Psychometric properties of QI-Disability in CDKL5 Deficiency Disorder: Establishing readiness for clinical trials.

CDKL5 Deficiency Disorder (CDD) is a rare genetic disorder with symptoms of epilepsy, developmental impairments, and other comorbidities. Currently, there are no outcome measures for CDD with comprehensive evidence of validation. This study aimed to evaluate the psychometric properties of the Quality of Life Inventory-Disability (QI-Disability) in CDD. Quality of Life Inventory-Disability was administered to 152 parent caregivers registered with the International CDKL5 Disorder Database (ICDD). Confirmatory factor analysis was conducted and the goodness of fit of the factor structure was assessed. Fixed-effects linear regression models examined the responsiveness of QI-Disability to reported changes in child health. A subset of parent caregivers (n = 56) completed QI-Disability, as well as additional health-related questions, on two occasions separated by four weeks to evaluate test-retest reliability. Test-retest reliability was assessed using intra-class correlations (ICCs) calculated from QI-Disability scores. Based upon adjustments for changes in child health, ICCs were recalculated to estimate responsiveness to change. Confirmatory factor analysis, internal consistency, and divergent validity were mostly satisfactory, except divergent validity was not satisfactory for the Social Interactions and Independence domains. The Physical Health, Social Interactions, Leisure, and Total scores responded to changes in the child's Physical health, and the Negative Emotions and Leisure domains responded to changes in the child's behavior. Unadjusted and adjusted ICC values were above 0.8 for the Positive Emotions, Negative Emotions, Social Interactions, Leisure, Independence domains and Total score, and above 0.6 for the Physical Health domain. Findings suggest that QI-Disability is suitable to assess the quality of life of children and adults with CDD and could be of value for upcoming clinical trials.

Physical and perceptual cooling: Improving cognitive function, mood disturbance and time to fatigue in the heat.

This study investigated the effects of menthol swilling and crushed ice ingestion on cognitive function, total mood disturbance (TMD), and time to fatigue (TTF). Twelve male long-distance runners completed three counterbalanced running trials (3 × 30 minutes at 65% VO2peak and a TTF run at 100% VO2peak ) in hot, humid conditions (35.3 ± 0.3°C, 59.2 ± 2.5% relative humidity). Trials consisted of precooling with crushed ice ingestion and mid-cooling by menthol swilling (MIX), precooling with water ingestion and mid-cooling by menthol swilling (MENTH), and control (CON). Swilling with either 25 mL of menthol solution or placebo occurred upon entry to the heat, at 15-minute intervals during the run and prior to the TTF run. Core temperature, forehead skin temperature, tympanic temperature, perceived thermal sensation, and TMD were significantly lower with MIX compared with MENTH and CON (P < .05). Thirst was satiated in MIX compared with CON; however, MENTH did not have a significant effect. After 90 minutes of running and post-TTF run, fewer errors occurred in the executive control task (P < .05), as well as decision-making and working memory (P > .05; d = 0.5-0.79) between MIX and CON; however, MENTH had no effect compared with CON. The TTF run was significantly longer with MENTH (34.38%; P = .02) and MIX (39.06%; P = .001) compared with CON, with no difference between MENTH and MIX (P = .618). The physical reduction in core and internal head temperature seen with crushed ice ingestion may lead to improvements in cognitive function; however, both MENTH and MIX were sufficient for improving exercise performance.

Enhanced Decision Making and Working Memory During Exercise in the Heat With Crushed Ice Ingestion.

PURPOSE: To examine the effects of precooling via crushed ice ingestion on cognitive function during exercise in the heat. METHODS: Eleven active men ingested either 7 g·kg-1 of crushed ice (ICE) or thermoneutral water (CON) 30 minutes before running 90 minutes on a treadmill at a velocity equivalent to 65% VO2peak in hot and humid conditions (35.0°C [0.5°C], 53.1% [3.9%] relative humidity). Participants completed 3 cognitive tasks to investigate decision making (8-choice reaction time [CRT]), working memory (serial seven [S7]), and executive control (color multisource interference task [cMSIT]) on arrival, after precooling, and after running. RESULTS: Precooling significantly decreased preexercise core (Tcore) and forehead skin temperature in ICE compared with CON, respectively (Tcore 0.8°C [0.4°C], -0.2°C [0.1°C]; Thead -0.5°C [0.4°C], 0.2°C [0.8°C]; P ≤ .05). Postrun, ICE significantly reduced errors compared with CON for CRT (P ≤ .05; d = 0.90; 90% confidence interval, 0.13-1.60) and S7 (P ≤ .05; d = 1.05; 90% confidence interval, 0.26-1.75). Thermal sensation was lower after precooling with ICE (P ≤ .05), but no significant differences were recorded between conditions for cMSIT errors, skin temperature, heart rate, or ratings of perceived exertion or perceived thirst (P > .05). CONCLUSIONS: Precooling via ICE maintained cognitive accuracy in decision making and working memory during exercise in the heat. Thus, ICE may have the potential to improve sporting performance by resisting deleterious effects of exercise in a hot and humid environment on cognitive function.

Internal precooling decreases forehead and core temperature but does not alter choice reaction time during steady state exercise in hot, humid conditions.

This study aimed to determine if precooling via crushed ice ingestion reduces forehead skin temperature (Thead) and core temperature (Tcore) during exercise in the heat and whether it has an effect on choice reaction time (CRT). Ten males commenced a 30 min precooling period, ingesting either 7 g kg-1 of crushed ice (ICE) or room temperature water (CON) prior to cycling 60 min at 55% V̇O2peak in hot, humid conditions (35.0 ± 0.3 °C, 50.2 ± 2.1% Relative Humidity). The CRT task was completed upon arrival and after the precooling period in the lab, then at 15 min intervals during exercise in the heat. Precooling reduced Thead and Tcore to a greater degree in ICE (Thead: -0.8 ± 0.31 °C; Tcore: -0.9 ± 0.3 °C) compared with CON (Thead: -0.2 ± 0.3 °C; Tcore: -0.2 ± 0.2 °C) (p ≤ 0.001). Choice reaction time performance improved throughout the cycle for both conditions (p ≤ 0.05). Ice ingestion lowered thermal sensation (p = 0.003) and skin temperature (d = 0.88; Tskin), while heart rate, ratings of perceived exertion and thirst were similar between conditions (p > 0.05). Precooling effectively reduced Thead and Tcore but did not provide additional improvement in CRT during moderate exercise in the heat. Further investigation is required to determine whether the lower central and peripheral temperature after ice ingestion is beneficial for tasks of greater cognitive effort.