Chapter 29 Neurodevelopmental Function and Dysfunction in the School-Aged Child
Terminology and Epidemiology
Overall estimates of the prevalence of academic disabilities range from 3-10%, with more recent data indicating that approximately 8% of children 3-17 yr of age have, at one point, been identified as having an academic disability. The prevalence rate for disorders of attention (e.g., ADHD; Chapter 30) in this same age span is reported at 7.4%, with boys identified at a significantly higher rate than girls (9.5% and 5.9%, respectively). Prevalence estimates can vary owing to numerous factors, including differences in definitions and criteria used for classification and diagnosis and differences in methods of assessment.
Etiology
Multiple factors underlie neurodevelopmental dysfunctions. These include genetic, medical, psychologic, environmental, and sociocultural influences. Some of the genes that contribute to neurodevelopmental dysfunction have been identified. It is well established that reading disorders can be both familial and heritable; studies have linked some reading disabilities to specific gene loci on chromosomes 6 and 15. Chromosomal abnormalities can lead to unique patterns of dysfunction, such as visual-spatial deficits in girls with Turner’s syndrome or language deficits in persons with fragile X syndrome (Chapter 76). Gene-deletion syndromes such as velocardiofacial syndrome are associated with predictable patterns of neurodevelopmental dysfunction (attention and working memory deficits, with academic difficulties in reading comprehension and mathematics conceptualization).
Perinatal risk factors that have been associated with neurodevelopmental dysfunction include very low birth weight, severe intrauterine growth restriction, perinatal hypoxic-ischemia encephalopathy, and prenatal exposure to alcohol and other drugs (Chapter 90). Increased risk of academic and attention disorders is associated with environmental toxins, including lead (Chapter 702); drugs such as cocaine; infections, such as meningitis and HIV; and brain injury secondary to intraventricular hemorrhage or head trauma. There have been conflicting reports regarding the contribution of persistent otitis media with effusion and associated conductive hearing loss to subsequent language problems.
Core Neurodevelopmental Functions
Language
Disordered language occurs in many forms. Some children have particular problems with phonology (Chapter 32). They experience unclear reception of language sounds and have difficulty discriminating between, and forming associations with, the sounds of their native language. For the brain to process these language sounds, it must, for example, accommodate the very rapid transition (∼30 msec in duration) from the sound k to the uh in kuh. In some cases, affected students have trouble processing these acoustic signals within language sounds rapidly enough. Commonly, a weak phonologic sense has a negative effect on reading, as well as other academic skills. A student with a poor appreciation of language sounds is likely to form unstable associative linkages between those sounds and visual symbols (letter combinations). It can be hard for these students to conceptualize words as made up of language sound segments (phonemes); thus, their ability to break words down into their constituent sounds and then reblend them into pronounceable words is impeded. They can also have problems manipulating language sounds in their minds and blending them to form a word.
Visual-Spatial and Perceptual Functioning
Vision begins well before birth, with continued development and refinement throughout childhood (Chapter 613). Important structures involved in the development and function of the visual system, beyond the eyes themselves, include the retina, optic cells (e.g., rods and cones), the optic chiasm, the optic nerves, the brainstem (control of automatic responses like pupil dilation), the thalamus (e.g., lateral geniculate nucleus for form, motion, color), and the primary (visual space and orientation) and secondary (color perception) visual processing regions located in and around the occipital lobe. Other brain areas, considered to be outside of the primary visual system, are also important to visual function, helping to process what (temporal lobe) is seen and where it is (parietal lobe). It is now well documented that the left and right cerebral hemispheres interact considerably in visual processes, with each hemisphere possessing more specialized functions including processing of details, patterns, and linear information mediated by the left hemisphere and processing of the gestalt, form, and integrative functions mediated by the right hemisphere.
Attention
Most brain processes are heavily dependent on functional arousal, alertness, and attention. Any malfunction within or across these systems will likely result in weaknesses in other areas. Functional attention subsumes intact neuroanatomic and neurochemical brain systems. Structurally, brain regions involved include subcortical, cortical, and association areas throughout the brain. Primary structures involved include brainstem regions (e.g., basal ganglia), the limbic system (e.g., amygdala and hippocampus), and the frontal lobes (e.g., prefrontal cortex). The neurotransmitter dopamine, along with its neuronal pathways, has been identified as a major chemical modulator of attention. From the perspective of development, attention begins very early, as infants regularly demonstrate attentional control. With increasing age, the external demands on the attention system increase exponentially. It is through the cognitive mechanisms of attention and executive functions (see next section) that the child’s brain acquires, organizes, and processes information and regulates, plans, and monitors behavior and thought. Children with attention dysfunction comprise a widely heterogeneous group who show various patterns of impairment of these systems (Chapter 30). The resulting symptoms not only affect behavior, learning, and academic skills development but also have an impact on the child emotionally and socially.
Executive Functioning
Inhibition, or inhibitory control, refers to the ability to control a response, whether it be cognitive or behavioral. Disinhibition, then, is the inability to delay or control a response. Failure to “control the behavioral response” most often manifests as impulsivity in the school-aged child, typically resulting in a host of undesired outcomes. Clinically, impulsivity is a behavioral descriptor that suggests that the child’s behavior is disinhibited, resulting in their acting or thinking without regard to the consequences of their actions or thoughts. This concept is also described in detail in Chapter 30 (on ADHD).
Intellectual Function
The expression of intellect is mediated by many factors, including language development, sensory-motor abilities, genetics, heredity, environment, and neurodevelopmental dysfunction or neuropathology. In terms of classification systems based on psychometric testing, one’s intellectual level can fall within a range, from profoundly deficient (IQ standard score ≤25) to well above average (IQ standard score ≥130) and higher. Test results have proved useful in predicting future functionality (e.g., academic, occupational, and social). Notwithstanding, intelligence test scores (e.g., IQ) reflect only part of an individual’s ability profile. Functionally, there are some common cognitive characteristics that distinguish children with lower intellectual functioning from those with average or above abilities. Typically, those at the lowest end of the spectrum (e.g., profound or severe intellectual deficiencies) are incapable of independent function, and require a highly structured environment with constant aid and supervision and an individualized relationship with a caregiver (Chapter 33). At the other end of the spectrum are those with unusually well developed intellect (e.g., gifted). Although this level of intellectual functioning offers many opportunities, it can also be associated with functional challenges related to socialization, learning style, and communication and perceptual differences.
Clinical Manifestations
Reading
Reading disorders (Chapter 31), also termed dyslexia, can stem from any number of neurodevelopmental dysfunctions as described earlier (Table 29-1). Most commonly, language and/or auditory processing weaknesses are present as evidenced by poor phonologic processing. Challenges with phonologic processing often result in deficiencies at the level of decoding individual words and, consequently, a delay in automaticity (e.g., acquiring a repertoire of words they can identify instantly) that causes reading to be slow, laborious, and frustrating. Without effective identification and intervention, reading comprehension, and ultimately the acquisition of knowledge may be seriously compromised.
Table 29-1 NEURODEVELOPMENTAL DYSFUNCTION UNDERLYING ACADEMIC DISORDERS
CADEMIC DISORDER | POTENTIAL UNDERLYING NEURODEVELOPMENTAL DYSFUNCTION |
---|---|
Reading |
Isolated neurodevelopmental dysfunction can lead to a specific academic disorder, but more often there is a combination of factors underlying weak academic performance. In addition to the dysfunction in neurodevelopmental domains as listed in the table, the clinician must also consider the possibility of limitations of intellectual and cognitive abilities or associated social and emotional problems.
Spelling and Writing
Spelling and writing impairments share many related underlying processing deficits with reading, so it is not surprising that the two disorders often occur simultaneously in school-aged children (see Table 29-1). Core neurodevelopmental weaknesses can include phonologic and decoding difficulties, orthographic problems (coding letters and words into memory), and morphologic deficits (use of suffixes, prefixes, and root words). Problems in these areas can manifest as phonetically poor, yet visually comparable approximations to the actual word (faght for fight), spelling that is phonetically correct but visually incorrect (fite for fight), and inadequate spelling patterns (played as plade). Children with memory disorders might misspell words because of coding weaknesses. Others misspell due to poor auditory WM that interferes with their ability to process letters. Sequencing weaknesses often result in transposition errors when spelling. Overall, the careful analysis of a child’s errors can provide valuable insights into the nature of their spelling problems. As children proceed through school, demands increase for large amounts of well-organized written output.
Writing difficulties have been classified as disorder of written expression, or dysgraphia (see Table 29-1). Although many of the same dysfunctions described for reading and spelling can contribute to problems with writing, written expression is the most complex of the language arts, requiring synthesis of many neurodevelopmental functions (e.g., auditory, visual-spatial, memory, executive). Deficits in any of these domains can be problematic. Even when a child’s phonologic and/or orthographic skills are functional, he or she can experience writing problems owing to weaknesses with language, attention, EFs, sequencing and/or fine motor development. These weaknesses can occur in written output that is difficult to comprehend, disjointed, and/or poorly organized. The child with WM challenges can lose track of what he or she intended to write. Attention deficits can make it hard for a child to mobilize and sustain the mental effort, pacing, and self-monitoring demands necessary for writing. In many cases, writing is laborious because of an underlying graphomotor dysfunction (e.g., fluency does not keep pace with ideation and language production). Thoughts may also be forgotten or underdeveloped during writing because the mechanical effort is so taxing.
Mathematics
Delays in mathematical ability, known as mathematics disorder or dyscalculia, can be especially refractory to correction, partly because math involves the assimilation of both procedural knowledge (e.g., calculations) and higher-order cognitive processes (e.g., WM) (see Table 29-1). In a school-based study, it was found that no student who was delayed >6 mo in mathematics in 6th grade ever caught up, and another study has shown persistence of severe arithmetic disorder in half of affected preteen children. Factors associated with persistence of difficulties included the disorder’s severity and heritability. Significant mathematical weaknesses can become virtually insurmountable because the subject is so cumulative in its structure.
Nonacademic Problems
Neurodevelopmental dysfunctions commonly have effects that extend far beyond academic performance. These effects may be related to the dysfunctions themselves or to secondary sequelae (e.g., persistent failure and frustration). The impulsivity and lack of effective self-monitoring of children with attention and impulse-control deficits can lead to unacceptable actions that were unintentional. Children with neurodevelopmental dysfunctions can experience excessive performance anxiety or clinical depression, and sadness, self-deprecatory comments, declining self-esteem, chronic fatigue, loss of interests, and even suicidal ideation can ensue. Some children lose motivation. They tend to give up and exhibit learned helplessness, a sense that they have no control over their destiny. Therefore, they feel no need to exert effort and develop future goals. These children may be easily led toward dysfunctional interpersonal relationships, detrimental behaviors (e.g., delinquency), and the development of mental health and personality disorders, such as mood disorders (Chapter 24) or antisocial personality disorder.
Assessment and Diagnosis
Pediatricians can be helpful in gathering and organizing data on a child with neurodevelopmental dysfunctions. They can obtain such data through the use of questionnaires completed by the parents, the school, and (if old enough) the child. These questionnaires can provide up-to-date information about behavioral adjustment, patterns of academic performance, and traits associated with specific developmental dysfunctions. Questionnaires can elicit relevant data about a child’s health history, family background, and demographic variables relevant to an academic difficulty. Screening instruments such as the Pediatric Symptoms Checklist and standardized behavioral questionnaires, including the Child Behavior Checklist (CBCL) and the Behavior Assessment System for Children—Second Edition (BASC-2) can aid in evaluation (Chapter 18).
Treatment
Medication
Psychopharmacologic agents may be especially helpful in lessening the toll of neurodevelopmental dysfunctions. Most commonly, stimulant medications are used in the treatment of children with attention deficits. Although most children with attention deficits have other associated dysfunctions (such as language disorders, memory problems, motor weaknesses, or social skill deficits), medications such as methylphenidate, dextroamphetamine, lisdexamphetamine, mixed amphetamine salts, and atomoxetine can be important adjuncts to treatment because they seem to help some children focus more selectively and control their impulsivity. These medications, including their indications, administration, and complications, are described in Chapter 30. When depression or excessive anxiety is a significant component of the clinical picture, antidepressants or antianxiety drugs may be helpful. Other drugs may improve behavioral control (Chapter 19). Children receiving medication need regular follow-up visits that include a history to check for side effects, a review of current behavioral checklists, a complete physical examination, and appropriate modifications of the medication dose. Periodic trials off medication are recommended to establish whether the medication is still necessary.
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