Tone and reflex development before term.

The evolution of tone and reflexes from 25 weeks postmenstrual age (gestational age plus chronologic age) to term in a population of 42 surviving infants is described. The infants were born in 1983 at the Johns Hopkins Hospital, had birth weights less than 1300 g, were examined weekly until neonatal intensive care unit discharge, and did not develop cerebral palsy. Lower-extremity flexor tone was first detectable at 29 weeks post-menstrual age by the popliteal angle and heel to ear maneuvers. Flexor tone, recoil, and hyperreflexia were all noted 2 to 3 weeks earlier in the lower extremities (33 to 35 weeks) than in the upper extremities (35 to 37 weeks). Hip tone (35 to 37 weeks) followed knee flexor tone, but preceded shoulder tone (37 to 38 weeks). Trunk tone on ventral suspension emerged closer to term (36 to 40 weeks), and more than half of infants evaluated at term continued to demonstrate head lag when pulled to sitting position. The emergence of the primitive and pathologic reflexes reflects (both in timing and pattern) the evolution of tone: development of the reflexes in the lower extremities precedes that of those in the upper extremities, and development of the distal reflexes precedes that of the proximal. Maturation of tone, deep tendon reflexes, pathologic reflexes, and primitive reflexes occurs in an orderly, sequential manner, with a well-defined pattern: caudocephalad (lower extremities to upper extremities) and centripetal (distal to proximal).

Neonatal neurodevelopmental examination as a predictor of neuromotor outcome in premature infants.

There was a marked correlation (P less than .000001) between neonatal neurodevelopmental examination results and neuromotor outcome at 1 year of age or older in 210 high-risk premature infants (mean birth weight 1,107 g, mean gestational age 28.4 weeks). This neonatal examination consisted of assessment of posture, extremity and axial tone, deep tendon reflexes, pathologic reflexes, primitive reflexes, symmetry, oromotor function, cranial nerve function, auditory and visual responses, and behavior. Premature infants whose neonatal neurodevelopmental examination results were abnormal had significantly higher incidences of both cerebral palsy (38% v 6%, P less than .000001) and minor neuromotor dysfunction (27% v 13%, p less than .05) than did premature infants whose examination results were normal. This correlation continued to be highly significant even with the analysis of subgroups (infants born at or before 27 weeks' gestation, infants with chronic lung disease discharged with oxygen supplementation, infants with periventricular hemorrhage) and when a variety of individual perinatal, demographic, and social variables were used as controls. Normal or nearly normal neonatal neurodevelopmental examination results can be used to reassure parents of high-risk premature infants. Although abnormal neonatal neurodevelopmental examination results cannot be used to diagnose handicap in premature infants, they can be used to select a group of high-risk infants who should be carefully monitored during infancy and childhood.

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.

MRI in motor delay: important adjunct to classification of cerebral palsy.

Cranial magnetic resonance imaging (MRI) was performed prospectively in 45 children (ages 3-27 months) with clinically documented motor delay to evaluate the ability of MRI to determine etiologic factors, to determine whether myelination correlated with motor delay, and whether the clinical category corresponded with the imaging findings. Of the 22 children diagnosed clinically as having major motor delay (i.e., cerebral palsy), 77% had magnetic resonance imaging abnormalities. In 23%, etiologic associations were established from MRI alone and in 32% a clinically suspected etiology was supported. No children had myelination delay as the sole abnormality. In 23 children with minor motor delay, only 17% had abnormal scans. Clearly, MRI provided useful information in the majority of children with cerebral palsy; therefore, a classification system is proposed in which MRI can be used in conjunction with clinical assessment to specify more precisely the etiologic factors in cerebral palsy.

Primitive reflex profile. A pilot study.

Seven primitive reflexes used by physical and occupational therapists in evaluating children with cerebral palsy were each graded on a 0 to 4+ scale to constitute a Primitive Reflex Profile. The reflexes studied were the asymmetrical tonic neck reflex, the symmetrical tonic neck reflex, the tonic labyrinthine reflex, the positive support reflex, the derotational righting reflex, the Moro reflex, and the Galant reflex. The Primitive Reflex Profile was studied in 53 cerebral palsied patients to assess both the feasibility of its administration and its usefulness in discriminating functional levels of ambulation. The Primitive Reflex Profile was administered by at least two members of a team consisting of four pediatric developmentalists and two physical therapists. In using this instrument, the extreme functional groups were clearly defined and showed the expected overlap with the intermediate classification.

A pediatric overview of the spectrum of developmental disabilities.

The developmental disabilities are specific diagnoses on a spectrum of chronic neurologic handicaps. Complete diagnosis and habilitation planning demands awareness of the underlying continuum of cerebral dysfunction common to all the disabilities. Careful attention to this continuum can result in the recognition of critically important associated deficits. Developmental dissociation is a useful developmental phenomenon in the early diagnosis of a particular disability. Developmental deviancy is an aid in early detection of milder disabilities or associated deficits. Professionals familiar with the entire spectrum are necessary to provide optimal care for developmentally disabled children.

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.

Dyslexia: initial assessment and outcome.

The initial multidisciplinary evaluations on 40 dyslexic children were reviewed in an attempt to identify factors which might favor a positive outcome from educational remediation. The children manifested a high incidence of dysarticulation, impaired auditory discrimination and visual motor perceptual delays. All of the subjects exhibited progress at a constant rate (reading quotient) while enrolled in a full-time remedial program for dyslexic children. Factors predictive of significant improvements could not be identified. The hypothesis that brighter dyslexic children improve more in a remedial setting was not supported. Initial mathematics quotients, however, were higher and demonstrated more improvement than initial reading quotients in all groups. Implications for educational policy and pediatric practice are discussed.

Extrapyramidal cerebral palsy: a changing view.

Earlier surveys of cerebal palsy characterized patients with extrapyramidal (choreoathetoid) cerebral palsy as having little cognitive impairment and few associated deficits. Anoxia, which has now replaced neonatal hyperbilirubinemia as a major cause of this type of cerebral palsy, predictably produces a diffuse type of brain damage. Evaluations of children with extrapyramidal cerebral palsy seen in a pediatric habilitation center revealed high incidences of mental retardation, epilepsy, and other associated deficits. These findings have implications for the management and habilitation of children with extrapyramidal cerebral palsy.

Issues in the early identification of specific learning disability.

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).

Spectrum of developmental disabilities: continuum of motor dysfunction.

The spectrum of developmental disabilities encompasses a diverse group of interrelated disorders with multiple manifestations resulting from brain dysfunction. Motor disability has been highlighted, as it is the most frequent presenting sign of developmental disability. Such disability may vary from severe to minimal. Severe disability, or cerebral palsy, is well appreciated, as are the additional associated disabilities that affect habilitation. Mild to minimal motor disability (mild to minimal cerebral palsy), because it is usually nonhandicapping, is less frequently appreciated as a marker of brain dysfunction. Further delineation of the child's nonmotor abilities is necessary in order to define the scope of the disability. Cognitive limitation is not an important cause for the gross motor delay seen in children with mental retardation. It is our postulation that the motor deficit is on the basis of mild to minimal cerebral palsy. This diagnosis is frequently obscured by the mental retardation.

Understanding autism.

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.

Linguistic and auditory milestones during the first two years of life: a language inventory for the practitioner.

Although language is recognized as one of the best predictors of development, the lack of a simple evaluation tool has contributed to the general pediatrician's unfamiliarity with linguistic milestones. Here described is a 32-item instrument for the recording and assessment by the practicing pediatrician of language milestones during the first two years of life.

Age of walking in the cognitively impaired.

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.

Using the clinical linguistic and auditory milestone scale for developmental screening in high-risk preterm infants.

Eighty-one preterm infants (mean gestational age 29 weeks, range 24-36 weeks) discharged from The Johns Hopkins Hospital Neonatal Intensive Care Unit were followed up sequentially from birth to 2 years of age by use of the Clinical Linguistic and Auditory Milestone Scale (CLAMS) to evaluate language development. Children were studied during three time intervals: Interval 1: 3-5 months chronologic age (CA); Interval 2: 9-14 months (CA); and Interval 3: 18-24 months (CA). Psychometric test scores were compared with CLAMS Language Quotients (LQ) by use of full, partial (75%, 50%, 25%), and no "correction" for weeks of prematurity to determine whether "correcting" for prematurity would yield a more accurate estimate of eventual cognitive outcome. CLAMS LQ at Interval 1 was highly correlated with CLAMS LQ at Interval 2 and CLAMS LQ at Interval 2 correlated well with CLAMS LQ at Interval 3 (r = 0.57 and 0.64, respectively, P = 0.0001). Correlations indicated that there was an orderly, sequential development of language in the preterm infant. CLAMS evaluations correlated significantly with psychometric test results during Interval 2 and Interval 3 (r = 0.34, P < 0.02 and r = 0.75, P = 0.0001, respectively). The CLAMS proved to be a useful instrument for monitoring preterm language development in the primary pediatric care setting.

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.

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.

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.

Acquired epileptic aphasia. Diagnostic aspects of progressive language loss in preschool children.

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.