Baylor Scott and White Research Institute

Raymond, G. V. and R. Schiffmann (2018). “Cerebrotendinous xanthomatosis: The rare “treatable” disease you never consider.” Neurology Dec 7. [Epub ahead of print].

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Cerebrotendinous xanthomatosis (CTX; Online Mendelian Inheritance in Man No. 213700) is an autosomal recessive disorder due to pathogenic variant in the CYP27A1 gene resulting in a defect in the mitochondrial enzyme sterol 27-hydroxylase. The enzyme catalyzes multiple hydroxylation reactions involved in cholesterol metabolism and bile acid synthesis. When affected, it results in decreased synthesis of bile acids, with the resultant production of cholestanol and cholesterol affecting all tissues.

Minns, S., A. Levihn-Coon, E. Carl, J. A. J. Smits, W. Miller, D. Howard, S. Papini, S. Quiroz, E. Lee-Furman, M. Telch, P. Carlbring, D. Xanthopoulos and M. B. Powers (2018). “Immersive 3D exposure-based treatment for spider fear: A randomized controlled trial.” J Anxiety Disord Dec 20. [Epub ahead of print].

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Stereoscopic 3D gives the viewer the same shape, size, perspective and depth they would experience viewing the real world and could mimic the perceptual threat cues present in real life. This is the first study to investigate whether an immersive stereoscopic 3D video exposure-based treatment would be effective in reducing fear of spiders. Participants with a fear of spiders (N = 77) watched two psychoeducational videos with facts about spiders and phobias. They were then randomized to a treatment condition that watched a single session of a stereoscopic 3D immersive video exposure-based treatment (six 5-minute exposures) delivered through a virtual reality headset or a psychoeducation only control condition that watched a 30-minute neutral video (2D documentary) presented on a computer monitor. Assessments of spider fear (Fear of Spiders Questionnaire [FSQ], Behavioral Approach Task [BAT], & subjective ratings of fear) were completed pre- and post-treatment. Consistent with prediction, the stereoscopic 3D video condition outperformed the control condition in reducing fear of spiders showing a large between-group change effect size on the FSQ (Cohen’s d = 0.85) and a medium between-group effect size on the BAT (Cohen’s d = 0.47). This provides initial support for stereoscopic 3D video in treating phobias.

Asmundson, G. J. G., A. S. Thorisdottir, J. W. Roden-Foreman, S. O. Baird, S. M. Witcraft, A. T. Stein, J. A. J. Smits and M. B. Powers (2019). “A meta-analytic review of cognitive processing therapy for adults with posttraumatic stress disorder.” Cogn Behav Ther 48(1): 1-14.

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Numerous studies have demonstrated the efficacy of cognitive processing therapy (CPT) for treating posttraumatic stress disorder (PTSD). Two prior meta-analyses of studies are available but used approaches that limit conclusions that can be drawn regarding the impact of CPT on PTSD outcomes. The current meta-analysis reviewed outcomes of trials that tested the efficacy of CPT for PTSD in adults and evaluated potential moderators of outcomes. All published trials comparing CPT against an inactive control condition (i.e. psychological placebo or wait-list) or other active treatment for PTSD in adults were included, resulting in 11 studies with a total of 1130 participants. CPT outperformed inactive control conditions on PTSD outcome measures at posttreatment (mean Hedges’ g = 1.24) and follow-up (mean Hedges’ g = 0.90). The average CPT-treated participant fared better than 89% of those in inactive control conditions at posttreatment and 82% at follow-up. Results also showed that CPT outperformed inactive control conditions on non-PTSD outcome measures at posttreatment and follow-up and that CPT outperformed other active treatments at posttreatment but not at follow-up. Effect sizes of CPT on PTSD symptoms were not significantly moderated by participant age, number of treatment sessions, total sample size, length of follow-up, or group versus individual treatment; but, older studies had larger effect sizes and percent female sex moderated the effect of CPT on non-PTSD outcomes. These meta-analytic findings indicate that CPT is an effective PTSD treatment with lasting benefits across a range of outcomes.

Ko, J. M., K. S. White, A. H. Kovacs, K. M. Tecson, S. Apers, K. Luyckx, C. Thomet, W. Budts, J. Enomoto, M. A. Sluman, J. K. Wang, J. L. Jackson, P. Khairy, S. C. Cook, R. Subramanyan, L. Alday, K. Eriksen, M. Dellborg, M. Berghammer, B. Johansson, A. S. Mackie, S. Menahem, M. Caruana, G. Veldtman, A. Soufi, S. M. Fernandes, E. Callus, S. Kutty, A. Gandhi, P. Moons and A. M. Cedars (2018). “Physical Activity-Related Drivers of Perceived Health Status in Adults With Congenital Heart Disease.” Am J Cardiol 122(8): 1437-1442.

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Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients.

Arora, K., J. M. Sequeira, J. M. Alarcon, B. Wasek, E. Arning, T. Bottiglieri and E. V. Quadros (2018). “Neuropathology of vitamin B12 deficiency in the Cd320(-/-) mouse.” FASEB J Oct 10: [Epub ahead of print].

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In humans, vitamin B12 deficiency causes peripheral and CNS manifestations. Loss of myelin in the peripheral nerves and the spinal cord (SC) contributes to peripheral neuropathy and motor deficits. The metabolic basis for the demyelination and brain disorder is unknown. The transcobalamin receptor-knockout mouse ( Cd320(-/-)) develops cobalamin (Cbl) deficiency in the nervous system, with mild anemia. A decreased S-adenosylmethionine: S-adenosylhomocysteine ratio and increased methionine were seen in the brain with no significant changes in neurotransmitter metabolites. The structural pathology in the SC presented as loss of myelin in the axonal tracts with inflammation. The sciatic nerve (SN) showed increased nonuniform, internodal segments suggesting demyelination, and remyelination in progress. Consistent with these changes, the Cd320(-/-) mouse showed an increased latency to thermal nociception. Further, lower amplitude of compound action potential in the SN suggested that the functional capacity of the heavily myelinated axons were preferentially compromised, leading to loss of peripheral sensation. Although the metabolic basis for the demyelination and the structural and functional alterations of the nervous system in Cbl deficiency remain unresolved, the Cd320(-/-) mouse provides a unique model to investigate the pathologic consequences of vitamin B12 deficiency. -Arora, K., Sequeira, J. M., Alarcon, J. M., Wasek, B., Arning, E., Bottiglieri, T., Quadros, E. V. Neuropathology of vitamin B12 deficiency in the Cd320(-/-) mouse.