordan & Murdoch, 1993; Jordan, Ozanne, & Murdoch, 1988, 1990).
The adult aphasia literature includes an extensive series of report of category-specific
semantic disorders, with reports of selective semantic impairment of particular concepts,
such as those related to animate things and food compared to man-made objects (e.g., Sartori
& Job, 1988). Interpretation of category-specific naming deficits is still controversial (see
Mahon & Caramazza, 2009; Martin & Caramazza, 2003).
Modality-specific anomia has been described in children (e.g., Dennis, 1976). Reading and
spelling may be relatively preserved with cortical lesions in the left-hemispheric posterior
language areas in children with anomia (Dennis, 1980b; Hynd et al., 1995). Further, a child
with anomia from stroke was able to perform tactile–visual matches of misnamed objects
(Dennis, 1980b). These data suggest a problem between modality-specific semantic systems
and the activation of auditory word forms.
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Literal meaning has been studied by analysis of symptoms of fluent adult aphasia, such as
logorrhea, verbal stereotypies, perseverations, neologisms, jargon, and paraphasias, which
were once thought to be rare in children (Alajouanine & Lhermitte, 1965). Aphasic
symptoms in children have proved to be quite varied (Van Hout, 1991), with a number of
adult aphasic syndromes being described in children: jargon aphasia (Visch-Brink & Van de
Sandt-Koenderman, 1984; Woods & Teuber, 1978); Wernicke’s aphasia and transcortical
sensory aphasia (Van Hout, Evrard, & Lyon, 1985); conduction aphasia (Van Dongen,
Loonen, & Van Dongen, 1985); transcortical sensory aphasia (Cranberg, Filley, Hart, &
Alexander, 1987; Van Dongen & Paquier, 1991); anomic aphasia (Hynd, Semrud-Clikeman,
Lorys, Novey, & Eliopulos, 1990); and alexia without agraphia (Makino et al., 1988;
Paquier et al., 1989). To be sure, adult aphasic syndromes observed in children occur with
different base frequencies (Rapin, 1995).
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Figurative meaning: Idioms are nonliteral phrases (e.g., kick the bucket) whose figurative
meanings (here, to die) cannot be derived from the literal meanings of their individual words
(here, kick and bucket). Idioms are ubiquitous (Brinton, Fujiki, & Mackey, 1985) in
conversational (approximately four figures of speech occur in each minute of conversation;
Pollio, Barlow, Fine, & Pollio, 1977) and instructional language (Lazar, Warr-Leeper,
Nicholson, & Johnson, 1989).
Studies of figurative language have identified two types of idiom: one processed like literal
language, the other requiring semantic decomposition. Nondecomposable idioms (e.g., kick
the bucket) are learned and represented in the mental lexicon as units; being syntactically
and lexically inflexible, they depend on context for interpretation (Gibbs, 1991). Children
with SBM have difficulty understanding idioms, even when they can understand the
individual words in the idiom. Of interest, they are better able to understand idioms whose
meaning can be derived from the individual words (e.g., “talk a mile a minute”) than
nondecomposable idioms that require them to integrate the words and the context (HuberOkrainec, Blaser, & Dennis, 2005). Their relatively well-preserved syntax and grammar
skills for literal language (Dennis, Hendrick, Hoffman, & Humphreys, 1987) may facilitate
the semantic compositional analysis of decomposable, but not nondecomposable, idioms.
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The neurobiology of idioms may involve a distributed neural system. For children with
SBM, variability in idiom comprehension is related to the integrity of the corpus callosum
(Huber-Okrainec et al., 2005).
Text—The derivation of meaning in oral or written texts involves complex (van Dijk &
Kintsch, 1983) inferencing and integration among a text representation built on current
words and sentences, prior context, and world knowledge (Perfetti & Frishkoff, 2008). The
flow of topics, narrative script, and the plotline form the schematic structure. The text
macrostructure concerns the relations among sentences sharing a topic, and deficits in
macrostructure are usually manifest as failures of text coherence. Text microstructure
concerns local sentential relations, and these are usually manifest as failure of text cohesion.
Schematic structure: Problems in schematic structure are not uncommon in children with
brain injury. Both children with TBI and children with hydrocephalus, most with SBM, have
deficits in producing and understanding social scripts (Chapman et al., 1992; Dennis &
Barnes, 1990).
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Macrostructure and microstructure: Problems in text macrostructure are also common in
children with brain injury. In telling stories, children with hydrocephalus, most with SBM,
produce poorly coherent narratives that are difficult to process, unclear, and uneconomic
(Dennis, Jacennik, & Barnes, 1994). Children with TBI also have problems in discourse
macrostructure (Chapman et al., 2004; Ewing-Cobbs, Brookshire, Scott, & Fletcher, 1998).
Problems in text microstructure have been identified in children with CNS injury. Children
with left hemi-decortication for congenital left hemisphere damage have impaired referential
cohesion in texts, failing to sustain a chain of anaphoric reference (Lovett, Dennis, &
Newman, 1986). Children with early focal brain injury also show impairments in referential
cohesion (Reilly, Bates, & Marchman, 1998). Compared to controls, the narratives of
children with hydrocephalus, most with SBM, include referentially ambiguous material
(Dennis et al., 1994).
Inferencing—Text meaning is constructed over time, not always literally stated. Text
inferences create a text representation, through coherence inferences (integration of semantic
knowledge with lexical content to interpret the text) and elaborative inferences (creation of a
mental model of the situation the text describes; Morrow, Bower, & Greenspan, 1990;
Whitney, 1987; Zwaan, Langston, & Graesser, 1995). Children with TBI make coherence
but not elaborative inferences (Barnes & Dennis, 2001; Dennis & Barnes, 2001).
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Text inferences: Text-based representations denote the literal meaning of the text and are
constructed through integration of information in the text and revision of information in
relation to the unfolding context (Clifton & Duffy, 2001; Kintsch, 1988; Schmalhofer,
McDaniel, & Keefe, 2002). Integration is effected through processes such as pronominal
reference, which links characters, objects, and events with their referent pronouns, and by
bridging inferences, which integrate ideas or sentences explicitly stated within a text.
Initially, word meanings are passively activated without respect to the context; typically,
more semantic information is activated than will be required to represent the text
(Schmalhofer et al., 2002). As text becomes integrated, contextually irrelevant meanings are
suppressed or their activation is not sustained, and appropriate meanings are enhanced
(Gernsbacher, 1990; Gernsbacher & Faust, 1991). For example, in order to interpret the
sentence, “Jim picked up the spade,” the reader may retrieve information from earlier in the
text about Jim helping his mother in the garden. Children and adolescents with
hydrocephalus, most with SBM, have difficulty integrating propositions within texts, take
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longer to integrate information over larger chunks of text, continue to show substantial
interference effects in ambiguity resolution tasks beyond the point at which their peers have
suppressed contextually irrelevant meanings, and are less efficient in constructing a coherent
and integrated text base (Barnes et al., 2004).
Knowledge-based inferences: The online working model of text meaning includes realworld knowledge and goals, such as inferences about space, time, causality, and the goals of
the characters (Kintsch, 1988; Schmalhofer et al., 2002; Zwaan & Radvansky, 1998).
Constructing situation models requires knowledge-based inferences, which we have studied
in typical (Barnes, Dennis, & Haefele-Kalvaitis, 1996) and atypical (Barnes & Dennis, 1996,
2001; Cain, Oakhill, Barnes, & Bryant, 2001) development, using a paradigm in which
children learn a new knowledge base about a make-believe world and then integrate new
knowledge with events in the text. Children with hydrocephalus, most with SBM, make
fewer knowledge-based inferences than controls (Barnes & Dennis, 1998). Differences with
same-age peers are magnified when the processing load is high (i.e., when inferences have
to be made by retrieving knowledge from memory as stories unfold in time), and the
differences are attenuated when the processing load is low (i.e., when they are cued with the
knowledge needed to make an inference; e.g., Barnes & Dennis, 2001; Cain et al., 2001).
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Text-based comprehension—Text meaning is continuously and actively constructed
through a series of online comprehension processes that operate at the word, sentence, and
text levels, and which draw on cognitive resources such as working memory. In a recent
model of meaning comprehension (Barnes, Huber, Johnson, & Dennis, 2007) we have
proposed that meaning involves a surface code, a text base, and a situation model, the last
two supported by an integration and revision buffer fueled by WM and inhibitory control.
The surface code directly activates old meanings stored in memory. Online iterative cycles
of integration and revision facilitate the construction of new meaning.
Models of comprehension distinguish between automatic activation of surface codes (the
passive activation of meaning from stored lexical representations; Clifton & Duffy, 2001)
and resource-intensive construction and revision of text-based representations and situation
models. Children with SBM can construct text-based representations when the demands on
integration and revision are relatively low, but not when significant revision processes are
required. They seem unable to integrate across sentences to update and revise situation
models. They also have difficulty constructing situation models by integrating text and
world knowledge to understand ongoing narratives.
Phonology
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The segmental aspect of phonology and speech processing involves phonological perception
and integration (Plante, Holland, & Schmithorst, 2006) of features such as vowels,
consonants, and syllables that have direct, identifying relationships with utterances (Crystal,
1973). The nonsegmental aspect of phonology concerns a range of features, loosely referred
to as “tone of voice,” that include intonation, stress, rhythm, and speed of speaking and that
have a variable relation to the segmental, verbal aspects of utterances (Crystal, 1973).
Segmentals—Disorders of phonological processing commonly follow both adult
(Buckingham & Christman, 2008) and child brain injury (Aram & Nation, 1982).
Impairments in word–sound planning are demonstrated by substitutions involving phonemes
with dissimilar distinctive features and/or omissions and misordering of phonemes
(phonemic paraphasias). Brain-injured children’s word-finding errors include phonemic
paraphasias, phonemic jargon, and neologisms (Dennis, 1980b; Van Dongen & Paquier,
1991; Visch-Brink & Van de Sandt-Koenderman, 1984). Phonological-processing deficits
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are among the language and reading impairments reported in children who have sustained
left hemisphere strokes in middle childhood (Dennis, 1980b; Pitchford, 2000). Compared to
his cotwin, a child with a left hemisphere stroke in middle childhood regained phonological
skills lost at the time of the stroke, although he failed to acquire new phonological skills
(Hetherington & Dennis, 2004).
Phonological compromise is often considered the core deficit in developmental dyslexia
(Lyon, 1995). Structural and functional neuroimaging studies of normal readers and
individuals with dyslexia have demonstrated the engagement of left hemisphere structures in
both reading and phonological processing (e.g., Pugh et al., 2000; Simos, Breier, Fletcher,
Bergman, & Papanicolaou, 2000).
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Suprasegmentals—Prosody, or suprasegmental information, cues both emotional state
(emotional prosody, including the emotional state of the speaker) and language structure,
including sentence type (e.g., whether question or statement), the occurrence of phrasal units
within sentences, and boundaries or words within phrases (Plante et al., 2006). Disturbances
in emotional prosody have been observed in adults with brain injury (Van Lancker-Sidtis,
2008) and in children with severe TBI (Hattiangadi et al., 2005). In terms of neurobiology,
prosodic disturbances have been observed in children with right hemisphere dysfunction
(Cohen, Branch, & Hynd, 1994) and young adults with congenital agenesis of the corpus
callosum (Paul, Van Lancker-Sidtis, Schieffer, Dietrich, & Brown, 2003).
Morphology
Morphology is critical to the production of complex words (Jarema, 2008), and components
of the language production system are important for producing free-standing function words
and inflectional morphemes in words and sentences. These morphological components are
different from vocabulary words because they do not bear nonemphatic stress in derived
words or sentence structures, and they do not follow regular word formation processes. The
speech of adult aphasic patients with anterior lesions is often agrammatic, with a breakdown
of sentence structure and the omission or misuse of grammatical morphemes, even while
access to content words, such as verbs and nouns, is relatively unimpaired. In adults, a
disturbance affecting the production of inflections and derivational morphemes has been
termed agrammatism or paragrammatism, and the characteristic is the omission or
substitution of function words and affixes (Menn, Obler, & Goodglass, 1990).
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The neurobiology of morphology suggests some hemispheric lateralization in some cases of
childhood CNS damage. Curtiss and Schaeffer (1997) analyzed the language of children
with hemispherectomy with respect to the inflectional (I) system and its subcategories tense,
subject agreement, and object agreement. Compared to those with right hemispherectomy,
children with left hemispherectomy use a restricted range and number of I-system
morphemes and have particular problem with auxiliaries, despite intact syntactic and
morphological structures of other types (Curtiss & Schaeffer, 1997). Children with left
hemispherectomy also have particular problems producing tag questions, which involve
inflectional morphology (Dennis & Whitaker, 1976), and deficits that occur in some adults
with paragrammatism. These data suggest a high level of vulnerability of the inflectional
system to developmental, childhood-acquired, and adult-acquired language pathology.
Speech production
Phonetics—Phonetic deficits, termed developmental apraxia of speech or developmental
verbal dyspraxia, involve difficulty in coordinating voluntary movements of the speech
articulation mechanism (Aram & Nation, 1982; Shriberg, Aram, & Kwiatkowski, 1997a,
1997b). They are a neurological childhood speech sound disorder in which the precision and
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consistency of speech movements are impaired in the absence of neuromuscular deficits
such as abnormal reflexes. They may occur from known neurological impairment or as an
idiopathic speech sound disorder, and the core impairment is in planning and/or
programming the spatiotemporal parameters of movement sequences that result in errors of
speech sound production and speech prosody (American Speech-Language-Hearing
Association, 2007). They may co-occur with language disorders and/or speech fluency
disorders, described below.
Motor speech—Congenital malformations of the cerebellum are associated with a form of
ataxic dysarthria, which is a motor speech deficit involving dysfluency, ataxic dysarthria
(articulatory inaccuracy, prosodic excess, and phonatory–prosodic insufficiency), and
slowed speech rate (Brown, Darley, & Aronson, 1970; Darley, Aronson, & Brown, 1969).
The narratives of both children and young adults with SBM are characterized by all three
motor speech deficits (Huber-Okrainec, Dennis, Brettschneider, & Spiegler, 2002).
A range of acquired CNS insults in children result in mutism, often resolving to ataxic
dysarthria, and termed mutism with subsequent dysarthria (MSD). There is an initial period
of mutism in herpes simplex encephalitis (Paquier & Van Dongen, 1991; Van Hout et al.,
1985), and mutism has also been described in children treated for cancer (Hudson et al.,
1990) and children with anoxic encephalopathy (Cooper & Flowers, 1987).
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The most fully studied form of MSD is the one identified in children treated for posterior
fossa tumors, who acutely or progressively lose previously acquired language skills (Dailey,
McKhann, & Berger, 1995; Di Cataldo et al., 2001; Doxey, Bruce, Sklar, Swift, & Shapiro,
1999; Humphreys, 1989; Rekate, Grubb, Aram, Hahn, & Ratcheson, 1985; Van Dongen,
Catsman-Berrevoets, & van Mourik, 1994). Nearly all MSD patients are less than 10 years
of age, and the condition has been described in children as young as age 2 (Van Dongen et
al., 1994).
The MSD syndrome involves a complete but transient loss of speech, resolving into a
dysarthria with imprecise consonants, articulatory breakdowns, prolonged phonemes,
prolonged intervals, slow rate of speech, lack of volume control, harsh voice, pitch breaks,
variable pitch, and explosive onset (Hudson, Murdoch, & Ozanne, 1989; van Mourik,
Catsman-Berrevoets, Yousef-Bak, Paquier, & Van Dongen, 1998). Improvement of the
dysarthria to normal speech seems to be related to the recovery of complex movements of
the mouth and tongue (Van Dongen et al., 1994).
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Long-term recovery of the ataxic dysarthria is incomplete. Very-long-term survivors of
childhood cerebellar tumors continue to show ataxic dysarthric features in their spontaneous
speech (Huber, Dennis, Bradley, & Spiegler, 2007); further, tumor survivors with a history
of MSD show more ataxic dysarthria than those without MSD at the time of tumor treatment
(Huber-Okrainec, Dennis, Bradley, & Spiegler, 2006).
Because mutism has been most commonly associated with cerebellar tumors, its
neurobiology has been fairly well studied. Mutism has been associated with posterior fossa
tumors located in the midline or vermis of the cerebellum and with tumors that invade the
cerebellar hemispheres or the deep nuclei of the cerebellum (Humphreys, 1989; Rekate et
al., 1985) and brainstem (Doxey et al., 1999; van Mourik et al., 1998). Children with
postoperative mutism show brainstem compression, which may represent white matter
injury (McMillan et al., 2009). It has been proposed that the mutism of MSD is related to
bilateral involvement of the dentate nuclei, and, further, that the subsequent dysarthric
speech represents a recovering cerebellar mechanism (Ammirati, Mirzai, & Samii, 1989).
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LANGUAGE AND OTHER COGNITIVE SYSTEMS
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Language does not exist in isolation. Both comprehension and production of language are
grounded in the motor system. Language is related to general-purpose brain memory
systems and is instantiated by cognitive resources such as working memory and inhibitory
control.
Language as embodied action
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Earlier views of language assumed it to have conceptual content that was more abstract than
information in sensory and motor systems. More recently, however, the embodied cognition
framework has argued that conceptual content is grounded in basic sensory and motor
processes (e.g., Gallese & Lakoff, 2005; Zwaan, 2004). Current theoretical accounts of
language as embodied action (Fernandino & Iacoboni, in press; Fischer & Zwaan, 2008) are
unraveling the interaction between the brain’s systems of language and action for both
production and comprehension (reviewed in Zwaan, Taylor, & de Boer, in press). For
example, verbs describing actions activate areas of the motor cortex that are also active
when the action is performed. Verbs referring to hand actions activate the hand area of the
motor strip, verbs referring to leg actions the more dorsal leg area, and verbs denoting mouth
actions the mouth area (Hauk, Johnsrude, & Pulvermüller, 2004; but see Kemmerer,
Gonzalez Castillo, Talavage, Patterson, & Wiley, 2008, who found somatotopic activation
for hand and leg verbs, but not for mouth verbs). Similar effects have been reported in
neuroimaging studies of sentence comprehension (Aziz-Zadeh, Wilson, Rizzolatti, &
Iacobini, 2006; Tettamanti et al., 2005). Consistent with this, behavioral evidence suggests
motor involvement in language comprehension (e.g., lexical stimuli have been shown to
momentarily affect hand aperture in a reaching-to-grasp task; Gentilucci & Gangitano, 1998;
Glover, Rosenbaum, Graham, & Dixon, 2004). Similarly, hand–arm actions are influenced
by sensibility judgments about sentences (e.g., Glenberg & Kaschak, 2002; Zwaan &
Taylor, 2006).
General purpose systems
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It has been proposed (Ullman, 2008; Ullman et al., 1997) that lexical information depends
on declarative memory (specialized for arbitrary associations and grounded in temporal lobe
structures) while grammar relies on procedural memory (specialized for rules and sequences
and rooted in frontal and basal ganglia structures). The argument is that, given that word
forms are like facts in being arbitrary, the declarative memory system may subserve words
as well as facts and events, and, given that rules are like skills in requiring the coordination
of procedures in real time, the procedural memory system may process grammatical rules as
well as motor and perceptual skills. Support for this proposal has come from several sources.
Although there are different views about whether regular and irregular past tense forms of
verbs are both computed by rules (Chomsky & Halle, 1968) or memory (MacWhinney &
Leinbach, 1991), Ni et al. (2000) hypothesized that irregular past tense verb forms are
memorized, while regular forms (verb stem +ed) are generated by a rule that comprises two
operations: copying the stem and adding a suffix. Patients with impaired declarative memory
(Alzheimer’s disease) or lexical memory (posterior aphasia) were found to overgeneralize
the past tense suffix and to have more difficulty converting irregular verbs to past tense
forms than they did converting regular or novel verbs. Patients with procedural impairments
and damage to the frontal-basal ganglia system (Parkinson’s disease) or with agrammatism
(anterior aphasia) showed the opposite pattern. In a fMRI paradigm, form errors (e.g.,
“Trees can grew”) triggered increased activation in the left inferior frontal area (Broca’s
area), whereas meaning anomalies (e.g., “Trees can eat”) resulted in superior temporal and
middle and superior frontal activation (Ni et al., 2000).
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The broad dissociation between form and procedural memory, on the one hand, and meaning
and semantic memory, on the other, is evident in children with brain injury. Despite a range
of cognitive and language problems in the semantic and pragmatic domains (Fletcher,
Barnes, & Dennis, 2002), children with SBM have intact procedural learning and memory
(Edelstein et al., 2004), as well as relatively intact ability to assign thematic roles in
syntactic comprehension tasks (Dennis et al., 1987). This dissociation is also demonstrable
after childhood-acquired brain injury. Comparing language recovery in 13-year-old identical
twins, one of which had sustained a left hemisphere arteritic stroke at age 7 (Hetherington &
Dennis, 2004), we reported that the twin with the stroke showed full recovery of semantic
memory and word production, but had persisting impairments in tag question production and
the ability to assign thematic roles in syntactic comprehension tasks. Considered together, a
group of studies suggest an association between the procedural system and grammar and a
dissociation between the procedural system and semantics within a form of congenital brain
injury and, further, an association between recovery of semantic memory and word finding
and a dissociation between recovery of semantic memory and the persistence of syntactic
impairments in childhood-acquired stroke.
Processing resources
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Language comprehension is constrained by processing resources. WM is the process by
which information is temporarily activated in memory for rapid manipulation and retrieval.
Inhibitory control is the ability to stop or modulate ongoing actions or to switch between
competing representations. In adults, verbal WM capacity is related to language
comprehension (Caplan & Waters, 2006), and, in children, WM efficiency is linked to a
range of language, learning, and academic competencies (e.g., Bull & Scerif, 2001). Both
WM and inhibitory control have a resource-limited capacity (Gazzaley, Cooney, McEvoy,
Knight, & D’Esposito, 2005), so WM involves the activation of information, and inhibitory
control keeps irrelevant information out of WM (Dempster, 1993; Engle, Conway, Tuholski,
& Shisler, 1995; Harnishfeger & Bjorklund, 1994) through processes like suppression.
Working memory—Understanding sentences and texts is subject to capacity constraints,
and text comprehension requires active memory for meaning construction (Clifton & Duffy,
2001; van den Broek, Young, Tzeng, & Linderholm, 1999). Because texts extend over time,
with semantic representations being iteratively modified by reactivating old information and
linking it with incoming information, working memory is a capacity component of many
language comprehension models (e.g., Kintsch, 1988; van den Broek et al., 1999).
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Capacity constraints limit comprehension in children with language disorders. Sentence and
text comprehension is impaired in SBM (e.g., Dennis & Barnes, 1993), with comprehension
deficits being driven, at least in part, by capacity limitations related to integrating meaning
over longer text segments (Barnes et al., 2004). WM links semantic information and its
source and integrates distant information across text segments, and WM limitations are
associated with poor text comprehension.
WM is a source of syntactic comprehension disorder (Caplan & Hildebrandt, 1988). One
source is a specific disturbance with functional argument structure and difficulties with
noncanonical orders of sentence constituents, possibly because of defective representations
or procedures for handling the traces of moved semantic roles in sentences like passives and
object relatives with noncanonical word orders (Berndt, Mitchum, & Wayland, 1997).
Another source is a reduction in the computational resources for syntactic comprehension
(Caplan, Waters, DeDe, Michaud, & Reddy, 2007). For example, the poor formation of
syntax in adult Broca’s aphasia occurs not because of insufficient knowledge of syntactic
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dependencies, but because of failure of real-time implementation of these specific
representations (Love, Swinney, Walenski, & Zurif, 2008).
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Individuals with hemispherectomy for early left hemisphere injury have a combined
impairment. They are insensitive to the role of function words that cue syntactic structure,
even on metacognitive tasks with no time constraints (Dennis, 1980a), which suggests that
they have trouble constructing functional argument structures. In addition, they are slower to
respond correctly to noncanonical sentences, and they make fewer errors when they respond
slowly (Dennis & Kohn, 1975), which suggests some limitation in WM resources, with
performance deficits becoming attenuated when more WM resources are allocated to the
task.
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Inhibitory control: Activation and suppression—Inhibitory control may be
automatic or effortful (Friedman & Miyake, 2004). Automatic inhibitory control, such as the
ability to avoid returning gaze to a previously explored location, develops in infancy or early
in childhood (Richards, 2003). Effortful forms of inhibitory control, involving the ability to
stop performing automatic or routine behaviors when they become undesirable due to
changing circumstances or to altered intentions, have the more protracted development
(Band, van der Molen, Overtoom, & Verbaten, 2000; Harnishfeger & Pope, 1996; Houghton
& Tipper, 1994; Pascual-Leone, 2001; Williams, Ponesse, Schachar, Logan, & Tannock,
1999). One form of effortful inhibition (Kipp, 2005) involves the maintenance of competing
cognitive representations and the successive activation and suppression of irrelevant
representations (Perner, Lang, & Kloo, 2002).
Suppression is that aspect of inhibitory control preventing no-longer-relevant information
from remaining activated. Suppression is an active process, not simply a passive decay, and
one that is driven by top-down semantic representations (Gernsbacher, 1995). Suppression
uses mental resources; for example, the inhibition of inappropriate lexical interpretations
puts demands on cognitive processes (Tompkins, Lehman-Blake, Baumgaertner, &
Fassbinder, 2002).
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Children with hydrocephalus, most with SBM, fail to suppress contextually irrelevant
meaning, which is associated with poor text comprehension (Barnes et al., 2004), in
agreement with the findings in adults that active suppression is important for both literal and
figurative language comprehension (Gernsbacher & Faust, 1991; Gernsbacher & Robertson,
1999). Deficient suppression may not only preempt other comprehension processing
resources, but also provide incomplete input to mental computations that generate a wellspecific semantic representation (Tompkins et al., 2002). Children with SBM have poorly
specified semantic representations that contain extraneous, contextually irrelevant
information, which may explain the long-standing observation (Taylor, 1961) that their
conversational language is referentially underspecified and tangential. The consequences of
impaired semantic representation are considerable. Semantic representations may be well
specified or underspecified, the latter form of shallow parsing being sufficient for some
tasks, such as generating an index or chatting at a cocktail party, although not for others,
such as providing or understanding specific instructions, communicating referentially
detailed information, or academic learning (Sanford & Sturt, 2002).
CODA
Children exhibit a range of language disorders after acquired or congenital brain injury.
Language breakdown may occur at the level of pragmatics, syntax, semantics, phonology,
morphology, or speech production. While earlier views of language considered it in relative
isolation, current views highlight how language is grounded in motor functions, is part of
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general-purpose cognitive systems, and relies on domain-general resources. Much is yet to
be understood about the neurobiology of language disorders following CNS injury, but even
more remains to be discovered about how language disorders engage with movement and
cognition.
Acknowledgments
Preparation of this paper was supported in part by National Institute of Child Health and Human Development
Grants P01 HD35946 and P01 HD35946–06, “Spina Bifida: Cognitive and Neurobiological Variability,” by
National Institute of Neurological Diseases and Stroke Grant 2R01NS 21889–16, “Neurobehavioral Outcome of
Head Injury in Children,” and by National Institutes of Health Grant 1RO1 HD04946, “Social Outcomes in
Pediatric Traumatic Brain Injury.” I thank Arianna Stefanatos for assistance with manuscript preparation.
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Figure 1.
Language taxonomy. The leftmost column shows levels of language representation. The
next column shows functional domains within each representation level. The next column
shows examples of each functional domain. The rightmost column provides examples of
clinical deficits for each functional domain.
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NIH-PA Author Manuscript
J Clin Exp Neuropsychol. Author manuscript; available in PMC 2011 March 15.