Mortality in French people with polyhandicap/profound intellectual and multiple disabilities.

BACKGROUND: In recent decades, progress has been made in the care of people with polyhandicap/profound intellectual and multiple disabilities (PIMD) through a better understanding of the pathophysiology and the development of new care management and rehabilitation strategies adapted to these extreme pathologies. Although there is a lack of knowledge about the health status and care management of the oldest people, a better understanding of the natural course of life of people with polyhandicap/PIMD would consequently allow the optimisation of preventive and curative care management strategies. Few robust data on mortality and life expectancy have been documented for this population in France. Our aims are to estimate the median survival time and assess the factors associated with mortality in people with polyhandicap/PIMD receiving care in France. METHODS: This study included people with polyhandicap/PIMD, followed by the French national cohort 'Eval-PLH' since 2015. These individuals were included in specialised rehabilitation centres and residential institutions. The people included in the first wave of the cohort (2015-2016) were eligible for the present study. Vital status on 1 January 2022 (censoring date) was collected in two ways: (1) spontaneous reporting by the participating centre to the coordinating team and (2) systematic checking on the French national death platform. According to the vital status, survival was calculated in years from the date of birth to the date of death or from the date of birth to the censoring date. The factors associated with mortality were evaluated using the Cox proportional regression hazards model. RESULTS: Data from 780 individuals aged between 3 and 67 years were analysed. At the censoring date, 176 (22.6%) had died, and the mean survival was 52.8 years (95% confidence interval: 51.1-54.5). Mortality was significantly associated with a progressive aetiology, recurrent pulmonary infections, drug-resistant epilepsy and a higher number of medical devices. CONCLUSIONS: This study shows for the first time the survival and impact of factors associated with mortality in people with polyhandicap/PIMD in France.

Development and initial validation of the polyhandicap severity scale.

OBJECTIVES: Providing a new tool, based on the point of view of experts in polyhandicap, which assesses the global severity of the health status of polyhandicapped persons is necessary. We present herein the initial validation of the polyhandicap severity scale (PSS). METHODS: The initial development of the tool was undertaken in two steps: item selection and validation process. The final set included 10 items related to abilities and 17 items related to comorbidities and impairments. The patient selection criteria were as follows: age>3 years, age at onset of cerebral lesion under 3 years old, with a combination of motor deficiency and profound intellectual impairment, associated with restricted mobility and everyday life dependence. External validity, reproducibility (20 patients), responsiveness (38 patients), and acceptability were explored. RESULTS: During the 18-month study period, a total of 875 patients were included. Two scores were calculated: an abilities score and a comorbidities/impairments score (higher score, higher severity). The 2 scores were higher for: older patients, patients with a progressive etiology, patients with more devices and more medications, patients with higher dependency and lower mobility. Indicators of reproducibility and responsiveness were satisfactory. The mean time duration of fulfilling was 22minutes (standard deviation 5). CONCLUSIONS: Quantifying the health severity of polyhandicapped persons is necessary for both healthcare workers and health decision makers. The polyhandicap severity scale provides the first reliable and valid measure of the health severity status for children and adults.

Salt tolerance of Beta macrocarpa is associated with efficient osmotic adjustment and increased apoplastic water content.

The chenopod Beta macrocarpa Guss (wild Swiss chard) is known for its salt tolerance, but the mechanisms involved are still debated. In order to elucidate the processes involved, we grew wild Swiss chard exposed to three salinity levels (0, 100 and 200 mm NaCl) for 45 days, and determined several physiological parameters at the end of this time. All plants survived despite reductions in growth, photosynthesis and stomatal conductance in plants exposed to salinity (100 and 200 mm NaCl). As expected, the negative effects of salinity were more pronounced at 200 mm than at 100 mm NaCl: (i) leaf apoplastic water content was maintained or increased despite a significant reduction in leaf water potential, revealing the halophytic character of B. macrocarpa; (ii) osmotic adjustment occurred, which presumably enhanced the driving force for water extraction from soil, and avoided toxic build up of Na(+) and Cl(-) in the mesophyll apoplast of leaves. Osmotic adjustment mainly occurred through accumulation of inorganic ions and to a lesser extent soluble sugars; proline was not implicated in osmotic adjustment. Overall, two important mechanisms of salt tolerance in B. macrocarpa were identified: osmotic and apoplastic water adjustment.

[Epilepsy progress in the dawn of the 21st century]. / L'epilepsie a l'aube du 21eme siècle.

AIM: Review of development on recent acquisitions in Epileptology. METHODS: A research in the medical literature on PubMed and an exhaustive review of the Published summaries and reports of Epileptology International Meetings, in the last five years. We included cohort studies, reviews and randomized double blinded therapeutic trials publications related to the pathogenic, diagnostic and therapeutic advances. Case reports and pure research studies have been excluded. RESULTS: Defective genes and/or physiopathological mechanisms were identified for some clinical forms of epilepsy. New antiepileptics drugs (AED) with a more accurate pathogenic target were, therefore, developed. Modern technologies of cerebral explorations and in particular the MRI (magnetic resonance imagery) and the PET (Positron Emission Tomography) constitute a progress not only for research but also for the diagnostic and therapeutic evaluation in particular for patients candidates to surgery. Five new drugs are on sale, several others are being studied. Diets would be alternatives to the AED in case of intractable epilepsy. Surgery indication is currently extended to the elderly. The global care of epileptic patients implies also the fight against exclusion. CONCLUSION: The formidable development in epilepsy knowledge in fundamental and in clinical research, is not an exclusive matter of researchers and specialists in this discipline but also with the generalists, the pediatric and all the professionals of health implicated on epileptic's care; thus allowing an improvement of the prognosis and the quality of life of these patients.

[Familial multiple sclerosis: study of 2 cases of a northern African family]. / Sclérose en plaques familiale: étude de deux cas d'une famille nord-africaine.

We report two cases of multiple sclerosis (MS) beginning in a mother and her daughter at 40 years of age. The diagnosis of MS was certain for both patients (Poser et al., 1983). Clinical features, evolution and response to treatment are comparable in both cases. Cerebral and medullar cord MRI and/or CT Scan showed characteristic lesions of demyelination on periventricular white matter, corpus callosum and brainstem.

Growth and biochemical changes in mammary glands of ewes from 1 to 18 months of age.

Growth and development of the mammary gland of ewe lambs was characterized by changes of various biochemical constituents. Mammary glands were removed from 49 ewe lambs ranging between 1 and 18 mo of age, weighed before and after trimming, and samples of the trimmed glands were fat extracted and dried. The dried fat-free tissues were weighed, ground, and analyzed for nucleic acids, nitrogen, sodium, potassium, and chloride contents. Fresh samples were used for glycogen determination. Trimmed weight of mammary glands increased rapidly after the 9th mo of age. The pattern was similar for nucleic acid content of mammary glands. Dry matter and fat contents of mammary glands increased up to the 9th mo, fat decreased thereafter, and dry matter remained constant. Protein content was low up to the 9th mo and increased thereafter. Sodium and glycogen content in mammary glands increased, but potassium decreased linearly from 1 to 18 mo of age. Allometric growth of mammary tissue started at about 3 mo of age, before onset of puberty at 8 mo of age, but intense mammary metabolic activity started only after the ewe attained puberty.