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Congenital Central Hypoventilation Syndrome (CCHS) is a rare neurocristopathy characterized by severe hypoventilation and autonomic dysregulation, with typical presentation in the neonatal period, and deficient cognitive skills in school-aged patients. We hypothesized that younger (preschool) children with CCHS would also show neurocognitive delay and that CCHS-related physiologic factors would impact neurocognitive test results.We studied developmental (Bayley) test results collected during routine clinical care in 31 children (mean age 25.0 ± 8.5 months; range, 6-40 months) with PHOX2B mutation-confirmed CCHS by comparing them with the normative reference mean from the Bayley standardization sample; we also examined associations between Bayley scores and CCHS disease-related factors.Preschool patients with CCHS fell significantly below the normative mean of 100 on Bayley indices of mental (mean, 83.35 ± 24.75) and motor (mean, 73.33 ± 20.48) development (P.001 for both). Significantly lower Bayley mental and motor scores were associated with severe breath-holding spells, prolonged sinus pauses, and need for 24 h/d artificial ventilation. Lower Bayley motor scores were also associated with seizures. Bayley scores differed among children with the three most common polyalanine repeat expansion mutation genotypes (mental, P = .001; motor, P = .006), being essentially normal in children with the 20/25 genotype but significantly lower in the other genotype groups (P.05).These results confirm neurodevelopmental impairment of CCHS preschoolers, with severity related to physiologic compromise and PHOX2B genotype. These findings suggest that adverse effects begin early in the disease process, supporting the need for neurodevelopmental monitoring and intervention from early infancy.

International audience; We report on a new method which allows extracting thermoelastic images by full-field method using in-plane measurement. The aim of this work is to show that it is possible to extract the in-plane thermoelastic field. Recently, a vibrometric measurement method has been developed at FEMTO-ST Institute for measuring in-plane displacement with nanometer and sub nanometer resolutions. The use of this method with thermography imaging allowed us to extract thermal expansion of micro samples heated by photothermal or thermoelectrical excitation. In this paper, we present an innovating technique to image thermoelastic field of these investigated samples. This technique is based on a CCD (charge-coupled device) camera which has been optimized for the previously presented applications.

International audience; We report on the dynamic and static thermal characterization of microsystems using a visible and near-infrared (NIR) thermography system based on a low-cost standard CCD sensor. The interest of this method is that it is possible to obtain a true spatial resolution better than 500 nm, which is necessary in high spatial resolution applications (microsystem applications). Another interesting point of this optical method is that the temperature error versus the emissivity error is always very low (compared to infrared thermography). We show, in this study, that this behavior originates in the high sensitivity of Planck's law in this wavelength range (compared to infrared range). Thus, we demonstrate the principal advantages of this method for micromachined heater application. Thermal measurements (in dynamic and static modes) were performed on micro-heaters commonly used in microsystems, platinum- and silicon-based micromachined heaters. The results show the capability of the method in terms of the thermal resolution and spatial resolution as well as the capacity to quickly obtain static and dynamic thermal images of the studied sample.