Migraine treatment: Position paper of the French Headache Society
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.
Imaging of impulse control disorders in Parkinson's disease
Impulse control disorders (ICD) are frequent and cumbersome behavioral disorders in patients with Parkinson's disease (PD). Understanding their pathophysiological underpinnings is crucial. Molecular imaging using positron emission tomography (PET) and single-photon emission computed tomography (SPECT) clearly indicates preexisting vulnerability and abnormal sensitization of the pre- and postsynaptic dopaminergic system. Functional magnetic resonance imaging (fMRI) studies reveal abnormal connectivity within the reward system involving the ventral striatum and orbitofrontal cortex. These alterations pinpoint the dysfunction of reinforcement learning in ICD, which is biased toward the overvaluation of reward and underestimation of risk, and the deficit in inhibitory control mechanisms related to abnormal connectivity within and between the limbic and the associative and motor networks.
Reprint of: Scientific statement from the French neurovascular and cardiac societies for improved detection of atrial fibrillation after ischaemic stroke and transient ischaemic attack
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.
Mediterranean-like diets in multiple sclerosis: A systematic review
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.
Adding corticosteroids to galcanezumab in medication overuse headache: A three-arm head-to-head prospective observational cohort study
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.
History and novelties in autoimmune encephalitis and paraneoplastic neurological syndromes
Inflammatory myopathies in 2024: Better classify them to better treat them
The discovery, over the last forty years or so, of specific myositis auto-antibodies (easily dosed in routine nowadays) and the fine clinically and pathologically phenotypic descriptions of affected patients have made it possible to review the classification of inflammatory myopathies. The arrival of "omic" techniques has also led to the discovery of different pathophysiological mechanisms among these different subgroups of myositis. Naturally, therapeutic approaches specifically targeting the representative abnormal pathways of each subgroup are being evaluated. This modern approach to myositis, which is clinical, pathophysiological, and therapeutic in the making, is presented in this review article.
Iron overload revealing the cytoarchitecture of the red nucleus: A case study
The neurobiology and immunology of CASPR2-associated neurological disorders
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.
New treatment strategies in Myasthenia gravis
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.
MRI findings in autoimmune encephalitis
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.
Quantitative spinal cord imaging: Early ALS diagnosis and monitoring of disease progression
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.
Congenital myasthenic syndromes by Epsilon subunit mutations: Phenotypic profiles of 17 Algerian families
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.
French validation of the sexual complaints screener (SCS) for patients with multiple sclerosis
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.
Epidemiology of optic disc edema in 2021/2022: Results from a cohort of 197 patients
The aim of our study was to determine the etiologies of optic disc edema between 2021 and 2022.
Postural balance and visual dependence in patients with demyelinating neuropathies differ between acquired and hereditary etiologies
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.
Spinal and bulbar muscular atrophy: Kennedy's disease and its first description by Hiroshi Kawahara in 1897
Vanishing white matter in multiple mitochondrial dysfunction syndrome-2 due to compound heterozygosity for novel BOLA3 variants
Increased incidence of ischemic stroke in young: A population-based stroke registry study from 2008 to 2018
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.
Validation of the Arabic version of Parkinson's Disease Sleep Scale-Revised Version (PDSS-2)
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.