Obstructive Sleep Apnea Syndrome in Prader-Willi Syndrome: An Unrecognized and Untreated Cause of Cognitive and Behavioral Deficits?

Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by a range of physical, psychological, and physiological abnormalities. It is also distinguished by the high prevalence of obstructive sleep apnea syndrome (OSAS), i.e., repetitive upper airway collapse during sleep resulting in hypoxia and sleep fragmentation. In non-PWS populations, OSAS is associated with a range of neurocognitive and psychosocial deficits. Importantly, these deficits are at least partly reversible following treatment. Given the findings in non-PWS populations, it is possible that OSAS may contribute to neurocognitive and psychosocial deficits in PWS. The present review examines this possibility. While acknowledging a primary contribution from the primary genetic abnormality to central neural dysfunction in PWS, we conclude that OSAS may be an important secondary contributing factor to reduced neurocognitive and psychosocial performance. Treatment of OSAS may have potential benefits in improving neurocognitive performance and behavior in PWS, but this awaits confirmatory investigation.     

The Question of Questions: What is a Gene? Comments on Rolston and Griffths & Stotz

If the question “What is a gene?” proves to be worth asking it must be able to elicit an answer which both recognizes and address the reasons why the concept of the gene ever seemed to be something worth getting excited about in the first place as well analyzing and evaluating the latest develops in the molecular biology of DNA. Each of the preceding papers fails to do one of these and sufferrs the consequences. Where Rolston responds to the apparent failure of molecular biology to make good on the desideratum of the classical gene by veering off into fanciful talk about “cybernetic genes,” Griffiths and Stotz lose themselves in the molecular fine print and forget to ask themselves why “genes” should be of any special interst anyway.     

Women, Evil, and Grey Zones

Gray zones, which develop wherever oppression is severe and lasting, are inhabited by victims of evil who become complicit in perpetrating on others the evils that threaten to engulf themselves. Women, who have inhabited many gray zones, present challenges for feminist theorists, who have long struggled with how resistance is possible under coercive institutions. Building on Primo Levi's reflections on the gray zone in Nazi death camps and ghettos, this essay argues that resistance is sometimes possible, although outsiders are rarely, if ever, in a position to judge when. It also raises questions about the adequacy of ordinary moral concepts to mark the distinctions that would be helpful for thinking about how to respond in a gray zone.     

Global dot integration in typically developing children and in Williams Syndrome

Williams Syndrome (WS) is a neurodevelopmental disorder that results in deficits in visuospatial perception and cognition. The dorsal stream vulnerability hypothesis in WS predicts that visual motion processes are more susceptible to damage than visual form processes. We asked WS participants and typically developing children to detect the global structure Glass patterns, under “static” and “dynamic” conditions in order to evaluate this hypothesis. Sequentially presented Glass patterns are coined as dynamic because they induce illusory motion, which is modeled after the interaction between orientation (form) and direction (motion) mechanisms. If the dorsal stream vulnerability holds in WS participants, then they should process real and illusory motion atypically. However, results are consistent with the idea that form and motion integration mechanisms are functionally delayed or attenuated in WS. Form coherence thresholds for both static and dynamic Glass patterns in WS were similar to those of 4–5 year old children, younger than what is predicted by mental age. Dynamic presentation of Glass patterns improved thresholds to the same degree as typical participants. Motion coherence thresholds in WS were similar to those of mental age matches. These data pose constraints on the dorsal vulnerability hypothesis, and refine our understanding of the relationship between form and motion processing in development.     

Symptoms of Meares-Irlen/Visual Stress Syndrome in subjects diagnosed with Chronic Fatigue Syndrome

Several diagnostic symptoms of the visual-processing deficit Meares-Irlen/Visual Stress Syndrome are remarkably similar to symptom manifestations reported by individuals with chronic fatigue syndrome (CFS). We surveyed the specific incidences of nine widely-recognised symptoms of visual stress (VS) in a group of subjects (n = 20) previously diagnosed with CFS. The presence of each symptom of VS in the CFS group was compared to its respective presence in both an age and sex matched healthy comparison group (n = 46), and an age and sex matched group comprised of individuals (n = 14) diagnosed with VS. Results showed the frequencies of all nine VS symptoms in the CFS-diagnosed group to be significantly higher (p = .032 – p < .0005) than in the comparison group, with only two symptoms being statistically less frequent in the CFS group than in the VS-diagnosed group. The average number of VS symptoms reported by the CFS group was also significantly higher than the comparison group, yet not significantly different from the VS group. Thus, the occurrence of VS symptoms in subjects diagnosed with CFS appears to be far greater than previously reported, which in turn may indicate the interplay of some yet to be identified underlying factor(s) common to both conditions.     

Adaptation of the Children's Friendship Training Program for Children with Fetal Alcohol Spectrum Disorders

ABSTRACT

Previous research attests to the marked impairments in social functioning exhibited by children with Fetal Alcohol Spectrum Disorders (FASD), suggesting that such children are in need of social skills intervention. Recently, an existing evidence-based manualized behavioral treatment for improving children's friendships was implemented and demonstrated to be effective with children aged 6–12, diagnosed with FASD. In the present report, we describe methods for adapting this behavioral intervention in line with the specific cognitive and behavioral deficits seen in children with FASD and other developmental disabilities to enhance treatment efficacy.     

Finger gnosia: a predictor of numerical abilities in children?

This paper aimed to test the specificity of predicting power of finger gnosia on later numerical abilities in school-age children and to contribute to the understanding of this effect. Forty-one children were tested in the beginning of Grade 1 on finger gnosia, left–right orientation (another sign of the Gerstmann “syndrome”), and global development. Fifteen months later, numerical and reading abilities were assessed. Analyses of the results indicated that, contrary to the general measures of cognitive development, performance in the finger gnosia test was a good predictor of numerical skills 1 year later but not of reading skills, which proves the specificity of that predictor. The same conclusion was also true for the left–right orientation. However, finger gnosia could equally predict performance in numerical tasks that do or do not rely heavily on finger representation or on magnitude representation. Results are discussed in terms of the localizationist and the functional hypotheses.     

A Nonverbal Learning Disability in a Case of Central Hypoventilation Syndrome without a PHOX2B Gene Mutation

This study examines the neuropsychological profile of a boy with congenital central hypoventilation syndrome (CCHS) without a paired-like homeobox gene (PHOX2B) mutation. CCHS is a rare disorder of autonomic nervous system development characterized by an impaired ventilatory response to hypercarbia and hypoxemia. Mild intellectual deficits are common but a specific cognitive profile is not established in CCHS. We describe a nonverbal learning disorder as a CCHS endophenotype and recommend that detailed neuropsychological testing be performed on all individuals with CCHS. Defining the psycho-educational needs in CCHS may avert compounding the emotional and medical stresses of this already debilitating disorder.     

‘If I knew where the enemy,or even Germany was,we could have fired in that direction’: Bion's experience of war

The role of a child psychotherapist's gender is not often considered in terms of treatment outcome. This paper discusses how aspects of being a male child psychotherapist influenced the successful treatment of a very young boy with Asperger's Syndrome. The paper highlights how the nature of his very early relationship with his mother and the absence of his father were the basis of his difficulties resulting in that diagnosis. It shows that his desire to seize the opportunity of working with a male enabled him to understand his feelings about his father, why it was so difficult to want to be with him and not his mother, and why he could not be without her.

Le rôle joué par le sexe du psychothérapeute d'enfants dans les effets du traitement est rarement pris en compte. Dans cet article, l'auteur montre qu'à un certain niveau le fait d'être un psychothérapeute homme eut une influence sur la réussite de la thérapie d'un très jeune garçon présentant un syndrome d'Asperger. Selon l'auteur, la nature de la relation précoce entre ce petit garçon et sa mère, ainsi que l'absence de son père, étaient à l'origine des difficultés qui conduisirent à ce diagnostic. Il montre que le désir de l'enfant de se saisir de l'occasion ainsi offerte de travailler avec un homme lui permit de comprendre trois éléments essentiels : ses sentiments à l'égard de son père, la raison pour laquelle il lui était tellement difficile de vouloir être avec lui plutôt que de rester avec sa mère, et ce qui faisait qu'il ne pouvait pas se passer de celle-ci.

Die Rolle des Geschlechts des Kindertherapeuten wird nicht oft bezüglich des Behandlungsergebnis in Betracht bezogen. Dieser Artikel diskutiert, wie Aspekte dessen, ein männlicher Kindertherapeut zu sein, die erfolgreiche Behandlung eines sehr kleinen Jungen mit Asperger Syndrom beeinflussten. Der Artikel unterstreicht, wie die Art seiner frühen Beziehung zu seiner Mutter und die Abwesenheit seines Vaters die Grundlage seiner Schwierigkeiten bildeten, die zu dieser Diagnose führten. Es zeigt, dass sein Wunsch, die Gelegenheit zu ergreifen, mit einem Mann zu arbeiten, ihm ermöglichte, seine Gefühle seinem Vater gegenüber zu verstehen, warum es so schwierig war, mit ihm und nicht seiner Mutter sein zu wollen und warum er nicht ohne sie sein konnnte.

Riassunto: Il ruolo che gioca il sesso dello psicoterapeuta infantile non è solitamente considerato in relazione al risultato del trattamento. In questo articolo si discute come il fatto di avere un terapeuta maschile abbia influenzato per certi aspetti il risultato positivo del trattamento di un bambino molto piccolo che soffriva della Sindrome di Asperger. L'articolo illustra come la natura della sua primissima relazione con la madre e l'assenza del padre furono alla base delle sue difficoltà che portarono alla diagnosi. L'articolo mostra come il desiderio del bambino di lavorare con un terapeuta maschile gli ha permesso di capire i suoi sentimenti verso il padre, perché era così difficile voler restare con lui e lo stesso non succedeva con sua madre, e perché non poteva restare senza di lei.