Kytja K. S. Voeller

Intensive Remedial Instruction for Children with Severe Reading Disabilities: Immediate and Long-term Outcomes From Two Instructional Approaches

Sixty children with severe reading disabilities were randomly assigned to two instructional programs that incorporated principles of effective instruction but differed in depth and extent of instruction in phonemic awareness and phonemic decoding skills. All children received 67.5 hours of one-to-one instruction in two 50-minute sessions per day for 8 weeks. Both instructional programs produced very large improvements in generalized reading skills that were stable over a 2-year follow-up period. When compared to the growth in broad reading ability that the participants made during their previous 16 months in learning disabilities resource rooms, their growth during the intervention produced effect sizes of 4.4 for one of the interventions and 3.9 for the other. Although the childrens average scores on reading accuracy and comprehension were in the average range at the end of the follow-up period, measures of reading rate showed continued severe impairment for most of the children. Within 1 year following the intervention, 40% of the children were found to be no longer in need of special education services. The two methods of instruction were not differentially effective for children who entered the study with different levels of phonological ability, and the best overall predictors of long-term growth were resource room teacher ratings of attention/behavior, general verbal ability, and prior levels of component reading skills.

Attention Deficit- Hyperactivity Disorder and Asymmetry of the Caudate Nucleus:

The neurologic basis of attention deficit-hyperactivity disorder (ADHD) is poorly understood. Based on previous studies that have implicated metabolic deficiencies in the caudate-striatal region in ADHD, we employed magnetic resonance imaging to investigate patterns of morphology of the head of the caudate nucleus in normal and ADHD children. In normal children, 72.7% evidenced a left-larger-than-right (L > R) pattern of asymmetry, whereas 63.6% of the ADHD children had the reverse (L < R) pattern of asymmetry of the head of the caudate nucleus. This reversal of normal asymmetry in ADHD children was due to a significantly smaller left caudate nucleus. The reversal in asymmetry of the head of the caudate was most notable in ADHD males. These results suggest that normal (L > R) morphologic asymmetry in the region of the caudate nucleus may be related to asymmetries observed in neurotransmitter systems implicated in ADHD. The behavioral symptoms of ADHD may reflect disinhibition from normal levels of dominant hemispheric control, possibly correlated with deviations in asymmetric caudate-striatal morphology and deficiencies in associated neurotransmitter systems. (J Child Neurol 1993;8:339-347).

Allan-Herndon-Dudley Syndrome and the Monocarboxylate Transporter 8 (MCT8) Gene

Allan-Herndon-Dudley syndrome was among the first of the X-linked mental retardation syndromes to be described (in 1944) and among the first to be regionally mapped on the X chromosome (in 1990). Six large families with the syndrome have been identified, and linkage studies have placed the gene locus in Xq13.2. Mutations in the monocarboxylate transporter 8 gene (MCT8) have been found in each of the six families. One essential function of the protein encoded by this gene appears to be the transport of triiodothyronine into neurons. Abnormal transporter function is reflected in elevated free triiodothyronine and lowered free thyroxine levels in the blood. Infancy and childhood in the Allan-Herndon-Dudley syndrome are marked by hypotonia, weakness, reduced muscle mass, and delay of developmental milestones. Facial manifestations are not distinctive, but the face tends to be elongated with bifrontal narrowing, and the ears are often simply formed or cupped. Some patients have myopathic facies. Generalized weakness is manifested by excessive drooling, forward positioning of the head and neck, failure to ambulate independently, or ataxia in those who do ambulate. Speech is dysarthric or absent altogether. Hypotonia gives way in adult life to spasticity. The hands exhibit dystonic and athetoid posturing and fisting. Cognitive development is severely impaired. No major malformations occur, intrauterine growth is not impaired, and head circumference and genital development are usually normal. Behavior tends to be passive, with little evidence of aggressive or disruptive behavior. Although clinical signs of thyroid dysfunction are usually absent in affected males, the disturbances in blood levels of thyroid hormones suggest the possibility of systematic detection through screening of high-risk populations.

Attention deficit disorder in children A neglect syndrome

This study tests the hypothesis that children with attention deficit disorder (ADD) resemble adults with inattention and neglect secondary to right hemisphere dysfunction. A letter cancellation task was administered to seven boys with ADD and five controls. As a group, the subjects with ADD made significantly more overall errors of omission and left-sided errors than controls, suggesting that they resemble adults with right hemisphere dysfunction.

Phonological Awareness Training and Remediation of Analytic Decoding Deficits in a Group of Severe Dyslexics.

The goal of the present study was to evaluate the effectiveness of the Auditory Discrimination in Depth Program (ADD) in remediating the analytic decoding deficits of a group of severe dyslexics. A group of ten severely dyslexic students ranging in age from 93 to 154 months were treated in a clinic setting for 38 to 124 hours (average of 65 hours). Pre- and post-treatment testing was done with the Woodcock Reading Mastery Test and the Lindamood Auditory Conceptualization to assess changes in phonological awareness and analytic decoding skills. Results revealed statistically significant gains in phonological awareness and analytic decoding skills.

On defining Sydenham's chorea: Where do we draw the line?

Sydenhams chorea (SC) is a major manifestation of rheumatic fever characterized by an array of neuropsychiatric symptoms that vary in severity, timing, and character. Some of the same symptoms are seen in Tourettes syndrome and childhood-onset obsessive-compulsive disorder. Genetic vulnerability appears to play a role in all three conditions. The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcus) has been introduced to describe a putative subset of obsessive-compulsive disorder and Tourettes syndrome that bears some resemblance to Sydenhams chorea. This article discusses whether PANDAS should be subsumed under Sydenhams chorea, thus expanding the diagnostic boundaries of Sydenhams chorea to include primarily neuropsychiatric presentations now classified as cases of obsessive-compulsive disorder or Tourettes syndrome. We conclude that PANDAS is a useful construct, but that it would be premature to view it as a subset of Sydenhams chorea-whether defined narrowly or broadly.

XLMR syndrome characterized by multiple respiratory infections, hypertelorism, severe CNS deterioration and early death localizes to distal Xq28

We report on a family with severe X-linked mental retardation (XLMR) and progressive, severe central nervous system deterioration. Three of the five affected males died of secondary complications before the age of 10 years and none have survived past the age of 10. These complications included swallowing dysfunction and gastroesophageal reflux with secondary recurrent respiratory infections. In addition, hypotonia and a mild myopathy were also present. All had a characteristic facies, including downslanting palpebral fissures, hypertelorism, and a short nose with a low nasal bridge. The two older boys showed cerebral atrophy by CT. No metabolic abnormalities were identified. Three obligate carriers had an IQ less than 80. The causal gene has been localized distal to DXS8103 in Xq28, a region spanning 5cM. No other XLMR disorder with these manifestations have been localized to this region and this appears to be a new disorder.

Toward a neurobiologic nosology of attention deficit hyperactivity disorder.

From the Department of Psychiatry and the Division of Pediatric Neurology, Department of Pediatrics, University of Florida School of Medicine, Gainesville, FL. Address correspondence to Dr Kytja K.S. Voeller, Department of Psychiatry, Box J234, JHMHC, Gainesville, FL 326100234. this special supplement to the Journal of Child Neurology is devoted to attention deficit hyperactivity disorder (ADHD), as it is currently called. There is an enormous literature on this topic. Barkley’ pointed out a decade ago that there were some 2000 articles on this subject, and the number has undoubtedly increased exponentially since that time. Hopefully, this special issue presents a fresh look at an old subject. The clinical section includes articles by Bennett and Sally Shaywitz, who discuss comorbidity and Weinberg and Emslie, who review the complex issues of differential diagnosis. Hynd and colleagues present information about the cognitive profile of children with attention deficit disorder with and without hyperactivity. Martha Denckla discusses the manifestations of ADHD in the adult. I

When's a Smile a Smile? Or How to Detect a Message by Digitizing the Signal

Whats in a smile? Why do we respond so powerfully to this visual display? Or, phrased in information processing terms: What characteristics of the signal define the boundaries of the social message? We have used digital image analysis and subject ratings to answer this question for videotaped smiles. A simple measure of the information provided by facial movement—the entropy of the distribution of pixel intensities in the subtracted or difference images—traced the changing facial expression (the signal) through time. Raters categorized the individual videoprints in order to locate message boundaries. We found a remarkable coincidence between changes in signal entropy and message. In each smile, rapid increases in positive messages occurred within entropy crests. We conclude that quantitative measures of information transmission can be used to locate message boundaries and provide insight into how receivers parse the behavioral stream.

Altered neurodevelopment associated with mutations of RSK2: a morphometric MRI study of Coffin–Lowry syndrome

Coffin–Lowry syndrome (CLS) is a rare form of X-linked mental retardation caused by mutations of the RSK2 gene, associated with cognitive impairment and skeletal malformations. We conducted the first morphometric study of CLS brain morphology by comparing brain volumes from two CLS families with healthy controls. Individuals with CLS consistently showed markedly reduced total brain volume. Cerebellum and hippocampus volumes were particularly impacted by CLS and may be associated with specific interfamilial RSK2 mutations. We provide preliminary evidence that the magnitude of hippocampus volume deviation from that of controls may predict general cognitive outcome in CLS.