The early detection of mental retardation.
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Biomedical subjects
Publications and source records attributed to A J Capute.
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The Clinical Linguistic and Auditory Milestone Scale (CLAMS) and Bayley Scales of Infant Development (BSID) were independently administered to 43 children with mild-to-moderate motor delay at 15, 21, and 27 months of age. Highly significant correlations were noted for each examination. Sex, race, and age at time of examination did not significantly influence the relationship between a CLAMS quotient and a Bayley ratio quotient (BRQ). On the first examination gestational age exerted an effect that carried over to the total population. The initial CLAMS quotient also correlated with the results of the CLAMS and BSID administered 12 months later. The CLAMS identified children with a delayed BRQ (less than 70) as follows: sensitivity, 0.66; specificity, 0.79; predictive value of a positive test, 0.80; and predictive value of a negative test, 0.65. The CLAMS provides the pediatrician with a language assessment instrument that adequately detects cognitive deficits in motor-delayed children.
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.
At each well-child examination between birth and two years, parents of 448 infants were questioned about their child's age at attainment of 25 linguistic and auditory milestones. Parental reports were compared with the results of independently administered Bayley Mental Developmental Index (MDI) at one year of age. Parental recall of information was high, ranging from 70 to 99 per cent for 21 of the 25 milestones. The milestone performance of infants with normal MDI scores showed an orderly, sequential progression of expressive and receptive language. Across the entire population studied, the correlation between milestone attainment and MDI was statistically significant for 24 of the 25 milestones, and later attainment of milestones correlated with lower MDI. As a group, 'delayed' infants (MDI less than 68) attained milestones significantly later than 'average' infants (MDI 85 to 116) for 20 of 25 items. Attention to linguistic and auditory milestones early in infancy can contribute to the early detection and diagnosis of mental retardation and disorders of communication.
The early auditory and visual abilities of 47 extremely premature infants (31 born less than or equal to 28 weeks gestation) were assessed with a bell, a light and an optokinetic nystagmus drum. All the infants altered to the bell and blinked to the light from 25 weeks postconceptional age (PCA) and beyond. A few infants at first had only a change in heart rate or respiratory rate in response to the bell, or required a high-intensity light to elicit a blink. The majority appeared to habituate to the bell and light during their first examination at one week of age. None of the infants blinked in response to a threatening gesture. Optokinetic nystagmus could be elicited as early as 30 weeks PCA, could be elicited in the majority by 36 weeks PCA, and universally by term (40 weeks PCA). The responses of 15 fullterm newborn infants were not significantly different from those of the preterm infants at term. Alerting to a bell, blinking to light and habituation to both are simple bedside maneuvers for assessing extremely premature infants less than 30 weeks PCA. Optokinetic nystagmus may be useful in assessing the visual abilities of premature infants closer to term.
A case of acquired epileptic aphasia presenting as language delay and hyperactivity is presented and clinical aspects reviewed. Acquired epileptic aphasia is a diffuse neurologic process of unknown etiology that presents in preschool children. Although progressive language loss and seizures are the major manifestations, deficits in attention and socialization may be noted. The electroencephalogram is usually diagnostic. While almost all (80%) children develop seizures, approximately half present as language delay without seizures. Recognition of the progressive and global nature of the language dysfunction may permit earlier diagnosis and intervention.
A longitudinal study describes the pattern of appearance of eight primitive reflexes in a population of 47 viable extremely premature infants, beginning as early as 25 wk postconceptional age (PCA). Infants were examined weekly, from 1 wk of age until discharge from the neonatal intensive care unit. Primitive reflexes were graded as to completeness and intensity of response. Three patterns emerged: the upper and lower extremity grasp reflexes were present in all premature infants, from 25 wk and beyond, the Moro, asymmetric tonic neck reflex and Galant (lateral trunk incurvature reflex) were present in some premature infants as early as 25 wk PCA, and in the majority by 30 wk PCA, and the lower extremity placing, positive support, and stepping were occasionally present prior to 30 wk PCA, yet were not uniformly present and/or complete even at term. In each case, the primitive reflex became stronger, more complete, more consistently elicited and more prevalent with increasing postconceptional age. The pattern of primitive reflexes in the premature infant at term (40 wk PCA) is similar to that of full-term newborns. Sequential assessment of the primitive reflexes may be a useful method of evaluating extremely premature infants prior to term.
While motor milestones--the neurodevelopmental functional end point of the transitioning of the immature and mature primitive reflexes into volitional activity--have been highlighted in predicting future motor function, the primitive reflexes represent the earliest neurodevelopmental markers available for study. By pediatricians becoming familiar with their quantitative and qualitative aspects coupled with the time of their appearance and suppression, they will have this neuromotor tool available for the early detection of a significant motor handicap. Primitive reflexes II have been highlighted since they are available at birth to be clinically evaluated and followed during sequential office visits during the first 6 months of life, the time during which infants are more closely followed at office visits. Delay or deviancy (nonsequential appearance) of motor milestones are preceded by an exaggeration or delayed suppression of the primitive reflexes. It is anticipated that the study and standardization of an intrauterine primitive reflex profile will eventually lead to the identification of a high-risk fetus. This endeavor will be fostered by the further development and refinement of non-invasive roentgenographic techniques.
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.
A motor quotient was calculated by dividing the age of a child's best motor achievement by his or her chronologic age. A quotient less than 50 was said to predict gross motor delay (walking at or later than 24 months). Applying the quotient to 144 8- to 18-month-old children yielded a sensitivity of 87%, specificity of 89%, and overreferral and underreferral rates of 12%. By viewing motor development as a ratio, one is able to forecast motor delay.
The ages at attainment of 12 gross motor milestones were obtained prospectively during well-baby visits in the first two years of life for 381 children. All had been born at term and were judged to be normal at one year. A longitudinal analysis, using an index summarizing each child's progress for eight selected milestones, is reported for the 284 children for whom data were complete. A high percentage of parents were able to report the age at attaining milestones with an acceptable degree of variability. The children attained milestones at earlier ages than traditionally reported. There were only minor sex differences in age at attainment, but black children attained milestones earlier than white children.
The neurodevelopmental examination is a pediatric approach to developmental diagnosis that relies on evaluation of multiple streams of development. The value of the neurodevelopmental examination for the early diagnosis of cerebral palsy and mental retardation was studied by a retrospective analysis of prospectively (longitudinally) collected data. Both conditions were found to be accurately diagnosed in the first year of life. For cerebral palsy, sensitivity was 0.81 and specificity 1.00; for mental retardation, sensitivity was 0.56 and specificity 0.96. The trained pediatrician can make early diagnoses of developmental disabilities.
The relationship of gross motor development to later cognitive status was assessed by comparing the ages of attainment of four early milestones to later performance on the Stanford Binet. The ages of rolling supine to prone, sitting alone, crawling, and walking were collected in a prospective fashion via parental report for 213 Caucasian children. Stanford Binet IQ was measured at 3 years. Significant but low-order correlations were noted for age of milestone and Stanford Binet IQ. Stratifying the sample by IQ standard deviation groups revealed significant relationships, but this was largely the result of the difference between the extreme groups (117 vs. 85). Children with earlier ages of milestone attainment did not have higher IQs on average. The association of gross motor development and cognition is not strong enough to allow the use of one to predict the other.
This report describes quantitative standardization data on nine primitive reflexes for a cohort of 381 normal infants evaluated longitudinally at each visit between birth and two years of age. Normality was confirmed by the use of the Bayley Scales of Infant Development at one year of age. The standardization of this new examination technique complements the traditional infant neurological examination and may allow primitive reflexes to become a useful adjunct to the prediction of motor disability in early infancy.
Although much is known about autism, misconceptions persist. A case report is presented and used as a framework for dispelling some common myths about the condition. A simple, practical model for understanding autism is proposed and recommendations are provided for the practitioner managing a child with autistic-like features.
Currently the diagnosis of Specific Learning Disability (SLD) requires the demonstration of academic underachievement relative to cognitive potential. However, if the focus is shifted from academic underachievement to the detection of the deviant neurologic substrate, then the potential exists for diagnosing SLD prior to school. Circumstantial evidence from a variety of sources--studies of historical risk, the newborn examination, assessment of newborn behaviors, combination of newborn and subsequent examination, retrospective assessment of early development in SLD children, and aspects of infant development (motor or language) and SLD-suggests that the neurologic substrate for SLD can be identified in infancy. Early identification of SLD will permit early intervention when indicated, aid the assessment of therapeutic efficacy, and facilitate the evaluation of other interventions (e.g., neonatal care).
Age levels for independent walking were compared for groups of children with varying degrees of intellectual impairment, but without other neurologic handicaps. All walked independently, save for 13.5 per cent of the profoundly retarded. Although mental retardation was associated with a delay in walking, subgroups existed at all levels that walked at a normal age. This suggests that the cognitive level is not the sole determinant for the achievement of this milestone.