Risk factors associated with the severity of coronavirus disease 2019 (COVID-19) in patients with multiple sclerosis (MS) are unknown. Disease-modifying therapies (DMTs) may modify the risk of ...developing a severe COVID-19 infection, beside identified risk factors such as age and comorbidities.
To describe the clinical characteristics and outcomes in patients with MS and COVID-19 and identify factors associated with COVID-19 severity.
The Covisep registry is a multicenter, retrospective, observational cohort study conducted in MS expert centers and general hospitals and with neurologists collaborating with MS expert centers and members of the Société Francophone de la Sclérose en Plaques. The study included patients with MS presenting with a confirmed or highly suspected diagnosis of COVID-19 between March 1, 2020, and May 21, 2020.
COVID-19 diagnosed with a polymerase chain reaction test on a nasopharyngeal swab, thoracic computed tomography, or typical symptoms.
The main outcome was COVID-19 severity assessed on a 7-point ordinal scale (ranging from 1 not hospitalized with no limitations on activities to 7 death) with a cutoff at 3 (hospitalized and not requiring supplemental oxygen). We collected demographics, neurological history, Expanded Disability Severity Scale score (EDSS; ranging from 0 to 10, with cutoffs at 3 and 6), comorbidities, COVID-19 characteristics, and outcomes. Univariate and multivariate logistic regression models were used to estimate the association of collected variables with COVID-19 outcomes.
A total of 347 patients (mean SD age, 44.6 12.8 years, 249 women; mean SD disease duration, 13.5 10.0 years) were analyzed. Seventy-three patients (21.0%) had a COVID-19 severity score of 3 or more, and 12 patients (3.5%) died of COVID-19. The median EDSS was 2.0 (range, 0-9.5), and 284 patients (81.8%) were receiving DMT. There was a higher proportion of patients with a COVID-19 severity score of 3 or more among patients with no DMT relative to patients receiving DMTs (46.0% vs 15.5%; P < .001). Multivariate logistic regression models determined that age (odds ratio per 10 years: 1.9 95% CI, 1.4-2.5), EDSS (OR for EDSS ≥6, 6.3 95% CI. 2.8-14.4), and obesity (OR, 3.0 95% CI, 1.0-8.7) were independent risk factors for a COVID-19 severity score of 3 or more (indicating hospitalization or higher severity). The EDSS was associated with the highest variability of COVID-19 severe outcome (R2, 0.2), followed by age (R2, 0.06) and obesity (R2, 0.01).
In this registry-based cohort study of patients with MS, age, EDSS, and obesity were independent risk factors for severe COVID-19; there was no association found between DMTs exposure and COVID-19 severity. The identification of these risk factors should provide the rationale for an individual strategy regarding clinical management of patients with MS during the COVID-19 pandemic.
Background:
Leptomeningeal enhancement (LME) is a key feature of Susac syndrome (SuS) but is only occasionally depicted on post-contrast T1-weighted images (T1-WI).
Objective:
As post-contrast ...fluid-attenuated inversion recovery (FLAIR) may be more sensitive, our aim was to assess LME in SuS on this sequence.
Methods:
From 2010 to 2020, 20 patients with definite SuS diagnosis were retrospectively enrolled in this multicentre study. Two radiologists independently assessed the number of LME on post-contrast FLAIR and T1-WI acquisitions performed before any treatment. A chi-square test was used to compare both sequences and the interrater agreement was calculated.
Results:
Thirty-five magnetic resonance imagings (MRIs) were performed before treatment, including 19 post-contrast FLAIR images in 17 patients and 25 post-contrast T1-WI in 19 patients. In terms of patients, LME was observed on all post-contrast FLAIR, contrary to post-contrast T1-WI (17/17 (100%) vs. 15/19 (79%), p < 0.05). In terms of sequences, LME was observed on all post-contrast FLAIR, contrary to post-contrast T1-WI (19/19 (100%) vs. 16/25 (64%), p < 0.005). LME was disseminated at both supratentorial (19/19) and infratentorial (18/19) levels on post-contrast FLAIR, contrary to post-contrast T1-WI (3/25 and 9/25, respectively). Interrater agreement was excellent for post-contrast FLAIR (κ = 0.95) but only moderate for post-contrast T1-WI (κ = 0.61).
Conclusion:
LME was always observed and easily visible on post-contrast FLAIR images prior to SuS treatment. In association with other MRI features, it is highly indicative of SuS.
Whether some gray matter (GM) regions are differentially vulnerable at the early stages of MS is still unknown. The objective of this study is to investigate whether deep and cortical GM are ...differentially vulnerable after a clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS).
Fifty-six patients with CIS (PwCIS) and 38 healthy controls (HC) had conventional and diffusion tensor imaging (DTI) at baseline and 46 PwCIS and 20 HC were rescanned after 1 year. Deep GM (DGM) volumes, cortical thickness (CTh), and DTI metrics (FA: fractional anisotropy; MD: mean diffusivity) within these structures were calculated for each participant at each time-point and compared between PwCIS and HC. Linear regression models were used to investigate whether baseline DTI parameters could predict GM volume loss over time.
At baseline, GM volumes did not differ between PwCIS and HC, but hippocampal MD was higher in PwCIS than HC (
< 0.01). Over 1 year, GM alterations became more widespread with putamen and hippocampus volumes decreasing in PwCIS (
< 0.01), and cortical thinning in different parts of the cortex along with a significant increase of MD. Hippocampus MD at baseline could predict its volume loss (
= 0.159;
< 0.05) and cortical thinning was associated to microstructural damage (Spearman's rho ranging from -0.424 to -0.603 with
< 0.003).
Along with MS being a diffuse inflammatory disease, GM showed a differential vulnerability at the early stage spreading from hippocampus to the cortex. Hippocampus volume loss could be predicted by its MD at baseline.
•Women with multiple sclerosis require more information on reproductive health and prevention of cervical cancer.•Better contraceptive advice would reduce the number of unplanned pregnancies and ...avoid foetal exposure to potentially teratogenic treatment.•Long acting reversible contraceptives should be strongly encouraged.•Human papillomavirus vaccination should be promoted.•Close cervical screening should be recommended for patients on immunosuppressive treatments.
The gynaecological care of women with Multiple Sclerosis has received little attention; most reports focussed on pregnancy or sexuality. The objective of the present study was to evaluate if gynaecological follow-up for women of reproductive age with Multiple Sclerosis was adequate.
We performed a cross-sectional study on a large cohort of women with Multiple Sclerosis aged 18–40 years. All participants completed online questionnaires on general health status, gynaecological follow-up, and sexuality. Expanded Disability Status Scale (EDSS) scores were extracted from medical records. The study was registered in clinicaltrials.gov with the number NCT05248438, and in the European database ID-RCB with the number 2021-A02912–39.
Of the 192 patients who completed questionnaires, 157 (82.2%) reported gynaecological follow-up. Of the 155 patients on immunosuppressive treatments, only 31 (20%) underwent annual cervical screening. Of the 140 patients who met the French papillomavirus vaccination age recommendations, only 50 (35.7%) were vaccinated. A total of 128 (66.7%) patients used contraception. However, 16 (8.3%) patients reported unplanned pregnancies since the time of diagnosis.
Women with Multiple Sclerosis require more information on reproductive health and prevention of cancer. Better contraceptive advice would reduce the number of unplanned pregnancies and avoid foetal exposure to potentially teratogenic treatment.
Il existe peu de données concernant le suivi gynécologique des patientes atteintes de sclérose en plaques (SEP), la plupart des études portant sur la grossesse ou la sexualité.
L’objectif de ce ...travail est d’évaluer la qualité du suivi gynécologique chez les patientes en âge de procréer atteintes de SEP.
Nous avons réalisé une étude transversale chez des patientes avec SEP âgées de 18 à 40 ans suivies au CRC-SEP (Centre de Ressources et de Compétences de la SEP) du CHU de Bordeaux. Les participantes ont complété des questionnaires en ligne sur leur état de santé général, leur suivi gynécologique et leur sexualité. Les scores EDSS ont été extraits des dossiers médicaux.
Sur les 192 patientes de l’étude, 157 (82,2 %) ont rapporté avoir un suivi gynécologique. Sur les 155 patientes avec traitement immunosuppresseur, 31 (20 %) ont eu un frottis annuel. Sur les 140 patientes éligibles à la vaccination contre le human papillomavirus, 50 (35,7 %) ont été vaccinés. Une contraception est utilisée par 128 (66,7 %) patientes. Cependant, 16 (8,3 %) patientes rapportent des grossesses non planifiées depuis le diagnostic de SEP.
Le suivi gynécologique des patientes avec SEP peut être amélioré. Un dépistage cervical avec cytologie annuelle est à privilégier chez les patientes avec traitement immunosuppresseur. Il faut inciter à l’utilisation d’une contraception efficace pour diminuer les grossesses non planifiées.
Une meilleure information des professionnels de santé pourrait permettre d’améliorer le suivi gynécologique des patientes avec SEP. Il faut encourager la communication entre neurologues et gynécologues.
Introduction
No randomized controlled clinical trial of therapeutic plasma exchanges (TPE) has yet been performed for moderate‐to‐severe relapses of multiple sclerosis (MS).
Objective
To compare TPE ...to sham‐TPE in patients with a recent steroid‐resistant moderate‐to‐severe MS relapse.
Methods
Patients presenting with an MS relapse of less than 2 months without improvement and 15 days after a course of steroids were randomized. Specific criteria were used for each relapse type to define moderate‐to‐severe disability. The primary endpoint was the proportion of patients with at least a moderate improvement based on objective and functional evaluation after 1 month.
Results
Thirty‐eight patients were randomized. The intention‐to‐treat analysis included 14 patients in the TPE group and 17 in the Sham‐TPE group. The proportion of patients with at least moderate improvement at 1 month did not differ between the groups (P = .72), although 57.1% of the TPE group had full recovery compared with 17.6% of the sham group. Considering optic neuritis (ON), a significant difference in the proportion of different levels of improvement was observed in favor of the TPE group (P = .04). The combined Kurtzke's functional systems scores were significantly more improved in the TPE group than in the sham‐TPE group at months 1 (P < .01), 3 (P < .05), and 6 (P < .05). No major side effects were observed.
Conclusions
A significant difference between TPE and Sham‐TPE at the primary endpoint was only observed in patients with ON. Neurological function improved significantly more often in the TPE group than in the sham‐TPE group.
La gestion des grossesses sous Natalizumab est complexe en raison du risque de réactivation de la maladie à l’arrêt du Natalizumab et du peu de données disponibles sur l’exposition anténatale.
...Décrire les effets indésirables maternels et fœtaux suite à l’exposition au Natalizumab (NTZ) pendant la grossesse chez des patientes traitées pour une sclérose en plaques (SEP) récurrente rémittente.
Nous avons mené une étude observationnelle rétrospective au CHU de Bordeaux. Toutes les patientes atteintes de SEP rémittente récurrente, traitées par NTZ en hospitalisation de jour et ayant débuté une grossesse sous NTZ entre janvier 2014 et janvier 2019 ont été incluses. Les données recueillies ont concerné le déroulement de la grossesse et de l’accouchement, l’état de santé des enfants à la naissance ainsi que l’évolution clinique et radiologique de la SEP.
24 grossesses ont été décrites dont une interruption volontaire de grossesse et deux fausses couches spontanées. Concernant les 21 nouveau-nés, il n’a été observé ni malformation ni prématurité. Cependant, des anomalies hématologiques (thrombopénie ou anémie) spontanément réversibles ont été fréquemment notées. Parmi les 17 patientes traitées par NTZ pendant toute la grossesse (EDSS médian à la conception : 1,5), 2 ont présenté une poussée clinique traitée par corticoïdes au cours du post-partum.
Dans notre étude rétrospective, les données recueillies sur l’exposition anténatale au Natalizumab ont mis en évidence essentiellement des anomalies hématologiques chez les nouveau-nés, transitoires et sans manifestation clinique. Cependant une étude multicentrique avec un nombre de grossesses plus important est nécessaire. Un suivi prolongé après la naissance des enfants exposés au Natalizumab en anténatal serait également intéressant.
Concernant les grossesses entièrement exposées au Natalizumab, les données de notre étude semblent rassurantes avec des risques néonataux principalement hématologiques non graves à court terme et transitoires.
Few data are available regarding patients with very late-onset inflammatory demyelinating events. (VLO-IDE).
The aim of this study was to describe the clinical, biological, and radiological ...characteristics and aetiological diagnosis of very late first inflammatory demyelinating events of the central nervous system.
We conducted a national descriptive retrospective multicentre study on a case series of patients aged >70 years at the time of VLO-IDE. Patients were recruited from a national call on behalf of the 'Société Francophone de la Sclérose en Plaques' (French Multiple Sclerosis Society).
Twenty-five patients were referred (F:M sex ratio 2.1:1). The most frequent clinical impairment was a spinal cord deficit (23/25), usually severe (disability score, median EDSS 4.5 2-9.5). Spinal cord lesions were usually extensive, spanning at least three segments (11/25), and large brain lesions were also observed (lesions >20 mm in 6/25). The final aetiological diagnoses comprised multiple sclerosis (9/25), neuromyelitis optica spectrum disorders (7/25), neurosystemic lupus erythematosus (2/25), transverse myelitis without aetiological diagnosis (6/25) and optic neuritis (1/25).
This study highlights a particular phenotype of first clinical inflammatory demyelinating events in predominantly female patients aged >70 years who have severe motor impairment with common longitudinal extensive myelitis and large and common very active radiological inflammatory lesions. Neuromyelitis optica spectrum disorders seem overrepresented.
Background and purpose
Patients with acute ischaemic stroke and a large vessel occlusion who present to a non‐endovascular‐capable centre often require inter‐hospital transfer for thrombectomy. ...Whether the inter‐hospital transfer time is associated with 3‐month functional outcome is poorly known.
Methods
Acute stroke patients enrolled between January 2015 and December 2022 in the prospective French multicentre Endovascular Treatment of Ischaemic Stroke registry were retrospectively analysed. Patients with an anterior circulation large vessel occlusion transferred from a non‐endovascular to a comprehensive stroke centre for thrombectomy were eligible. Inter‐hospital transfer time was defined as the time between imaging in the referring hospital and groin puncture for thrombectomy. The relationship between transfer time and favourable 3‐month functional outcome (modified Rankin Scale 0–2) was assessed through a mixed logistic regression model adjusting for centre and symptom‐onset‐to‐referring‐hospital imaging time, age, sex, diabetes, referring hospital National Institutes of Health Stroke Scale score, Alberta Stroke Programme Early Computed Tomography Score, occlusion site and intravenous thrombolysis use.
Results
Overall, 3769 patients were included (median inter‐hospital transfer time 161 min, interquartile range 128–195; 46% with favourable outcome). A longer transfer time was independently associated with lower rates of favourable outcome (p < 0.001). Compared to patients with transfer time below 120 min, there was a 15% reduction in the odds of achieving favourable outcome for transfer times between 120 and 180 min (adjusted odds ratio 0.85; 95% confidence interval 0.67–1.07), and a 36% reduction for transfer times beyond 180 min (adjusted odds ratio 0.64; 95% confidence interval 0.50–0.81).
Conclusions
A shorter inter‐hospital transfer time is strongly associated with favourable 3‐month functional outcome. A speedier inter‐hospital transfer is of critical importance to improve outcome.
Le syndrome de la personne raide (SPR) se caractérise par une rigidité et des spasmes des membres et du tronc pouvant être associés à des anticorps anti-glutamate acid decarboxylase (GAD).
Décrire ...une cohorte rétrospective de SPR diagnostiqués depuis 2000 au CHU de Bordeaux avec prise en charge diagnostique et thérapeutique, initiales et lors du suivi.
Après interrogatoire du programme de médicalisation des systèmes d’information avec le mot-clé « syndrome de la personne raide », de la base de données du service neuromusculaire et du laboratoire d’immunologie concernant les anti-GAD positifs du 01/01/2000 au 07/12/2015, les dossiers informatisés ont été étudiés. Les SPR retenus comme diagnostics principaux ont été sélectionnés. Les caractéristiques cliniques, électrophysiologiques, biologiques et radiologiques ont été étudiées.
Sept cas, cinq femmes et deux hommes, ont été retenus, 5 ayant une forme généralisée et 2 une forme monomélique. L’âge moyen des premiers symptômes était de 60ans (± 12,3), le délai avant diagnostic était de 4 à 36 mois. Les patients avaient tous des pathologies auto-immunes. En première intention, des immunoglobulines intraveineuses et/ou une corticothérapie étaient prescrits, en association à un traitement symptomatique. En cas d’inefficacité ou d’intolérance, des immunosuppresseurs étaient utilisés.
Il existe une association systématique à des pathologies auto-immunes. Les données d’électroneuromyogramme étaient évocatrices de SPR dans 43 % des cas. Les thérapeutiques de première ligne étaient des immunoglobulines intraveineuses dans 86 % des cas. D’autres thérapeutiques ont pu être utilisées dans les formes résistantes (50 %) ou pour iatrogénicité (30 %). Les diagnostics différentiels seront discutés.
Le syndrome de la personne raide reste une entité rare de diagnostic difficile. Sa méconnaissance, au-delà de la sphère neurologique, peut entraîner un retard diagnostique de plusieurs mois à années.