Revue Neurologique (REV NEUROL-FRANCE)
Au service de l'actualité neurologique. La Revue Neurologique publie des mises au point, des mémoires originaux, des brèves communications, des lettres de l'Editeur... en neurologie, neurochirurgie, neurophysiologie, neuropathologie, neurologie expérimentale et autres disciplines associées. Tribune des équipes francophones et internationales. Organe de diffusion de la recherche francophone et internationale, elle publie des articles scientifiques qui vous permettent de vous tenir informé des grandes acquisitions en neurologie clinique et en neurosciences. Un renouveau éditorial. Soucieuse de maintenir sa place parmi les différents outils de travail des neurologues, la Revue Neurologique maintient une ligne éditoriale de qualité pour répondre aux exigences de ses lecteurs. Soutenue par un Comité Editorial chargé de susciter la soumission d'articles d'équipes de premier rang, la rédaction a mis au point une partie de formation post-universitaire avec un grand nombre de rubriques tres variées
Journal Impact: 0.20*
Journal impact history
|2016 Journal impact||Available summer 2017|
|2015 Journal impact||0.20|
|2014 Journal impact||0.19|
|2013 Journal impact||0.25|
|2012 Journal impact||0.48|
|2011 Journal impact||0.36|
|2010 Journal impact||0.38|
|2009 Journal impact||0.31|
|2008 Journal impact||0.28|
|2007 Journal impact||0.35|
|2006 Journal impact||0.31|
|2005 Journal impact||0.35|
|2004 Journal impact||0.49|
|2003 Journal impact||0.33|
|2002 Journal impact||0.31|
|2001 Journal impact||0.39|
|2000 Journal impact||0.37|
Journal impact over time
|Website||Revue Neurologique website|
|Material type||Internet resource|
|Document type||Internet Resource, Computer File, Journal / Magazine / Newspaper|
Publications in this journal
- [Show abstract] [Hide abstract] ABSTRACT: Auditory verbal hallucinations (AVH) are among the most characteristic symptoms of schizophrenia and have been linked to likely disturbances of structural and functional connectivity within frontal, temporal, parietal and sub-cortical networks involved in language and auditory functions. Resting-state functional magnetic resonance imaging (fMRI) has shown that alterations in the functional connectivity activity of the default-mode network (DMN) may also subtend hallucinations. Non-invasive neurostimulation techniques such as repetitive transcranial magnetic stimulation (rTMS) have the ability to modulate activity of targeted cortical sites and their associated networks, showing a high potential for modulating altered connectivity subtending schizophrenia. Notwithstanding, the clinical benefit of these approaches remains weak and variable. Further studies in the field should foster a better understanding concerning the status of networks subtending AVH and the neural impact of rTMS in relation with symptom improvement. Additionally, the identification and characterization of clinical biomarkers able to predict response to treatment would be a critical asset allowing better care for patients with schizophrenia.
- [Show abstract] [Hide abstract] ABSTRACT: Nemaline myopathy (NM) is one of the most common forms of congenital myopathy. The condition is defined by the histopathological finding of nemaline bodies (rods) on muscle biopsy and is associated with hypotonia and muscle weakness. The clinical spectrum encompasses lethal forms presenting in the neonatal period with profound weakness and less severe congenital diseases of later onset. NM is significantly heterogeneous from a genetic point of view, and its inheritance can be autosomal-dominant (AD), sporadic or autosomal-recessive (AR). To date, 11 genes encoding proteins of skeletal muscle thin filaments, Kelch domain-associated proteins and an unconventional myosin have been implicated in NM. The mechanisms leading to nemaline body formation and muscle weakness are still largely unclear. This report reviews the clinical, histopathological and genetic features of NM, with a focus on some of the recently discovered forms.
- [Show abstract] [Hide abstract] ABSTRACT: We are approaching the centenary of the first description of Guillain-Barré syndrome. The past 30 years had witnessed an amazing progress in the understanding of the immunological and pathological mechanisms of this disorder. We now recognize that Guillain-Barré syndrome is remarkably heterogeneous and under this umbrella term are several variants and subtypes with distinct clinical, electrophysiological and immunopathological features. This review is a historical journey, through a personal perspective, following the milestones that led to the current substantial knowledge of Guillain-Barré syndrome.
- [Show abstract] [Hide abstract] ABSTRACT: Sporadic inclusion-body myositis (sIBM) is the most frequent myopathy after 50 years of age. As the clinical presentation may often be typical, pathological confirmation by muscle biopsy appears necessary, but sometimes difficult. Further delineation of the framework of this particular disease, especially during its early-onset stage, appears to be challenging. New classification of diagnostic criteria as well as the identification of new diagnostic hallmarks appear to be the two main tools towards to achieve this purpose. sIBM pathophysiology has long been discussed and remains yet controversial. Since its initial description, there have been two major pathogenic hypotheses: inflammatory and degenerative. To date, the debate is still ongoing, as recent works support both pathophysiological mechanisms, although the inflammatory process seems to be slightly more preeminent in the recent literature. Treatment remains the most disappointing aspect of the disease as, despite various therapeutic attempts, no significant efficacy has been reported thus far. Nevertheless, advances in our pathophysiological understanding of the disease are paving the way for further therapeutic perspectives that might arise in the years to come. The objective of the present work was to summarize the most significant data published on sIBM during the past 2 years.
- [Show abstract] [Hide abstract] ABSTRACT: Neurography and EMG are complementary techniques used in the diagnosis and monitoring of neuropathies. Both assess function of the peripheral nervous system and provide clinically useful information regarding the functional status of peripheral nerves. This information is not readily obtainable using biochemical, genetic or imaging techniques. I will discuss the role of these techniques in the diagnosis and management of neuropathies and some limitations of these techniques. These methods are routinely used in an EMG lab. These are most useful when used in conjunction with clinical examination to answer a well-defined clinical question. Reference values are required for interpretation of the data.
- [Show abstract] [Hide abstract] ABSTRACT: Myofibrillar myopathies (MFM) are mostly adult-onset diseases characterized by progressive morphological alterations of the muscle fibers beginning in the Z-disk and the presence of protein aggregates in the sarcoplasm. They are mostly caused by mutations in different genes that encode Z-disk proteins, including DES, CRYAB, LDB3, MYOT, FLNC and BAG3. A large family of French origin, presenting an autosomal dominant pattern, characterized by cardiac arrhythmia associated to late-onset muscle weakness, was evaluated to clarify clinical, morphological and genetic diagnosis. Muscle weakness began during adult life (over 30 years of age), and had a proximal distribution. Histology showed clear signs of a myofibrillar myopathy, but with unusual, large inclusions. Subsequently, genetic testing was performed in MFM genes available for screening at the time of clinical/histological diagnosis, and desmin (DES), αB-crystallin (CRYAB), myotilin (MYOT) and ZASP (LDB3), were excluded. LMNA gene screening found the p.R296C variant which did not co-segregate with the disease. Genome wide scan revealed linkage to 7q.32, containing the FLNC gene. FLNC direct sequencing revealed a heterozygous c.3646T>A p.Tyr1216Asn change, co-segregating with the disease, in a highly conserved amino acid of the protein. Normal filamin C levels were detected by Western-blot analysis in patient muscle biopsies and expression of the mutant protein in NIH3T3 showed filamin C aggregates. This is an original FLNC mutation in a MFM family with an atypical clinical and histopathological presentation, given the presence of significantly focal lesions and prominent sarcoplasmic masses in muscle biopsies and the constant heart involvement preceding significantly the onset of the myopathy. Though a rare etiology, FLNC gene should not be excluded in early-onset arrhythmia, even in the absence of myopathy, which occurs later in the disease course.
- [Show abstract] [Hide abstract] ABSTRACT: Facioscapulohumeral muscular dystrophy (FSHD), an inherited and progressive muscle disorder, is among the most common hereditary muscle disorders. From a clinical vantage point, FSHD is characterized by weakness of the facial, shoulder (often with scapular winging), arm (including biceps and triceps) and abdominal muscles. Forearm muscles are usually spared and weakness is usually asymmetrical. Over the past few decades, muscle magnetic resonance imaging (MRI) has become established as a reliable and accurate noninvasive tool for the diagnosis and assessment of progression in neuromuscular diseases, showing specific patterns of muscle involvement for a number of myopathies. More recently, MRI has been used to noninvasively identify quantitative biomarkers, allowing evaluation of the natural progression of disease and assessment of therapeutic interventions. In the present review, the intention was to present the most significant MRI developments related to diagnosis and pattern recognition in FSHD and to discuss its capacity to provide outcome measures.
- [Show abstract] [Hide abstract] ABSTRACT: Chronic inflammatory demyelinating polyradiculoneuropathy is an orphan disease of poorly understood cause. While first line treatments with corticosteroids, intravenous immunoglobulin and plasma exchange have at least short-term efficacy, no trial has shown that immunosuppressants work. In our dream, we will take advantage of the recently improved EU regulations to launch a Europe wide trial which will investigate the cause of the disease. It will compare three parallel groups, the anti-B cell agent rituximab, the anti-T cell agent abatacept and usual care. The trial will not be blinded and the design will be very simple. The primary outcome measure will be improvement from baseline of the overall neuropathy limitations scale (ONLS) score by 1 or more grades at 12 weeks without increase in concomitant corticosteroids or IVIg or use of plasma exchange. There will be an option to substitute improvement in the Rasch-built overall disability scale depending on future experience with that scale as the primary outcome measure. The trial will require 3 groups of 60 participants to detect an increase from 20% in the usual care group to 30% with one of the other agents with a power of 90% and P-value of 5%. It will be larger than any trial of an immunosuppressant agent so far performed in CIDP. However, recruitment will be easier because inclusion criteria will be broad and allow randomisation of any patient in whom their neurologist wishes to introduce an immunosuppressant. Avoidance of blinding and use of simple monitoring with facetime will simplify running the trial and reduce expense. The trial will follow participants and measure outcomes at 12 months. Other outcomes will consist only of grip strength, time to walk 10 m and Euroqol, the last allowing us to estimate the cost per QALY of rituximab or abatacept. Even including central analysis of key biomarkers, the trial will only cost 3 million euros, a fraction of the cost of the usual phase III pharmaceutical company trial.
Article: Myofibrillar and distal myopathies[Show abstract] [Hide abstract] ABSTRACT: Distal myopathies and myofibrillar myopathies are both rare subcategories of muscle diseases. Myofibrillar myopathies are genetically heterogeneous group of diseases characterized by distinctive histopathology of abnormal protein aggregations and myofibrillar disintegration. All genes causing myofibrillar myopathy encode proteins that either reside in or associate with the Z-disc. Distal myopathies are also genetically heterogeneous muscular dystrophies in which muscle weakness presents distally in the feet and/or hands. A subgroup of distal myopathies, desminopathy, distal myotilinopathy, ZASPopathy and alpha-B crystallin-mutated distal myopathy, belong to myofibrillar myopathies and show similar pathological changes in muscle biopsies. Common features of these diseases are dominant inheritance and adult-onset of symptoms starting in the feet and slowly progressing to encompass other muscle groups. Cardiomyopathy is not a common feature in distal MFM myopathies.
- [Show abstract] [Hide abstract] ABSTRACT: Metabolic myopathies encompass muscle glycogenoses (GSD) and disorders of muscle fat oxidation (FAOD). FAODs and GSDs can be divided into two main clinical phenotypes; those with static symptoms related to fixed muscle weakness and atrophy, and those with dynamic, exercise-related symptoms that are brought about by a deficient supply of ATP. Together with mitochondrial myopathies, metabolic myopathies are unique among muscle diseases, as the limitation in exercise performance is not solely caused by structural damage of muscle, but also or exclusively related to energy deficiency. ATP consumption can increase 50–100-fold in contracting, healthy muscle from rest to exercise, and testing patients with exercise is therefore an appropriate approach to disclose limitations in work capacity and endurance in metabolic myopathies. Muscles rely almost exclusively on muscle glycogen in the initial stages of exercise and at high work intensities. Thus, patients with GSDs typically have symptoms early in exercise, have low peak work capacities and develop painful contractures in exercised muscles. Muscle relies on fat oxidation at rest and to a great extent during prolonged exercise, and therefore, patients with FAODs typically develop symptoms later in exercise than patients with GSDs. Due to the exercise-related symptoms in metabolic myopathies, patients generally have been advised to shun physical training. However, immobility is associated with multiple health issues, and may even cause unwanted metabolic adaptations, such as increased dependence on glycogen use and a reduced capacity for fatty acid oxidation, which is detrimental in GSDs. Training has not been studied systematically in any FAODs and in just a few GSDs. However, studies on single bouts of exercise in most metabolic myopathies show that particularly moderate intensity aerobic exercise is well tolerated in these conditions. Even low-intensity resistance training of short duration is tolerated in McArdle disease. Training in patients with FAOD potentially can also expand the metabolic bottleneck by increasing expression of the defective, but partially functional enzyme. Exercise performance in metabolic myopathies can be improved by different fuel supplementations and dietary interventions and should be considered as adjunct therapy to exercise training.
- [Show abstract] [Hide abstract] ABSTRACT: Mutations in the skeletal muscle ryanodine receptor (RYR1) gene are associated with a wide spectrum of inherited myopathies presenting throughout life. Malignant hyperthermia susceptibility (MHS)-related RYR1 mutations have emerged as a common cause of exertional rhabdomyolysis, accounting for up to 30% of rhabdomyolysis episodes in otherwise healthy individuals. Common triggers are exercise and heat and, less frequently, viral infections, alcohol and drugs. Most subjects are normally strong and have no personal or family history of malignant hyperthermia. Heat intolerance and cold-induced muscle stiffness may be a feature. Recognition of this (probably not uncommon) rhabdomyolysis cause is vital for effective counselling, to identify potentially malignant hyperthermia-susceptible individuals and to adapt training regimes. Studies in various animal models provide insights regarding possible pathophysiological mechanisms and offer therapeutic perspectives.
- [Show abstract] [Hide abstract] ABSTRACT: Characteristics of the intermediate filament proteins (IFPs) expressed during the development and cell differentiation of peripheral neurons are here reviewed. Neurofilament triplet proteins (NFPs), peripherin, α-internexin, synemin, syncoilin, nestin, vimentin and glial fibrillary acidic protein (GFAP) are each produced by different genes. NFPs, the most extensively studied, are thought to maintain axonal caliber, thus ensuring normal axonal transport, but this network is highly disrupted in several diseases, particularly motor neuron diseases. α-internexin has been proposed as the fourth NFP subunit. The relative plasticity of the peripherin network may account for its possible role during development, when axons have to find their targets, and when axons regenerate. In addition to their expression in muscle, other IFPs, such as syncoilin and synemin, are also expressed in neuronal tissues. Syncoilin modulates peripherin filament networks. Synemin M, associated with peripherin, is present in small unmyelinated fibers, whereas synemin L is produced in large neurons with myelinated fibers positive for the light-chain neurofilament (NF-L) subunit. Nestin is an IFP expressed in dividing cells during early stages of development in the central and peripheral nervous systems, and in muscles and other tissues. After differentiation, nestin is downregulated and replaced by tissue-specific IFPs. IFPs in glial cells are primarily composed of GFAP, although vimentin is also expressed; vimentin is also widely distributed in mesenchymal derivatives and established cell lines. In the peripheral nervous system, NFPs appear early in its development and progressively replace vimentin, which is expressed before NFPs in most, if not all, dividing neuroepithelial cells. In addition, in tissues undergoing an injury response, the unique and complex cell and tissue distribution of IFPs can be markedly modified.
- [Show abstract] [Hide abstract] ABSTRACT: Myoclonus is a sudden brief (20-250 ms) contraction (positive myoclonus), or a brief and sudden cessation of tonic muscle (negative myoclonus) inducing a simple jerky movement of body part. Myoclonus could have different origins in almost every part of the nervous system, from the cortex to the peripheral nerve, sharing a large panel of etiologies. It is regarded as the paradigmatic movement disorder causing jerks, although not the sole. This paper aims to depict the clinical and neurophysiological characteristics of myoclonus. It shows how neurophysiological investigations including surface polymyography and methods exploring cortical excitability, namely conventional EEG, EEG - jerk-locked back-averaging, somatosensory evoked potentials and C-reflex studies are required to define the generator of myoclonus in the central nervous system and clearly classify myoclonus as cortical, corticothalamic, subcortical - resulting from lesions or dysfunctions of basal ganglia/reticular system - or spinal. This paper also enlightens other movement disorders that may mimic myoclonus appearances, including psychogenic jerks, simple motor tics, spasms and startle syndromes. Finally, it raises few unresolved questions regarding the propriospinal myoclonus or peripheral myoclonus entities, the role of the cerebellum in myoclonic diseases and the relationship between cortical and epileptic myoclonus.
- [Show abstract] [Hide abstract] ABSTRACT: Paroxysmal movement disorders comprise both paroxysmal dyskinesia, characterized by attacks of dystonic and/or choreic movements, and episodic ataxia, defined by attacks of cerebellar ataxia. They may be primary (familial or sporadic) or secondary to an underlying cause. They can be classified according to their phenomenology (kinesigenic, non-kinesigenic or exercise-induced) or their genetic cause. The main genes involved in primary paroxysmal movement disorders include PRRT2, PNKD, SLC2A1, ATP1A3, GCH1, PARK2, ADCY5, CACNA1A and KCNA1. Many cases remain genetically undiagnosed, thereby suggesting that additional culprit genes remain to be discovered. The present report is a general overview that aims to help clinicians diagnose and treat patients with paroxysmal movement disorders.
Article: Current concepts of essential tremor[Show abstract] [Hide abstract] ABSTRACT: Essential tremor is clinically defined but there is increasing evidence that it is not a unique entity. Its pathophysiology has been studied with many methods but may also vary between subtypes. Neurophysiologically, there is strong evidence that a specific cerebello-thalamo-cortical loop is abnormally oscillating. The cause of its uncontrolled oscillation is not yet understood. The clear proof of a degenerative cause is still lacking and abnormal receptors or other causes of altered non-progressive functional disturbance cannot be excluded. Strong evidence supports the major involvement of the cerebellum and there is ample evidence that GABA is the main neurotransmitter involved in the pathophysiology in ET. Genetics have provided so far only a few rare subtypes which are due to specific mutations but there is no doubt that it is mostly a hereditary condition. There is evidence that the large subgroup of late onset tremor is a separate condition and this tremor is an independent risk factor for earlier mortality and comes with signs of premature aging (aging-related tremor). It will be important to improve phenotyping of patients in more detail possibly to include not only features of the tremor itself but also other clinical assessments like force measurements or cognitive testing. Based on these variables, we may be able to better understand the presumably different mechanisms underlying different variants of the disease.
- [Show abstract] [Hide abstract] ABSTRACT: Introduction: Hypnosis might represent an interesting complementary therapeutic approach to movement disorders, as it takes into account not only symptoms, but also well-being, and empowers patients to take a more active role in their treatment. Methods: Our review of the literature on the use of hypnosis to treat movement disorders was done by systematically searching the PubMed database for reports published between 1984 and November 2015. The following variables were extracted from each selected paper: study design; sample size; type of movement disorder; hypnotic procedure; treatment duration; and efficacy. Results: Thirteen papers were selected for detailed analysis. Most concerned tremor in Parkinson's disease and tics in Gilles de la Tourette syndrome. Although promising, the data were insufficient to allow conclusions to be drawn on the efficacy of hypnosis in movement disorders or to recommend its use in this setting. Conclusion: Well-designed studies taking into account some specific methodological challenges are needed to determine the possible therapeutic utility of hypnosis in movement disorders. In addition to the potential benefits for such patients, hypnosis might also be useful for studying the neuroanatomical and functional underpinnings of normal and abnormal movements.
- [Show abstract] [Hide abstract] ABSTRACT: Evidence-based medicine is a difficult goal to achieve in rare diseases where randomized controlled trials are lacking. This report provides guidelines that capitalize on both the literature and expertise of the French National Huntington Disease Reference Centre to optimalize pharmacological therapeutic interventions for Huntington's disease (HD).
- [Show abstract] [Hide abstract] ABSTRACT: Stereotypies have been defined as non-goal-directed movement patterns repeated continuously for a period of time in the same form and on multiple occasions, and which are typically distractible. Stereotypical motor behaviors are a common clinical feature of a variety of neurological conditions that affect cortical and subcortical functions, including autism, tardive dyskinesia, excessive dopaminergic treatment of Parkinson's disease and frontotemporal dementia. The main differential diagnosis of stereotypies includes tic disorders, motor mannerisms, compulsion and habit. The pathophysiology of stereotypies may involve the corticostriatal pathways, especially the orbitofrontal and anterior cingulated cortices. Because antipsychotics have long been used to manage stereotypical behaviours in mental retardation, stereotypies that present in isolation tend not to warrant pharmacological intervention, as the benefit-to-risk ratio is not great enough.
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