Search

Neurodegenerative diseases represent a group of disorders characterized by progressive degeneration of neurons in the central nervous system, leading to a range of cognitive, motor, and sensory impairments. In recent years, there has been growing interest in the association between neurodegenerative diseases and olfactory dysfunction (OD). Characterized by a decline in the ability to detect or identify odors, OD has been observed in various conditions, including Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), and Amyotrophic Lateral Sclerosis (ALS). This phenomenon often precedes the onset of other clinical symptoms, suggesting its potential utility as an early marker or prodromal symptom of neurodegenerative diseases. This review provides a vast literature overview on the current knowledge of OD in PD, AD, ALS, and HD in order to evaluate its potential as a biomarker, particularly in the early and prodromal stages of these diseases. We summarize the most common methods used to measure olfactory function and delve into neuropathological correlations and the alterations in neurotransmitter systems associated with OD in those neurodegenerative diseases, including differences in genetic variants if applicable, and cater to current pitfalls and shortcomings in the research.

ABSTRACT Introduction: Myotonic dystrophy type 1 (DM1) patients might represent a high‐risk population for severe COVID‐19 disease, as cardiopulmonary symptoms are part of the clinical spectrum of DM1. The COVID‐19 pandemic may have impacted DM1 patients. We aimed to determine the effect of the COVID‐19 pandemic on DM1 patients to guide management strategies in possible future pandemics. Methods: Data on the presence of a COVID‐19 infection were retrieved from 195 DM1 patients in the MYODRAFT database. Between August 12 and October 4, 2021, 82 patients and proxies filled out a questionnaire on COVID‐19 symptoms, well‐being, and organization of care. Data were compared to prepandemic data. Results: A total of 18 patients had COVID‐19 (13 confirmed, 5 probable infections). The prevalence of COVID‐19 in our cohort was 9.2%, which was lower than in the Dutch population (11.5%). Four patients (22.2%) were hospitalized due to a COVID‐19 infection, which was higher than in the Dutch population (3.6%). Two infected patients died. A high rate of canceled appointments was reported. Patients reported no change in physical functioning during the pandemic, whereas proxies reported a deterioration in mental and physical well‐being of patients. Conclusions: The prevalence of COVID‐19 infections was not higher in DM1 patients than in the general population, but DM1 patients are more susceptible to complicated disease when infected. Longitudinal data on patient‐reported physical functioning suggest that the COVID‐19 pandemic and the pandemic management strategies implemented did not influence the course of disease in DM1 patients, and similar strategies can be re‐used in comparable situations.

Le projet d'Assurance autonomie (AA) visait à introduire une assurance publique des soins de longue durée dans le système de santé et de services sociaux du Québec. Développé à partir d'une revue des assurances de ce type implantées ailleurs dans le monde, ce projet a fait l'objet de travaux intensifs pour l'opérationnaliser dans le contexte québécois. Le projet s'appuyait sur des acquis du système québécois : la présence de gestionnaires de cas chargés de l'évaluation et du suivi des personnes âgées dans le cadre de réseaux intégrés de services, un outil d'évaluation standardisé permettant la classification des individus en 14 profils associés à une intensité de services requis et un système d'information. Suite à l'évaluation du besoin de l'usager et à la détermination du profil, une allocation de soutien à l'autonomie peut donc être attribuée et servir à financer les services publics ou à acheter des services à des organismes privés et communautaires. Le financement de l'AA était assuré par les impôts via un programme budgétaire spécifique dédié. Tous les aspects légaux, administratifs, financiers et informatiques liés à ce projet ont été traités par les autorités ministérielles. Le livre blanc fut bien accueilli par les parties intéressées en commission parlementaire et le projet de loi créant l'AA a été déposé à l'Assemblée nationale en décembre 2013. Des élections précipitées ont toutefois provoqué la mort du projet.