This narrative review concerned the studies performed on representative samples of the French general population carried out over more than thirty years and aims to provide an update on the French migraine epidemiology....This narrative review concerned the studies performed on representative samples of the French general population carried out over more than thirty years and aims to provide an update on the French migraine epidemiology. Eleven studies were selected (GRIM-1, MIG-ACCESS, GRIM-2, FRAMIG-2000, FRAMIG-3, GRIM-3, EUROLIGHT, IBMS, SNDS PACA/CORSICA, EGB FRANCE, CaMEO-I). The data extracted relates to four healings: prevalence and distribution, individual burden, recognition and care and societal burden.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. This degeneration leads to muscular weak...Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. This degeneration leads to muscular weakness, progressively impairing motor functions and ultimately resulting in respiratory failure. The clinical, genetic, and pathological heterogeneity of ALS, combined with the absence of reliable biomarkers, significantly challenge the efficacy of therapeutic trials. Despite these hurdles, neuroimaging, and particularly spinal cord imaging, has emerged as a promising tool. It provides insights into the involvement of both upper and lower motor neurons. Quantitative spinal imaging has the potential to facilitate early diagnosis, enable accurate monitoring of disease progression, and refine the design of clinical trials. In this review, we explore the utility of spinal cord imaging within the broader context of developing spinal imaging biomarkers in ALS. We focus on a both diagnostic and prognostic biomarker in ALS, highlighting its pivotal role in elucidating the disease's underlying pathology. We also discuss the existing limitations and future avenues for research, aiming to bridge the translational gap between academic research and its application in clinical practice and therapeutic trials.
Gaillard N, Deharo JC, Suissa L
… +8 more, Defaye P, Sibon I, Leclercq C, Alamowitch S, Guidoux C, Cohen A, French Neurovascular Society, French Society of Cardiology
Atrial fibrillation (AF) is the primary cause of ischaemic stroke and transient ischaemic attack (TIA). AF is associated with a high risk of recurrence, which can be reduced using optimal prevention strategies, mainly an...Atrial fibrillation (AF) is the primary cause of ischaemic stroke and transient ischaemic attack (TIA). AF is associated with a high risk of recurrence, which can be reduced using optimal prevention strategies, mainly anticoagulant therapy. The availability of effective prophylaxis justifies the need for a significant, coordinated and thorough transdisciplinary effort to screen for AF associated with stroke. A recent French national survey, initiated and supported by the Société française neurovasculaire (SFNV) and the Société française de cardiologie (SFC), revealed many shortcomings, such as the absence or inadequacy of telemetry equipment in more than half of stroke units, insufficient and highly variable access to monitoring tools, delays in performing screening tests, heterogeneous access to advanced or connected ambulatory monitoring techniques, and a lack of dedicated human resources. The present scientific document has been prepared on the initiative of the SFNV and the SFC with the aim of helping to address the current shortcomings and gaps, to promote efficient and cost-effective AF detection, and to improve and, where possible, homogenize the quality of practice in AF screening among stroke units and outpatient post-stroke care networks. The working group, composed of cardiologists and vascular neurologists who are experts in the field and are nominated by their peers, reviewed the literature to propose statements, which were discussed in successive cycles, and maintained, either by consensus or by vote, as appropriate. The text was then submitted to the SFNV and SFC board members for review. This scientific statement document argues for the widespread development of patient pathways to enable the most efficient AF screening after stroke. This assessment should be carried out by a multidisciplinary team, including expert cardiologists and vascular neurologists.
BACKGROUND: Medication overuse headache (MOH) is a condition where pain relief medications cause chronic headaches due to excessive use. Recent advancements highlight the effectiveness of preventive treatments like anti-...BACKGROUND: Medication overuse headache (MOH) is a condition where pain relief medications cause chronic headaches due to excessive use. Recent advancements highlight the effectiveness of preventive treatments like anti-CGRP monoclonal antibodies. Current strategies combine medication withdrawal and preventive treatments, with corticosteroids traditionally used to ease withdrawal symptoms. METHODS: This is a prospective three-arm observational cohort study comparing the effectiveness and safety of galcanezumab alone, galcanezumab plus prednisone and prednisone alone for the treatment of MOH. We enrolled 75 patients. Prednisone was administered at an initial dose of 50mg daily, and then tapered off over 28days. Duration of follow-up was 3months. RESULTS: All treatments proved effective (P<0.001). We found a significant reduction of mean monthly days with headache in the galcanezumab plus prednisone group (baseline: 25, IQR: 20-30; after 3months: 7, IQR: 5-10), in the galcanezumab group (baseline: 25, IQR: 20-30; after 3months: 10, IQR: 5-14) and in the Prednisone group (baseline: 25, IQR: 20-28; after 3months: median: 15 days, IQR: 8-22days). Patients treated with prednisone reported a higher incidence of side effects (P=0.002). CONCLUSION: Our study indicates that both galcanezumab and prednisone decrease the frequency of headaches in patients with MOH. The combined usage of these treatments showed the highest reduction in mean monthly headache days. However, treatment with prednisone determined a significant rate of adverse events, therefore we suggest its use only in unresponsive patients. In all other patients galcanezumab appears to be a safe and effective option.
OBJECTIVES: Parkinson's disease sleep scale (PDSS) was recommended by the Movement Disorder Society task force for screening and grading the severity of sleep problems in Parkinson's disease (PD). This work aimed to exam...OBJECTIVES: Parkinson's disease sleep scale (PDSS) was recommended by the Movement Disorder Society task force for screening and grading the severity of sleep problems in Parkinson's disease (PD). This work aimed to examine the validity and reliability of an Arabic version of PDSS-2. METHODS: This cross-sectional study was carried out on 133 patients fulfilling diagnostic criteria for PD. The patients were clinically assessed using the following scales: Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), modified Hoehn and Yahr Scale (H&Y), non-motor symptoms scale (NMSS), Beck Depression Inventory (BDI), and Pittsburgh Sleep Quality Index (PSQI). PDSS-2 scale was translated according to approved translation standards into Arabic and back-translated into English. Patients were asked to respond based on their experience in the last week. RESULTS: The mean PDSS2 score for the included PD patients was 17±10. Cronbach's α coefficient value was 0.89, indicating good internal consistency. Most items showed high item-total correlation; the lowest was 0.375, considered higher than the conventional cut-off of 0.3. Test-retest reliability showed good agreement (ICC: 0.848). The exploratory factor analysis showed that items had been loaded over four factors. Total PDSS-2 score was positively correlated to age, disease duration, modified H&Y scale, MDS-UPDRS, NMSS, BDI, and global PSQI score. A cut-off point of 13 could differentiate poor sleepers from good sleepers with 91% sensitivity and 70% specificity (AUC 0.893, P<0.001). CONCLUSION: The Arabic version of PDSS-2 has appropriate validity and can be reliably used for assessing sleep-related problems in Arabic-speaking patients with PD.
Retho E, Tasseng Y, Consigny M
… +10 more, Le Bourhis L, Leblanc A, Jourdain A, Merrien FM, Rouhart F, Viakhireva-Dovganyuk I, Goas P, Lavenant C, Bruguet M, Timsit S
BACKGROUND: Studying changing patterns in stroke incidence is essential since strokes represent a burden for public health. The aim of our study was to evaluate the epidemiological trend of strokes from 2008 to 2018 in t...BACKGROUND: Studying changing patterns in stroke incidence is essential since strokes represent a burden for public health. The aim of our study was to evaluate the epidemiological trend of strokes from 2008 to 2018 in the population-based Brest Stroke Registry (BSR) focusing on patients under 60. METHODS: All first-ever-stroke, patients aged 15 and older, with imaging, in the Pays-de-Brest from January 1st 2008 to December 31st 2017 were included. Cumulative incidence was calculated, by periods of two years, and temporal trends were evaluated by age group, sex, stroke subtype (ischemic or hemorrhagic) using a Poisson regression. Trends in stroke variables were specifically studied for young people. Ischemic stroke mechanism subtypes were analysed according to TOAST and SSS-TOAST classifications. RESULTS: Over a 10-year study period, there were 6043 first-ever-strokes: 738 intracerebral hemorrhage (ICH) and 5305 ischemic strokes (IS). Mean age was 73.55±14.25years, 82.2% were older than 60 and 51.8% were women. IS incidence increased in young people from 30.4 [95% CI: 27.4; 33.8] to 37.3 [95% CI: 33.9; 41.0] per 100,000 inhabitants (P=0.0025). IS incidence≥60 decreased except for last studied period. ICH remained stable for older people but decreased in young from 5.6 [4.3; 7.1] to 2.8 [2.0; 4.0] per 100,000 inhabitants (P=0.0250). Under 60, the main risk factors were smoking (68.6%); hypertension (27.8%); high-alcohol intake (24.4%) and dyslipidemia (21%). Most IS were minor. Work-up in the IS young population was for the large majority brain magnetic resonance imaging (MRI) (74.5%); cardiac monitoring (80%): echocardiography (83.5%); and imaging of the neck vessels (89.9%). Among IS, 19.8% were taking antihypertensive drugs and 10.9% statins. According to the TOAST classification, there were 46.7% cryptogenic strokes, which was reduced to 25.5% for other cryptogenic strokes, and 2.3% for cryptogenic embolism according SSS-TOAST classification. In IS, risk profiles, clinical parameters and prior-stroke treatments did not significantly change. Dyslipidemia and use of statins were fluctuant. Diagnostic work-up improved but the frequencies of IS mechanism subtypes remained stable. CONCLUSION: IS incidence increased while ICH incidence declined in young people. Major use of MRI may have contributed to the IS trend. The traditional risk factors studied remained stable and did not explain the increase in IS incidence. Improvement in diagnostic work-up did not lead to any change in subtypes of stroke mechanisms. Using the SSS-TOAST classification reduced the rate of cryptogenic strokes. The role of non-traditional risk factors may be explored in an attempt to explain increased incidence.
BACKGROUND: Mediterranean-like diet is an anti-inflammatory diet with high-fiber consumption and lower intake of saturated fatty acids which is proposed to have beneficial effects in patients with multiple sclerosis (MS)...BACKGROUND: Mediterranean-like diet is an anti-inflammatory diet with high-fiber consumption and lower intake of saturated fatty acids which is proposed to have beneficial effects in patients with multiple sclerosis (MS). This investigation aims to explore the impacts of this style of diet on people living with MS, based on clinical evidence. METHODS: This study was conducted following the 2020 version of the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. Both interventional and observational clinical studies which evaluated the effects of Mediterranean-like diets on MS patients were considered for inclusion. Review articles, letters, commentaries, case reports, non-English papers, and conference abstracts were excluded. PubMed, Web of Science, Scopus, and EMBASE databases were searched until March 23rd, 2023, and risk of bias in randomized-controlled trials (RCTs) was evaluated based on the second version of the Cochrane RoB assessment tool (RoB.2). In addition, for the observational studies, Joanna Briggs Institute (JBI)'s critical appraisal tools were utilized. RESULTS: Of 161 records that were screened in the title/abstract stage, 13 reports of 11 studies were included in the systematic review. Three RCTs (including one pilot RCT), and eight observational studies reported the effects of Mediterranean-like diets on people living with MS. The sample sizes in clinical trials varied between 36 and 147 and for observational studies between 30 and 563 patients. Evidence suggested positive effects of a Mediterranean-like diet on inflammatory status and MS-related symptoms such as fatigue, quality of life, attack rate, and cognitive dysfunction. DISCUSSION: This systematic review pointed out possible beneficial effects of Mediterranean-like diets for MS patients. The limited number of well-designed RCTs was the main limitation of this study; therefore, large-scale multiple-center interventional studies are suggested. Variety in the assessed outcomes, study designs, and groups of the studies prevented meta-analysis which was the other limitation of this study.
BACKGROUND: Demyelinating polyneuropathies affect posture and can be either hereditary, as in Charcot-Marie-Tooth type 1A (CMT1A), or autoimmune, as in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Cl...BACKGROUND: Demyelinating polyneuropathies affect posture and can be either hereditary, as in Charcot-Marie-Tooth type 1A (CMT1A), or autoimmune, as in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Clinical differentiation between these two neuropathies can be challenging and biomarkers are lacking. No comparative analysis of their balance profiles has been conducted. METHODS: The postural balance of 23 patients with CIDP and 23 patients with CMT1A, matched for age, sex, and functional scores, were recorded using a force platform under various conditions. The effects of visual dependence were examined based on center of pressure velocity, 90% confidence ellipse area, and the Romberg quotient which represents the ratio between posturography with eyes closed and eyes open. RESULTS: With eyes open, the two groups exhibited similar area and velocity. They increased their postural sway when visual input was eliminated. Nevertheless, the increase in postural sway was less pronounced in CMT1A patients than in patients with CIDP, who then had a higher Romberg quotient. CONCLUSION: Patients with CMT1A appear to have developed compensatory mechanisms over time resulting in reduced visual dependence. Further studies are necessary to explore other compensatory mechanisms of equilibrium that could be targeted by rehabilitation for patients with CIDP.
OBJECTIVE: The aim of our study was to determine the etiologies of optic disc edema between 2021 and 2022. MATERIALS AND METHODS: This was a multicentric study at the Timone and Nord university hospitals in Marseille. Pa...OBJECTIVE: The aim of our study was to determine the etiologies of optic disc edema between 2021 and 2022. MATERIALS AND METHODS: This was a multicentric study at the Timone and Nord university hospitals in Marseille. Patients were retrospectively followed in ophthalmology departments, with inclusion between January 2021 and December 2022. All patients presenting with newly diagnosed uni- or bilateral optic disc edema, both adults and children, were included. Their ophthalmological evaluation included a fundus examination and optical coherence tomography if feasible. RESULTS: In total, 197 patients were included. Intracranial hypertension (IH) was the most frequent etiology (37.06%). The primary causes of IH were idiopathic (27/73), intracranial tumors (21/73), and cerebral venous thrombosis (12/73). The second etiology of optic disc edema was retinal vein occlusion in 19.9% of cases (39/197). Edema reactive to uveitis was found in 13.2% of cases (26/197). Finally, inflammatory (17/197) and ischemic (30/197) optic neuropathies were identified. CONCLUSION: This study updates the most frequent etiologies of optic disc edema in 2021 and 2022 to facilitate diagnostic hypotheses for de novo optic disc edema. It highlights the importance of a comprehensive and personalized evaluation in diagnosing optic disc edema, taking into account recent advances in imaging techniques and biomarkers.
Moisset X, Demarquay G, de Gaalon S
… +13 more, Roos C, Donnet A, Giraud P, Guégan-Massardier E, Lucas C, Mawet J, Valade D, Corand V, Gollion C, Moreau N, Grangeon L, Lantéri-Minet M, Ducros A
The French migraine management recommendations were published in 2021. However, in the last three years, new data have come to light and new drugs have been approved (eptinezumab, rimegepant and atogepant) by the Europea...The French migraine management recommendations were published in 2021. However, in the last three years, new data have come to light and new drugs have been approved (eptinezumab, rimegepant and atogepant) by the European Medicines Agency that require us to take a position on their use and to update certain elements of the recommendations. The first important message concerns the position of the French Headache Society on the use of preventive treatments (monoclonal antibodies and gepants) targeting the calcitonin gene-related peptide (CGRP) pathway. In terms of efficacy and safety, and as suggested by other national headache societies, these treatments can be offered as first-line treatment, although the scope defined by the French national health authority for possible reimbursement is limited to patients with severe migraine, at least eight headache days per month and for whom two previous preventive treatments have failed. Another important change concerns the position of topiramate as a preventive treatment for migraine in women of childbearing age. This treatment has been proposed as a first-line treatment for chronic migraine. However, recent pharmacovigilance data have highlighted a potential adverse effect on neurodevelopment in children exposed in utero. As a result, this treatment is formally contraindicated during pregnancy and must be used with extreme caution in women of childbearing age (effective contraception, no therapeutic alternative available and annual follow-up as with valproate). It can therefore no longer be offered as first-line treatment for women of childbearing age.
BACKGROUND: Sexual dysfunctions are frequent in the general population and even more frequent in individuals with chronic neurological disorders like multiple sclerosis (MS). Several studies indicate that patients would...BACKGROUND: Sexual dysfunctions are frequent in the general population and even more frequent in individuals with chronic neurological disorders like multiple sclerosis (MS). Several studies indicate that patients would like healthcare workers to address sexual problems. Indeed, such complaints are not currently sufficiently detected and treated. In clinical practice, a screening tool is lacking to help French-speaking patients and healthcare professionals address this issue. OBJECTIVE: The main aim of this study was to evaluate the reliability and validity of the French adaptation of the self-assessment Sexual Complaints Screener scale for Women (SCS-W) and Men (SCS-M). METHOD: A prospective study was conducted among patients with MS in two centers. The SCS-W was adapted into French (QPS-F: questionnaire de plaintes sexuelles - Femmes) and compared to the reference questionnaire Female Sexual Function Index-19 (FSFI-19). The SCS-M was adapted into French (QPS-H: questionnaire de plaintes sexuelles - Hommes) and compared to the reference International Index of Erectile Function-15 (IIEF-15). RESULTS: Included were 101 women and 35 men with MS. Median age was 40.5 (range: 20-68) years. Based on the Cronbach alpha coefficient, the internal coherence of the QPS in French was 0.89 for women (QPS-F) and 0.71 for men (QPS-H), indicating high reliability. For QPS-F, the bivariate Pearson correlation coefficient indicated good convergence for desire and satisfaction, and average convergence for orgasm, pain, and arousal excitability. For QPS-H, the convergence was good for desire, pleasure, and ejaculation. CONCLUSION: The French versions of the SCS-W/M scales, namely QPS-F and QPS-H, are reliable and validated tools compared with the reference questionnaires, FSFI and IIEF-15, respectively. The QPS-F/H are useful tools for brief, simple, and accurate screening and assessment of sexual complaints. They provide supportive information for clinicians who are less familiar with the clinical significance of sexual complaints and hence can be helpful to achieve more adapted care. These scales are adapted, but not specific, to MS. They could be used in other pathologies and the general population.
BACKGROUND: Congenital myasthenic syndromes (CMS) are a heterogeneous group of rare genetic disorders. The acetyl choline receptor contains five subunits, with a predominance of mutations affecting the epsilon subunit ge...BACKGROUND: Congenital myasthenic syndromes (CMS) are a heterogeneous group of rare genetic disorders. The acetyl choline receptor contains five subunits, with a predominance of mutations affecting the epsilon subunit gene called cholinergic receptor nicotinic epsilon (CHRNE) gene. OBJECTIVE: To study the clinical phenotype of 17 families with CHRNE gene mutations. METHODS: We report a series of 17 families with 22 affected patients carrying different mutations encoding CHRNE proteins. RESULTS: We studied their clinical and biological phenotypes, as well as their evolutionary profile and their response to the different therapies proposed. A phenotypic comparison was made between the families carrying the founding Maghrebian mutation and the other mutations found in this series. CONCLUSION: The CHRNE gene mutations are the most frequent ones in CMS. The phenotypes reported in this study are heterogeneous, and can depend on the causative mutation.
Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by muscle weakness and fatigue. The disease is primarily caused by antibodies targeting acetylcholine receptors (AChR) and muscle-specif...Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by muscle weakness and fatigue. The disease is primarily caused by antibodies targeting acetylcholine receptors (AChR) and muscle-specific kinase (MuSK) proteins at the neuromuscular junction. Traditional treatments for MG, such as acetylcholinesterase inhibitors, corticosteroids, and immunosuppressants, have shown efficacy but are often associated with significant long-term side effects and variable patient response rates. Notably, approximately 15% of patients exhibit inadequate responses to these standard therapies. Recent advancements in molecular therapies, including monoclonal antibodies, B cell-depleting agents, complement inhibitors, Fc receptor antagonists, and chimeric antigen receptor (CAR) T cell-based therapies, have introduced promising alternatives for MG treatment. These novel therapeutic approaches offer potential improvements in targeting specific immune pathways involved in MG pathogenesis. This review highlights the progress and challenges in developing and implementing these molecular therapies. It discusses their mechanisms, efficacy, and the potential for personalized medicine in managing MG. The integration of new molecular therapies into clinical practice could significantly transform the treatment landscape of MG, offering more effective and tailored therapeutic options for patients who do not respond adequately to traditional treatments. These innovations underscore the importance of ongoing research and clinical trials to optimize therapeutic strategies and improve the quality of life for individuals with MG.
Autoimmune encephalitis encompasses a spectrum of conditions characterized by distinct clinical features and magnetic resonance imaging (MRI) findings. Here, we review the literature on acute MRI changes in the most comm...Autoimmune encephalitis encompasses a spectrum of conditions characterized by distinct clinical features and magnetic resonance imaging (MRI) findings. Here, we review the literature on acute MRI changes in the most common autoimmune encephalitis variants. In N-methyl-D-aspartate (NMDA) receptor encephalitis, most patients have a normal MRI in the acute stage. When lesions are present in the acute stage, they are typically subtle and non-specific white matter lesions that do not correspond with the clinical syndrome. In some NMDA receptor encephalitis cases, these T2-hyperintense lesions may be indicative of an NMDA receptor encephalitis overlap syndrome with simultaneous co-existence of multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Encephalitis with leucine-rich glioma-inactivated 1 (LGI1)-, contactin-associated protein-like 2 (CASPR2)- or glutamic acid decarboxylase (GAD)- antibodies typically presents as limbic encephalitis (LE) with unilateral or bilateral T2/fluid attenuated inversion recovery (FLAIR) hyperintensities in the medial temporal lobe that can progress to hippocampal atrophy. Gamma aminobutyric acid-B (GABA-B) receptor encephalitis also often shows such medial temporal hyperintensities but may additionally involve cerebellar lesions and atrophy. Gamma aminobutyric acid-A (GABA-A) receptor encephalitis features multifocal, confluent lesions in cortical and subcortical areas, sometimes leading to generalized atrophy. MRI is unremarkable in most patients with immunoglobulin-like cell adhesion molecule 5 (IgLON5)-disease, while individual case reports identified T2/FLAIR hyperintense lesions, diffusion restriction and atrophy in the brainstem, hippocampus and cerebellum. These findings highlight the need for MRI studies in patients with suspected autoimmune encephalitis to capture disease-specific changes and to exclude alternative diagnoses. Ideally, MRI investigations should be performed using dedicated autoimmune encephalitis imaging protocols. Longitudinal MRI studies play an important role to evaluate potential relapses and to manage long-term complications. Advanced MRI techniques and current research into imaging biomarkers will help to enhance the diagnostic accuracy of MRI investigations and individual patient outcome prediction. This will eventually enable better treatment decisions with improved clinical outcomes.
CASPR2-associated neurological disorders encompass a wide clinical spectrum broadly divided into overlapping three autoimmune syndromes: CASPR2 limbic encephalitis, Morvan syndrome, and Isaacs syndrome. CASPR2 is a neuro...CASPR2-associated neurological disorders encompass a wide clinical spectrum broadly divided into overlapping three autoimmune syndromes: CASPR2 limbic encephalitis, Morvan syndrome, and Isaacs syndrome. CASPR2 is a neuronal protein expressed at different sites in the central and peripheral nervous system and has a variety of roles and functions regarding neuronal excitability, synaptic plasticity, and homeostasis of inhibitory networks, most of which are only partially understood. CASPR2 antibodies have various pathogenic effects including internalization of CASPR2, disruption of protein-protein interactions, and, possibly, complement activation. Their pathogenic effect is well demonstrated in the limbic encephalitis phenotype, but the role of pathogenic antibodies in the development of other clinical manifestations is less clear. CASPR2 limbic encephalitis also differ from the other CASPR2-associated disorders in regard to HLA allele and paraneoplastic associations, suggesting it has immunological mechanisms distinct from the other clinical forms. Future studies are needed to better understand how the immunological alterations lead to the different phenotypes associated with CASPR2 antibodies.