Neuropsychological Functioning in Children with Medium Chain Acyl Coenzyme A Dehydrogenase Deficiency (MCADD): The Impact of Early Diagnosis and Screening on Outcome

Children with medium chain acyl coenzyme A dehydrogenase deficiency (MCADD) have been reported to be at high risk for neurocognitive deficits. However this has not been systematically studied and little is known about the exact nature of neuropsychological sequelae or of the impact of early diagnosis and screening on outcome. We examined cognitive and adaptive outcome in children with MCADD (N?=?38, age range: 2 years, 2 months – 10 years, 3 months) diagnosed either through a newborn screening program (tandem mass spectrometry/MSMS) or upon clinical presentation. There was no evidence of overall intellectual impairment in either groups but there was some suggestion of poorer verbal and specific executive functioning (i.e., planning) abilities in the unscreened cohorts. Adaptive functioning was relatively intact with the exception of reduced Daily Living Skills in both our screened and unscreened groups. Early diagnosis and greater number of hospitalizations were related to higher verbal, communication, and socialization skills. Overall, our results highlight the importance of early diagnosis and management for children with MCADD.     

Whiteheadian Societies and Peirce's Law of Mind: Actuality and Potentiality in the Cosmic Process

Abstract

A more suitable philosophical position to counter the claims of scientific materialism re the nature of physical reality can possibly be found in combining the metaphysics of A.N. Whitehead and C.S. Peirce. Peirce postulated a habit-forming tendency or inbuilt potentiality of the cosmic process as a whole to move over time toward ever greater organization and more complex structure. But he is vague on how “the law of the mind” applies to nature below the level of human self-awareness. Whitehead's notion of actual entities (momentary self-constituting subjects of experience) as the “final real things of which the world is made up” logically allows for some primitive form of subjectivity with the requisite potentiality for habit-formation at all levels of existence and activity within nature. In addition, Whitehead's notion of structured societies made up of subsocieties with a “regnant” subsociety seems philosophically to justify the new emphasis on bottom-up rather than top-down causation in the emergence of more complex levels of existence and activity within the life-sciences.     

On the a priori and a posteriori assessment of probabilities

We argue that in spite of their apparent dissimilarity, the methodologies employed in the a priori and a posteriori assessment of probabilities can both be justified by appeal to a single principle of inductive reasoning, viz., the principle of symmetry. The difference between these two methodologies consists in the way in which information about the single-trial probabilities in a repeatable chance process is extracted from the constraints imposed by this principle. In the case of a posteriori reasoning, these constraints inform the analysis by fixing an a posteriori determinant of the probabilities, whereas, in the case of a priori reasoning, they imply certain claims which then serve as the basis for subsequent probabilistic deductions. In a given context of inquiry, the particular form which a priori or a posteriori reason may take depends, in large part, on the strength of the underlying symmetry assumed: the stronger the symmetry, the more information can be acquired a priori and the less information about the long-run behavior of the process is needed for an a posteriori assessment of the probabilities. In the context of this framework, frequency-based reasoning emerges as a limiting case of a posteriori reasoning, and reasoning about simple games of chance, as a limiting case of a priori reasoning. Between these two extremes, both a priori and a posteriori reasoning can take a variety of intermediate forms.     

Fear response to dissociation challenge

Abstract

Participants (N = 101) scoring high or low on a new scale for assessing fear of dissociative sensations (Dissociation Sensitivity Index) underwent an audio/visual sensory challenge using a device called the D.A.V.I.D. Participants' report of subjective fear and level of dissociation were measured before and after completing the audio/visual sensory challenge. Consistent with prediction, participants scoring high on the DSI responded to the challenge with significantly greater increases in subjective fear and dissociative symptoms relative to those scoring low on the DSI. Contrary to prediction, the DSI performed poorer than the Anxiety Sensitivity Index in predicting participants' response to the audio/visual sensory challenge. Theoretical and clinical implications of the findings are discussed.     

Arguing at Cross-Purposes: Discharging the Dialectical Obligations of the Coalescent Model of Argumentation

The paper addresses the manner in which the theory of Coalescent Argumentation [CA] has been received by the Argumentation Theory community. I begin (section 2) by providing a theoretical overview of the Coalescent model of argumentation as developed by Michael A. Gilbert (1997). I next engage the several objections that have been raised against CA (section 3). I contend that objectors to the Coalescent model are not properly sensitive to the theoretical consequences of the genuinely situated nature of argument. I conclude (section 4) by suggesting that the resolution to the dispute between Gilbert and his objectors hinges on the outcome of several foundational theoretical questions identified over the course of the paper.     

Empirical derivation of SVIB-Holland scales and conversion tables

Significant relationships between the Holland Vocational Preference Inventory (VPI) and the Strong Vocational Interest Blank (SVIB) were again empirically demonstrated in this study, and conversion equations were developed to use standard scores of SVIB scales, rather than items, to produce estimates of VPI scores. Cross validation on a limited population yielded supportive evidence that these conversion equations can provide meaningful VPI estimates from SVIB scale scores. The need for validation across a variety of populations was underscored in order to assess further generalizability of these findings.     

Dry-bed training: rapid elimination of childhood enuresis

Enuresis has been treated with moderate effectiveness by the urine-alarm method which requires many weeks of training. The present procedure used a urine-alarm apparatus but added such features as training in inhibiting urination, positive reinforcement for correct urinations, training in rapid awakening, increased fluid intake, increased social motivation to be nonenuretic, self-correction of accidents, and practice in toileting. After one all-night training session, the 24 enuretic children averaged only two bedwettings before achieving fourteen consecutive dry nights and had no major relapses. Little or no reduction in bedwetting occurred within the first two weeks for matched-control enuretics who were given the standard urine-alarm training. The results of a control-procedure showed that the new procedure did not involve Pavlovian conditioning. The new method appears to be a more rapid, effective and different type of treatment for enuresis.     

The restoration of extinguished avoidance responses

Following extensive signaled shuttlebox avoidance training, Fischer 344 rats were given extinction trials until they reliably stopped responding and, thereafter, they received occasional classical CS-UCS pairings during the course of restoration test trials in which shock was absent. Experiment I demonstrated that the classical contingency was both necessary and sufficient for the complete recovery of such behavior. Experiment II revealed that the degree of recovery was not differentially affected by the frequency of such CS-UCS pairings. However, in Experiment III recovery in the presence of this contingency was shown to depend on the number of previous extinction trials, although even after a large number of such trials, a considerable degree of recovery occurred. These findings demonstrated the central role of the classical CS-UCS contingency in restoring a previously extinguished avoidance response.     

Alcoholic drinking: suppression by a brief time-out procedure

Volunteer chronic alcoholics were given daily access to substantial quantities of alcohol within a residential research setting. Drinking was suppressed to an average of approximately one-half of Baseline levels when 10 or 15 min of physical and social isolation was required as an immediate consequence to receiving each one-ounce drink of 95-proof ethanol. This time-out procedure suppressed the drinking of nine out of ten subjects. Drinking returned to high levels when brief contingent time-out was discontinued. The relevance of such reversible controlling relationships to the treatment of alcoholism is discussed.