Adrenoleukodystrophy. Clinical-pathologic case history and literature review]

This article presents the clinical and pathological history of a 10-year-old boy with adrenoleukodystrophy as well as a tabular survey of 17 additional cases of which the necropsy records were published in the literature. So far as the case of adrenoleukodystrophy directly observed by the authors themselves is concerned, commencement of the disease was in the seventh year of life, with symptoms of Addison's disease and diffuse inflammatory cerebral sclerosis being observed at about the same time. The cerebral disease manifested itself as a progressive pseudobulbar and pyramidal tract symptomatology and as a progressive cerebral psychosyndrome reminiscent of an endocrinal psychosyndrome. Morphologically, the adrenoleukodystrophy was characterized by severe dystrophy of the cortex of the suprarenal gland and the brain are considered the points of action of a still unknown noxa.e suprarenal gland as well as by extensive sudanophilic processes of decomposition in the cerebromedullary substance, putamen, pallidum, and thalamus. Striated cells and cytoplasmic filamentous inclusions observed in the brain and suprarenal glands in a number of cases reported in the literature were not found by the present authors. The cortex of th     

The role of glutamate in anxiety and related disorders

Anxiety, stress, and trauma-related disorders are a major public health concern in the United States. Drugs that target the gamma-aminobutyric acid or serotonergic system, such as benzodiazepines and selective serotonin reuptake inhibitors, respectively, are the most widely prescribed treatments for these disorders. However, the role of glutamate in anxiety disorders is becoming more recognized with the belief that drugs that modulate glutamatergic function through either ionotropic or metabotropic glutamate receptors have the potential to improve the current treatment of these severe and disabling illnesses. Animal models of fear and anxiety have provided a method to study the role of glutamate in anxiety. This research has demonstrated that drugs that alter glutamate transmission have potential anxiolytic action for many different paradigms including fear-potentiated startle, punished responding, and the elevated plus maze. Human clinical drug trials have demonstrated the efficacy of glutamatergic drugs for the treatment of obsessive-compulsive disorder, posttraumatic stress disorder, generalized anxiety disorder, and social phobia. Recent data from magnetic resonance imaging studies provide an additional link between the glutamate system and anxiety. Collectively, the data suggest that future studies on the mechanism of and clinical efficacy of glutamatergic agents in anxiety disorders are appropriately warranted.     

Issues related to the diagnosis and treatment of autism spectrum disorders

This paper explores issues and implications for diagnosis and treatment, stemming from the growing number of children identified with autism spectrum disorders (ASDs). Recent developments and innovations in special education and Medicaid programs are emphasized. Eligibility determination policies, innovations in diagnostic practices, the cost and financing of assessment, variability among programs in diagnostic criteria, and racial/ethnic disparities in the timing of diagnosis all influence the capacity of service systems to provide diagnoses in a timely, coordinated, accurate, economical, and equitable manner. There are several barriers to the more widespread provision of intensive intervention for children with ASDs, including lack of strong evidence of effectiveness in scaled-up public programs, uncertainty about the extent of obligations to provide services under the Individuals with Disabilities Education Act, high cost of intervention, and variability among states in their willingness to fund intensive intervention via Medicaid. Innovative policy experiments with respect to financing intensive intervention through schools and Medicaid are being conducted in a number of states.     

Factors related to eating disorders in young adolescent girls

The objective of this study was to identify factors related to eating disorders in young adolescent girls. Three hundred eighty-five girls who were attending a career conference at a midwestern university completed the Eating Disorder Instrument (EDI) along with a biographical data sheet. The EDI is designed for the assessment of psychological and behavior traits common in eating disorders. While 81% of the young subjects were assessed to be within the range for ideal weight or were underweight, 78% preferred to weigh less. Only 14% were satisfied with their current weight. Findings for each of the subscales used revealed significant differences among the girls based on intact versus broken family; subjects' actual and preferred weight; whether the family ate meals together; average grades; age and grade in school; fathers' occupation; future career plans; place of residence; and feelings toward their mothers.     

Abnormal optokinetic and perceptual span parameters in cerebellar-vestibular dysfunction and related anxiety disorders

As prior studies indicated abnormal cerebellar-vestibular-based sensorimotor mechanisms and neurological and ENG diagnostic parameters in anxiety disorders and because ocular fixation and sequential scanning are cerebellar-vestibular-modulated, it appeared reasonable to measure these and related ocular functions in matched samples of anxiety-disordered and control subjects. In this study, the optokinetically-determined fixation, sequential scanning, and perceptual span capacities obtained by means of a newly revised blurring-speed method were significantly lower or impaired in 70 anxiety-disordered patients vs 70 controls. Such data supported further the hypothesis that there may be cerebellar-vestibular predispositions to anxiety disorders and the optokinetically-based tracking method may prove useful in separating a diverse array of CV-determined or related anxiety symptoms from those of other origins. However, independent validation as well as additional studies of anxiety disorders using larger samples vs random or "normal" controls are required before conclusions are justified.     

Factors related to the comorbidity between oppositional defiant disorder and anxiety disorders in preschool children

Background and objectives: The aim of the study is to identify factors related to comorbid oppositional defiant disorder (ODD) and anxiety disorders (ADs). Design: A sample of 622 children was assessed longitudinally at 3 and 5 years of age. Methods: At baseline, there were 310 boys (49.8%), most participants were of Caucasian–white ethnicity (89.1%) and attended to public school (64.0%), and families’ socioeconomic status was 64.3% medium-high, 14.1% medium and 20.5% medium-low. Children diagnosed with ODD and/or AD were selected: n?=?103 at 3 years of age (44 ODD, 42 AD and 17 ODD?+?AD) and n?=?106 at 5 years of age (31 ODD, 60 AD and 15 ODD?+?AD). Results: High levels of the child’s negative affectivity and the mother’s aggressive behavior (versus AD), and high scores in the father’s psychopathology measurements (versus ODD) were related to the presence of comorbid ODD?+?AD at 3 years of age. High scores in approach-positive anticipation, fears (only in boys, in girls the reverse effect occurred) compared to ODD and AD independently and aggressive behavior (versus AD), and low scores for smiling and laughter (versus ODD only and AD only) were predictive of comorbidity at the 5 years of age. Conclusions: Temperament traits may be a common factor in explaining longitudinal ODD?+?AD comorbidity.     

A study of impaired judgment of eye-gaze direction and related face-processing deficits in autism spectrum disorders

The purpose of this study was to examine whether individuals with autism spectrum disorders (ASD) use the same cognitive strategies as typically developing individuals when processing eye-gaze direction. Subjects viewed pictures of whole faces, the eye region alone, and pairs of arrows presented for 40, 70, or 100 ms, and responded according to the direction the eyes were looking or the arrows pointing (left, right, or straight ahead). Experiment 1 demonstrated that typically developing adults (n = 41) were more accurate and showed shorter reaction times when judging direction of averted eye gaze in the context of the whole face than when only the eyes were visible (eye-region-alone condition). Furthermore, in the eye-region-alone condition participants were more accurate and faster at judging direct eye gaze than averted eye gaze. The same task was used in experiment 2 to compare the performance of a group of individuals with ASD (n = 24) with that of a group of IQ-matched typically developing individuals (n = 26). The performance of the control participants was identical to that observed in experiment 1. Individuals with ASD were able to judge eye-gaze direction accurately at short exposure duration; however, they failed to show the typical advantage for judging averted gaze in whole faces and the increased sensitivity to direct gaze in the eye-region-alone condition. The findings are discussed in terms of impairments to discrete gaze-processing and face-processing mechanisms, and the connectivity between these mechanisms.     

The Yale Children''s Inventory—A screening tool for attention deficits and related disorders. Normative data for boys

Norwegian populated-based normative data on the Yale Children's Inventory (YCI) were provided for boys. All parents of boys aged 8 through 11 years in the county of Hammerfest received the YCI, and 77% responded. Mean scores on the YCI scales attention, activity, tractability, and fine motor were significantly lower in the Norwegian sample compared to the US normative data. Factor analysis of the 40 scale items yielded factors that corresponded generally well to the YCI scale constructs derived from US samples. We conclude that the scale constructs of the YCI seem to be transferable across these two cultures, but that restandardization is warranted. Further research is needed to establish cut-off values for clinical screening purposes. The Yale Children's Inventory has the potential to become a valuable screening tool for behavioral problems at school-age.     

The utility of screen for child anxiety related emotional disorders (scared) as a tool for identifying children at high risk for prevalent anxiety disorders

Abstract

The current study examined the utility of the Screen for Child Anxiety Related Emotional Disorders (SCARED) as a screening tool for the identification of children at high risk for prevalent childhood anxiety disorders. The child version of the Structured Clinical Interview for DSM (KSCID) was used as the diagnostic standard. It was investigated whether SCARED scores are indicative for the presence of generalized anxiety disorder, separation anxiety disorder, and social phobia. Five-hundred-and-thirty-seven children aged 7–14 years completed the SCARED. From this sample, 82 children were selected on the basis of their SCARED scores. A subgroup of these children scored relatively high on the generalized anxiety disorder, separation anxiety disorder, and/or social phobia scale(s) of the SCARED. A comparison group of children scored relatively low on these SCARED scales. Both groups of children then received the semi-structured interview to assess to what extent they fulfilled the DSM-IV criteria for the relevant anxiety disorders. Results provided some support for the predictive validity of the SCARED generalized anxiety disorder and separation anxiety disorder subscales. The implications of these findings for the detection of anxiety disorders in normal children are briefly discussed.     

Response inhibition in children with DSM-IV subtypes of AD/HD and related disruptive disorders: the role of reward.

The current study had four aims: (a) to replicate previous findings of slow response inhibition in Attention Deficit/Hyperactivity Disorder (AD/HD), (b) to explore whether poor response inhibition in children with AD/HD is a core problem or rather a result of an underlying problem related to reward, (c) to investigate the specificity of poor response inhibition and the role of reward in relation to AD/HD, and (d) to study whether findings would be different for three subtypes of AD/HD. In order to address these issues, a stop paradigm was administered under a reward condition and under a nonreward condition to an AD/HD group (n=24), an Oppositional Defiant Disorder (ODD)/Conduct Disorder (CD) group (n=21), a comorbid AD/HD+ODD/CD group (n=27), and a normal control (NC) group (n=41). Firstly, contrary to prediction, none of the Disruptive Behavior Disorder (DBD) groups differed from the NC group with respect to the speed of the inhibition process. Secondly, it was shown that children with AD/HD and children with comorbid AD/HD+ODD/CD, but not children with ODD/CD alone, slowed down more dramatically in the reward condition than normal controls. This finding was interpreted as a strategy to increase the chance of being rewarded in children with AD/HD and children with comorbid AD/HD+ODD/CD, but not in children with pure ODD/CD. Finally, analysis of AD/HD subtypes did not change the main findings of this study.