Specific reading disability: a multiplanar view.

In the past three decades a revolution has altered the way society approaches people with disabilities. Social changes resulted in a significant increase in fundamental and applied research that seeks to improve the lives of people with disabilities by facilitating better understanding of the mechanisms, manifestations, prevention, and treatment of functional impairment. Specific Reading Disability (SRD) has benefited from this revolution. This review focuses on the evolution of SRD, new information in its neurobiology and management, and the challenges that remain. Evidence from a wide spectrum of research provides strong support for the role of phonology in Specific Reading Disability. Despite the mounting evidence, the case is far from completely established. Adults with compensated SRD read but still demonstrate disordered phonology (Felton et al. [1990] Brain Language 39:485-497). Whether poor phonology is causal or a covariant remains to be demonstrated. Of children with poor phonology, it is not known how many are poor readers. While phonology is associated with SRD, other studies have questioned the uniqueness of SRD. Challenges have been made to the method of classification, the uniqueness of phonological dysfunction as a mechanism in SRD and the response to treatment. In the final analysis all poor readers may have a common core of dysphonology, independent of whether their reading is discrepant from their IQ.

Sequential neuromotor examination in children with intrauterine cocaine/polydrug exposure.

Data are presented on 157 newborn infants followed sequentially in a randomized home-based nursing-intervention trial for drug-exposed infants with follow up at 3 (N=118), 6 (N=124), and 12 months (N=77). The objectives of this study were to describe the longitudinal neurodevelopmental status of a cohort of children with intrauterine exposure to illicit drugs during their gestation, characterize the evolution of early tone abnormalities in a polydrug-exposed cohort, and determine whether neuromotor outcome is associated with drug-exposure patterns. For analysis, infants were grouped based on maternal drug-use pattern and the presence of drug metabolites in the neonatal drug screen. The sequential neuromotor examination was used at each age to define the neuromotor status of six domains and define categorical classifications as either normal, suspect, or abnormal. Multiple patterns of neuromotor abnormalities were observed during the neonatal period; most resolved over time. Axial hypotonia was a prominent finding in the neonatal period; however, it was infrequent in abnormal examinations at 12 months. Increased lower-extremity tone was a less frequent finding during the neonatal period. Infants whose neonatal urine drug screen was positive for both cocaine and opiates, were more likely than infants with negative urine drug screens, cocaine only, or opiate only drug screen results to have abnormal neuromotor examinations; while positive maternal drug screens for concurrent cocaine and opiate use were associated with peripheral hypertonia. Persistence of increased leg-extensor tone was found in 67% of the abnormal examinations at 12 months. Acquisition of rolling and walking was delayed in the drug-exposed cohort.

The prevalence of the FMR1 and FMR2 mutations among preschool children with language delay.

We examined the prevalence of the fragile X mental retardation (FMR1) full mutation and fragile X E mutation (FMR2) among preschoolers evaluated for language delay. A total of 534 preschoolers recruited from a Developmental Pediatric or Speech and Language Disorders clinic were tested with Southern blot and polymerase chain reaction DNA analyses; 3 were found to have the FMR1 full mutation. None of the 534 children tested positive for the FMR2 full mutation; however, 3 children had unusually small FMR2 alleles suggestive of FMR2 deletions. Screening for fragile X among language-delayed preschoolers is warranted, particularly when there is a family history of mental retardation, but regardless of sex or the presence of behavioral or physical features associated with the fragile X phenotype. The potential benefit of screening for FMR2 alterations is an unexpected implication of the study and is worthy of continued exploration.

The FMR1 and FMR2 mutations are not common etiologies of academic difficulty among school-age children.

The prevalence of the fragile X mental retardation (FMR) 1 and FMR2 full mutations (fM) was examined among 1014 school-age children with academic difficulties but without mental retardation. Both Southern blot and polymerase chain reaction analyses for FMR1 and FMR2 were performed on samples obtained from these children. No fM genes were found, and one FMR1 premutation was identified. The distribution of allele sizes for both genes was comparable to those reported for other clinical and normal population samples. These results suggest that neither the FMR1 nor the FMR2 mutation is a common etiology of academic failure among school-age children without mental retardation and that the prevalence of the FMR1 premutation is no more frequent in children with academic failure than it is in the general population.

Persistent school dysfunction: unrecognized comorbidity and suboptimal therapy.

To determine reasons for continued school dysfunction in children previously diagnosed as having attention deficit hyperactivity disorder (ADHD) or enrolled in a special education program (spec. ed.), a retrospective chart review of patients referred for interdisciplinary evaluations at a tertiary center for hyperactivity and learning problems was completed. Interdisciplinary clinical recommendations were used to define reasons for treatment failure in 116 children with prior diagnosis of ADHD or spec. ed. placement. Results showed 45% of children enrolled in spec. ed. had previously undiagnosed ADHD. Thirty-one percent of those with ADHD, 55% of those in spec. ed., and 55% of those diagnosed with ADHD and in spec. ed. (Both) received a new educationally handicapping diagnosis. Psychiatric comorbidity was present in 28% of those with ADHD, 18% of those in spec. ed., and 23% of Both subjects. Thirteen percent of those in spec. ed. had significant coexisting medical conditions. Special education services were insufficient in 55% of children in spec. ed. and 55% of Both subjects. A significant difference (P < 0.01) in medication use was noted between the groups with 56% of the ADHD group, 55% of the Both group, and none of the spec. ed. group treated with medication. Of all subjects with ADHD, 76% were receiving insufficient or no medication. This review suggests the following: (1) Comorbidity in children with school dysfunction is frequently not recognized. (2) Educational therapy alone may not be sufficient treatment for school dysfunction, and in cases where the treatment program is failing, the appropriateness of the program should be reviewed. (3) ADHD is commonly seen in conjunction with other educationally handicapping conditions. Therefore, in cases of continuing school dysfunction, children previously diagnosed has having ADHD should be assessed for other educationally handicapping conditions; those previously diagnosed as educationally handicapped should be assessed for ADHD. (4) Suboptimal medication use may be associated with treatment failure.

CAT/CLAMS. A tool for the pediatric evaluation of infants and young children with developmental delay. Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale.

The American Academy of Pediatrics recommends regular developmental screening as a part of routine child health supervision. However, the pediatrician has a limited number of tools available to further evaluate a child who is found to be suspect or abnormal on a developmental screening test. The Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS) was therefore developed to provide pediatricians with a technique to assess infants and toddlers with suspected developmental delay. The CAT/CLAMS demonstrated strong psychometric properties. Concurrent validity with the Bayley Scales of Infant Development (BSID) was demonstrated in 43 children ages 12 to 19 months who were tested on three occasions with both instruments (correlation coefficient ranging between 0.63 and 0.87; P < .001). Predictive validity 6 and 12 months later was also demonstrated in this population with correlation coefficients ranging between 0.73 and 0.77, significant at the P = .001 level. Utilizing the CAT/CLAMS as part of the pediatrician's evaluation of children with developmental concerns would allow the pediatrician to compare language and nonlanguage problem-solving abilities and, therefore, aid in diagnosis and appropriate referral.

Learning disabilities.

Although the major manifestations of learning disabilities are expressed in the classroom, the pediatrician has several important roles to play. Identification may be achieved in the early school years by systematic observation of the child's neurodevelopment and school progress. Confirmation of the diagnosis is the most commonly assumed role. Interpretation and explanation of learning disabilities to the parents and child follow. The goals of treatment of learning disabilities are achievement of academic competence, treatment of associated deficits, and prevention of adverse mental health outcomes. The long-term relationship between the family and pediatrician facilitates the performance of longitudinal monitoring to ensure that the program is accomplishing its goals and that outcome is optimal.

A proposed formula for calculating energy needs of children with cerebral palsy.

This study compared two methods of calculating the energy needs of children with CP: the traditional method, using RDA for chronological age; and the Krick method, which calculates the BMR and includes factors for muscle tone, movement or level of activity, and energy requirements for normal and catch-up growth. 30 tube-fed children, aged between nine months and 18 years, who were inpatients for longer than one week at the Kennedy Institute, were reviewed. 14 were female. They were weighed at admission and discharge to evaluate the rate of growth; calorie prescriptions at discharge were based on the clinical course. The Krick method was found to be a more potent predictor of the discharge prescription than the RDA method.

Detection of young children in need of reading help. Evaluation of specific reading disability formulas.

To determine whether formula-based definitions of specific reading disability (SRD) were related to the actual classroom performance of children aged 7 1/2, the authors applied five definitions of SRD to a cohort of 243 children. The children were followed from birth and not selected on the basis of cognitive, reading, or academic performance criteria. The authors determined the concurrent validity of each of the formulas by comparing the prediction of each definition to whether the child received additional reading help in the classroom. Although the years behind method was significantly better than other approaches, no method identified more than 48 percent of the children who were given additional reading help. This study questions the ability of formula-based definitions adequately to identify young children with reading dysfunction.

Precursors of reading delay: neurodevelopmental milestones.

The relationship between current reading ability and the achievement of early language and motor developmental milestones was evaluated in 240 children, aged 7 1/2 years, whose language and motor achievement had been charted at each well baby visit during the first 2 years of life. Those children whose composite reading score was 6 months behind their chronologic age on the Woodcock-Johnson Psychoeducational Battery were classified as having reading delay. Relationships to reading outcome were assessed for individual infant milestones, for critical screening values, and by statistical techniques that characterized the developmental process rather than single milestones. Significant differences (P less than .05) were noted between children with and without reading delays for the following milestones: 4 to 6 words, 7 to 20 words, 50 words, 2-word sentences, and 5 and 8 body parts. The positive predictive value of slower milestone achievement ranged from 0% to 50%. Techniques that focused on the developmental process during the first 2 years (either rate of achievement of neurodevelopmental milestones or order of milestone acquisition) were better able to classify children with reading delay (sensitivity = .73, specificity = .78). Although the language milestone measures did not classify children sufficiently well to be diagnostic, the data served to determine whether a child would be at high risk based on performance rather than historical factors.

Infant stimulation curriculum for infants with cerebral palsy: effects on infant temperament, parent-infant interaction, and home environment.

To assess the effects of intervention in cerebral palsy, 48 infants 12 to 19 months of age, with mild to severe spastic diplegia, were randomly assigned to receive either 6 months of infant stimulation followed by 6 months of physical therapy (test group) or 12 months of neurodevelopmental physical therapy (contrast group). The infant stimulation protocol consisted of cognitive, motor, sensory, and language activities. Outcome was assessed after 12 months by using Carey Infant Temperament Questionnaire subscores (activity, rhythmicity, adaptability, approach, threshold, intensity, mood, distractibility, and persistence); Roth Mother-Child Relationship Evaluation subscores (acceptance, overprotection, overindulgence, rejection); and Home Observation for Measurement of the Environment subscores (maternal responsiveness, avoidance of restriction and punishment, organization of environment, play materials, maternal involvement, and variety of daily stimulation). Motor and cognitive outcomes suggesting advantage for the test group have been reported previously. After 12 months of intervention, mothers with infants in the contrast group showed a greater improvement in emotional and verbal responsiveness as measured by the Home Observation for Measurement of the Environment (mean score change in control group = 1.2, test group = 0.3 P less than .04). None of the 19 other measures differed significantly between treatment groups in change from baseline. This study demonstrates no short-term systematic effect on temperament, maternal-infant interaction, or home environment attributable to the inclusion of an infant stimulation curriculum in an intervention program for infants with spastic diplegia. It suggests that motor and cognitive advantages associated with infant stimulation are not mediated by measurable changes in the psychosocial variables studied.

Giftedness. Can it be predicted in infancy?

A cohort of 200 children, who were followed from birth and periodically evaluated as part of a longitudinal study of child development, was used to determine the ability of the Bayley Scales of Infant Development (BSID) to predict children who would be considered intellectually gifted at 7.5 years. The cohort was predominantly white (91%), upper middle class (70% in Hollingshead class 1 or 2), and had preschool educational experiences (94%). At outcome, 36 children had WISC-R verbal, performance, or full scale IQs greater than 135 and were called gifted. On average, gifted children walked 0.7 months earlier and spoke two-word sentences 2.2 months earlier than the non-gifted group. As a group, gifted children showed statistically significant, but clinically small, advantages for age of walking, age of speaking two-word sentences, BSID, and Stanford Binet IQ. Of the 36 children who later proved to be gifted, only two had BSID IQs greater than 134. Of the four children who had BSID IQs greater than 134, two maintained their superior function but 2 did not. A discriminant function analysis was used to predict giftedness for individual children based on BSID performance. The resulting classification achieved sensitivity = 0.69 and specificity = 0.39. Although groups of infants who will be gifted have higher BSID scores, the BSID cannot be endorsed as a method of identifying individual infants who will later demonstrate superior cognitive function.

The effects of physical therapy on cerebral palsy. A controlled trial in infants with spastic diplegia.

Legislatively mandated programs for early intervention on behalf of handicapped infants often stipulate the inclusion of physical therapy as a major component of treatment for cerebral palsy. To evaluate the effects of physical therapy, we randomly assigned 48 infants (12 to 19 months of age) with mild to severe spastic diplegia to receive either 12 months of physical therapy (Group A) or 6 months of physical therapy preceded by 6 months of infant stimulation (Group B). The infant-stimulation program included motor, sensory, language, and cognitive activities of increasing complexity. Masked outcome assessment was performed after both 6 and 12 months of therapy to evaluate motor quotient, motor ability, and mental quotient. After six months, the infants in Group A had a lower mean motor quotient than those in Group B (49.1 vs. 58.1, P = 0.02) and were less likely to walk (12 vs. 35 percent, P = 0.07). These differences persisted after 12 months of therapy (47.9 vs. 63.3, P less than 0.01, and 36 vs. 73 percent, P = 0.01, respectively). We noted no significant differences between the groups in the incidence of contractures or the need for bracing or orthopedic surgery. Group A also had a lower mean mental quotient than Group B after six months of therapy (65.6 vs. 75.5, P = 0.05). The routine use of physical therapy in infants with spastic diplegia offered no short-term advantage over infant stimulation. Because of the limited scope of the trial, our conclusions favoring infant stimulation are preliminary. The results suggest that further study of the effects of both physical therapy and infant stimulation is indicated.

Age of presentation in developmental disability.

It has not been determined whether severity of handicap or other associated factors are more important in determining the age of presentation for developmental disabilities. The relationship between age at presentation and referral source, presenting complaint, diagnosis, and associated factors (medical illness, motor signs, or behavioral disturbances) was examined in 738 consecutive children referred for developmental evaluation during 1982-1983. The nature of the complaint or diagnosis (motor, language, behavioral, or educational) was a far better predictor of age of presentation than the severity of the disorder. The degree of mental retardation did not affect age of presentation. Behavior problems did not affect the age of presentation for school failure or learning disability, but were associated with later presentation for motor delay, language delay, communication disorder, and within all IQ groups. The association of topography of handicap rather than severity with age of presentation should be considered when establishing or evaluating efforts at early identification of developmental disability.

Growth of severely impaired children: neurological versus nutritional factors.

Nineteen children with cerebral palsy who have had gastrostomies since 1981 and who have been followed for at least six months postoperatively were reviewed to assess the effects of gastrostomy feeding on their growth and to determine whether growth failure was due to neurological or nutritional dysfunction. The children's ages ranged from five to 168 months (mean 60.4 months) and follow-up extended from six to 41 months (mean 23.1 months). All were profoundly handicapped, with involvement of all four limbs. 11 also had seizures and all 19 were severely to profoundly retarded. Heights and weights were recorded during the visit immediately before surgery and at most recent follow-up. Data were recorded as a standard deviation score (Z-score). Before surgery the mean weight/height was -2.71 Z. Postoperatively this improved to -1.18 Z. 16 of the 19 children increased their weight/height ratio. Three children had a weight/height ratio greater than 10th percentile preoperatively. 11 achieved this on follow-up. Seven children were greater than 25th percentile for weight/height. Improvement in weight/height did not correlate with length of follow-up. For severely neurologically impaired children, nutritional factors play a major rôle in growth. By ensuring adequate caloric intake via gastrostomy, a significant number of children achieved adequate growth and better nutritional status.