[Rapidly progressive dementia: management by the internist]. / Démence rapidement progressive : prise en charge par l'interniste.

Rapidly progressive dementia is distinguished from other neurocognitive disorders by its rapid onset (less than two years from first symptoms to diagnosis). It comprises a wide range of etiologies, including Creutzfledt-Jakob disease and Alzheimer's disease. When faced with a recent and rapid onset of major cognitive impairment, it is essential to rule out delirium or any other cause that may lead to rapid cognitive decline (such as stroke, encephalitis, or epileptic seizure). The work-up is based on a detailed history (drugs, exposure, medical history), a thorough clinical examination, and a broad biological work-up including MRI, lumbar puncture and EEG. Management should rapidly involve a neurologist or a specialized center.

Les démences rapidement progressives se distinguent des autres troubles neurocognitifs par leur rapidité d'installation, soit moins de deux ans entre les premiers symptômes et le diagnostic. Elles comprennent des étiologies très diverses, comme les maladies de Creutzfeldt-Jakob et d'Alzheimer. Face à un trouble cognitif rapidement progressif et récent, il est essentiel d'exclure un état confusionnel ou une autre cause pouvant mener à un déclin cognitif rapide (par exemple, AVC, encéphalite ou crise d'épilepsie). Le bilan repose sur une anamnèse étendue (médicaments, exposition, antécédents), un examen clinique complet et un panel d'examens paracliniques pouvant notamment comprendre une IRM, une ponction lombaire et un EEG. La prise en charge doit impérativement intégrer un neurologue ou un centre spécialisé.

[Pharmacological trials evaluating treatment options for neuropsychiatric symptoms in post-COVID. A narrative review]. / Essais pharmacologiques sur les traitements des symptômes neuropsychiatriques du post-Covid. Revue narrative de la littérature.

Post-COVID prevalence's is estimated at 10 % in the general population. The neuropsychiatric symptoms, which are frequent (up to 30 %), can severely affect the quality of life of patients affected by this condition, notably by significantly reducing their working ability. To date, no pharmacologic treatment is available for post-COVID, apart from symptomatic treatments. A large number of pharmacological clinical trials for post-COVID are underway since 2021. A number of these trials targets neuropsychiatric symptoms, based on the various underlying pathophysiological hypotheses. The objective of this narrative review is to provide an overview of these ongoing trials targeting neuropsychiatric symptoms in post-COVID.

La prévalence du syndrome post-Covid est évaluée à 10 % dans la population générale. Les symptômes neuropsychiatriques, fréquents (jusqu'à 30 %), peuvent sévèrement affecter la qualité de vie des patients qui en sont atteints et, notamment, en réduisant significativement leur capacité de travail. À ce jour, il n'existe pas de traitement médicamenteux pour le syndrome post-Covid, en dehors des traitements symptomatiques. C'est pourquoi, un grand nombre d'essais thérapeutiques concernant le post-Covid sont en cours depuis 2021. Un certain nombre d'entre eux ciblent les symptômes neuropsychiatriques en se basant sur les diverses hypothèses physiopathologiques élaborées sur le post-Covid. L'objectif de cette revue narrative est d'établir un état des lieux des essais thérapeutiques en cours ciblant les symptômes neuropsychiatriques du post-Covid.

[Neurology: what's new in 2022]. / Neurologie : ce qui a changé en 2022.

The year 2022 was marked by the development of numerous new treatments for refractory myasthenia gravis. The link between epilepsy and cerebrovascular disorder was studied and lamotrigine discovered to be the optimal treatment choice for epilepsy secondary to stroke to prevent mortality on patient of 45 years and older. New randomized study finally demonstrated the utility of thrombectomy in selected patients with basilar artery occlusion. The causal relationship between Epstein-Barr infection and multiple sclerosis has been proved thanks to a large cohort study. A new possibility of subcutaneous continuous levodopa administration gave promising result. Finally, numerous studies confirmed the efficacy and excellent tolerability of anti-CGRP antibodies.

L'année 2022 a été marquée par l'arrivée de nombreux traitements pour la myasthénie réfractaire. Le lien entre l'épilepsie et le risque cérébro-vasculaire a été bien étudié, démontrant que la lamotrigine semble être le meilleur traitement pour prévenir la mortalité chez les patients de 45 ans et plus. De nouvelles études ont enfin pu établir l'utilité de la thrombectomie dans les occlusions basilaires. Le lien entre le virus d'Epstein-Barr et la sclérose en plaques a pu être prouvé à la suite d'une importante étude de cohorte. Une nouvelle technique d'administration sous-cutanée de la lévodopa semble prometteuse. Enfin, de nombreuses études confirment l'efficacité et l'excellente tolérance des anticorps anti-CGRP (Calcitonine Gene Related Protein).

Encephalopathies Associated With Severe COVID-19 Present Neurovascular Unit Alterations Without Evidence for Strong Neuroinflammation.

OBJECTIVE: Coronavirus disease (COVID-19) has been associated with a large variety of neurologic disorders. However, the mechanisms underlying these neurologic complications remain elusive. In this study, we aimed at determining whether neurologic symptoms were caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) direct infection or by either systemic or local proinflammatory mediators. METHODS: In this cross-sectional study, we checked for SARS-CoV-2 RNA by quantitative reverse transcription PCR, SARS-CoV-2-specific antibodies, and 49 cytokines/chemokines/growth factors (by Luminex) in the CSF +/- sera of a cohort of 22 COVID-19 patients with neurologic presentation and 55 neurologic control patients (inflammatory neurologic disorder [IND], noninflammatory neurologic disorder, and MS). RESULTS: We detected anti-SARS-CoV-2 immunoglobulin G in patients with severe COVID-19 with signs of intrathecal synthesis for some of them. Of the 4 categories of tested patients, the CSF of IND exhibited the highest level of cytokines, chemokines, and growth factors. By contrast, patients with COVID-19 did not present overall upregulation of inflammatory mediators in the CSF. However, patients with severe COVID-19 (intensive care unit patients) exhibited higher concentrations of CCL2, CXCL8, and vascular endothelium growth factor A (VEGF-A) in the CSF than patients with a milder form of COVID-19. In addition, we could show that intrathecal CXCL8 synthesis was linked to an elevated albumin ratio and correlated with the increase of peripheral inflammation (serum hepatocyte growth factor [HGF] and CXCL10). CONCLUSIONS: Our results do not indicate active replication of SARS-CoV-2 in the CSF or signs of massive inflammation in the CSF compartment but highlight a specific impairment of the neurovascular unit linked to intrathecal production of CXCL8.

[Recent Advances in Neurology]. / Neurologie.

Significant developments were published in 2020 in the field of blood biomarkers in Alzheimer's disease. Several studies helped to define more accurately the management of status epilepticus and of epilepsy in women of childbearing age. The new Swiss guidelines for the pre-hospital management of acute stroke were issued, as are new targets for stroke prevention. Numerous advances concerning the management of NMO-SD (NeuroMyelitis Optica Spectrum Disorder) were published. Different neurological presentations linked to the COVID-19 pandemic were described (central and peripheral). Several studies confirmed the effectiveness of new migraine treatments (including anti-CGRP). New pharmacological therapies are available for Parkinson's disease.

L'année 2020 a vu d'importantes avancées dans le domaine des biomarqueurs sanguins pour le diagnostic biologique de la maladie d'Alzheimer. Plusieurs études permettent de mieux définir la prise en charge de l'épilepsie chez la femme en âge de procréer et de l'état de mal épileptique. Les nouvelles recommandations suisses pour la prise en charge préhospitalière de l'AVC aigu sont en cours de publication, tout comme de nouvelles cibles pour leur prévention secondaire. De nombreuses avancées concernant la prise en charge des Neuromyelitis Optica Spectrum Disorder ont été publiées. Divers tableaux neurologiques (centraux et périphériques) liés à la pandémie de Covid-19 ont été décrits. Plusieurs études ont permis de confirmer l'efficacité des nouveaux traitements de la migraine (notamment les anti-Calcitonin Gene-Related Peptide). Enfin, de nouvelles thérapies pharmacologiques sont disponibles pour la maladie de Parkinson.

Meningeal Relapse of Nodular Lymphocyte Predominant Hodgkin Lymphoma Transformed to T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Case Report.

Central nervous system involvement in Hodgkin lymphoma is extremely rare, especially in nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), which usually carries a favorable prognosis. Here we report a case of a young patient with NLPHL, who developed a progressive and fatal neurological deterioration requiring a very extensive work-up including two biopsies to obtain the diagnosis of T-cell/histiocyte-rich large B-cell lymphoma like transformation. This report, which includes post-mortem analysis, highlights the correlations between clinical, radiological, and biological data but also the difficulties encountered in reaching the correct diagnosis.