Thomas A. Blondis

OPHTHALMIC DISORDERS IN CHILDREN WITH DOWN SYNDROME

The goal of this study was to determine the frequency of ophthalmic disorders in a group of young children with Down syndrome who were unselected for ophthalmic abnormalities, and to determine whether examination by a pediatric ophthalmologist should become standard practice. Of 77 children referred to a Down syndrome clinic at a teaching hospital and evaluated by a pediatric ophthalmologist, 61 per cent had ophthalmic disorders needing treatment and monitoring. Furthermore, the percentage of children with ophthalmic disorders increased with age, from 38 per cent in the two‐ to 12‐month‐old group to 80 per cent in the five‐ to 12‐year‐old group. The authors conclude that children with Down syndrome should be evaluated by a pediatric ophthalmologist in the first six months of life and annually thereafter.

Asperger Syndrome Associated with Steinert's Myotonic Dystrophy

Steinerts myotonic dystrophy was diagnosed in a 10‐year‐old girl with Asperger syndrome. This form of myotonic dystrophy is one of a growing list of genetic diseases that can be diagnosed by analysis of a gene with trinucleotide repeats that may expand. The patient has the largest such expansion known within her pedigree. She has many of the psychological deficits that have been reported in adult‐onset myotonic dystrophy. This is the first known report of an association of this disorder with Asperger syndrome. Recently Moebius syndrome has been found in association with disorders of the autistic spectrum; physicians practicing developmental medicine should now also consider Steinerts myotonic dystrophy when evaluating children with facial diplegia.

Early Maturation of Motor-Delayed Children at School Age

Children underwent motor and sensory testing during each of 3 consecutive years beginning at kindergarten entrance. Children were assigned to either a motor-normal or motor-delayed group based on results from testing during the 1st year of the study. Motor-normal subjects (n = 17) had standard scores on a global motor-sensory test that fell within one standard deviation of the mean. Motor-delayed subjects (n = 12) had a global motor-sensory test standard score indicating greater than one standard deviation of motor delay and, in addition, were delayed by a minimum of one standard deviation on four of five of the motor-sensory test subtests. At the conclusion of the 3-year study, the mean standard score of the motor-delayed group remained greater than one standard deviation below the performance of the motor-normal group as measured by the global motor-sensory test. Statistical analysis using repeated measures analysis of variance indicated that the motor-delayed group made gains but did not close the gap between themselves and the motor-normal group during the 3-year study. Group x time interaction effects showed that during the study, significant gain was made by the motor-delayed group on the motor coordination subtest of the motor-sensory test. The study supports the conclusion that motor-delayed children without early intervention fail to outgrow their motor delays by age 8 years. (J Child Neurol 1993;8:323-329).

Measures of Attention Deficit Part I: Questionnaires

Despite much progress in the diagnosis and management of children with attentional problems, hyperactivity, and learning difficulties, current diagnostic techniques remain inadequate to readily distinguish a primary attentional disorder from a more complex secondary problem. Developmental and behavioral questionnaires to supplement office evaluation of hyperactivity, neuromaturation, and selected cognitive processes continue to be improved. These measures suffer from different biases, focus on different childhood characteristics and associated problems, and approach childhood interaction with the environment differently. Several are psychometrically more sound, and several provide the physician with developmental and social information that may help improve the habilitative prescription. The general pediatrician can use these questionnaires to complement his or her evaluation and follow-up of children who present with learning and behavioral problems. Prior to choosing an instrument, the clinician should review the advantages of each and choose the one most suited to his or her practice.

Measures of Attention Deficit Part II: Clinical Perspectives and Test Interpretation

After a detailed behavioral and developmental history has been supplemented by the use of formal questionnaires, the diagnosis of an attentional disorder can be further clarified by the judicious selection of psychoeducational and pediatric physical and neurodevelopmental examinations. The most common difficulty in the interpretation of psychoeducational test subscores and profiles is the significant overlap between the patterns generated by attentional deficits and those associated with memory-based learning disabilities. Certain other medical and behavioral conditions need to receive careful consideration prior to finalizing the diagnosis of Attention Deficit Hyperactivity Disorder (ADHD). A thorough but focused general examination coupled with the pediatric neurodevelopmental examination can contribute to this differentiation.

Developmental Disabilities: A Continuum

degree of such brain malformation or damage. Child development is a primary marker of neurologic integrity. The four principal domains of development involve motor skills (gross and fine), language skills (receptive and expressive), problem solving ability, and adaptive skills (emotional, social, and selfhelp) .2,3 A child’s failure to follow established sequences of age-anticipated milestones in any of these developmental areas should be noted and analyzed as part of the pediatric examination. Neurodevelopmental pathology can be described along two distinct but related axes: the spectrum of developmental diagnoses and the continuum of associated dysfunction.’ The spectrum represents a related group of chronic non-progressive CNS conditions. The continuum reflects the additional CNS

UNDERDIAGNOSIS AND OVERDIAGNOSIS OF ADHD: DIAGNOSTIC ACCURACY OF BEHAVIORAL RATINGS AND UTILITY OF PSYCHOMETRIC TESTING. 109

UNDERDIAGNOSIS AND OVERDIAGNOSIS OF ADHD: DIAGNOSTIC ACCURACY OF BEHAVIORAL RATINGS AND UTILITY OF PSYCHOMETRIC TESTING. 109