[American Academy of Neurology, Washington, 18-25 April 2015]. / American Academy of Neurology, Washington, 18-25 avril 2015.

CEREBROVASCULAR DISEASES: The benefit of the thrombectomy using stents retrievers in the acute stroke phase is now demonstrated when there is a proximal occlusion of an intracranial artery, whatever its mechanism. The place of the anticoagulants in the management of cervical artery dissections remains uncertain, while the benefit of the blood pressure control in the secondary prevention of deep and lobar intracerebral hemorrhages is critical. The development of cardiac MRI, prolonged cardiac monitoring and transcranial doppler seems to improve the diagnosis of cardio-embolic sources of stroke. EPILEPSY: A specialized urgent-access single seizure clinic represents a model which reduces wait-times and improves patient access after a first fit. Co-locating a psychiatrist within outpatient epilepsy center leads to a reduction in psychiatric symptoms and people with psychogenic non-epileptic seizures. When neurologists around the world assess identical case scenarios for the diagnosis of epilepsy, concordance is between moderate and poor, showing that epilepsy diagnosis remains difficult. More than one third of elderly with new-onset epilepsy of unknown etiology exhibit temporal lobe atrophy on brain imaging. MOVEMENT DISORDERS: There is no major progress in the therapeutic approach of Parkinson's disease but the discovery of new genetic markers such as glucocerebrosidase mutations may greatly change our knowledge of the disease process and may induce new therapeutic strategies in the future. The natural history of the disease is also better understood from the prodromal phase to the post-mortem analysis of the brain and the classification of the processes based on abnormal protein deposits. DEMENTIA: The respective value of biomarkers (amyloid imaging versus CSF biomarkers) for in vivo diagnosis of Alzheimer's disease (AD) has been detailed. Therapeutic expectations mainly rely on anti-Aß immunization trials performed in preclinical (and no longer prodromal) stages of AD, with the aim of slowing the evolution of neuronal loss. Besides a lot of communications on dementia genetics or physiopathogeny, fascinating and promising results were presented on deep brain stimulation for depression resistant to medical treatment. PERIPHERAL NEUROPATHY: Ibudilast, administered with riluzole, is safe and tolerable in patients with amyotrophic lateral sclerosis (ALS), improves ALS function and delays progression. Patients with painful small fiber neuropathy have a high rate of mutations in the SCN9A gene, coding for Nav1.7 voltage-gated sodium-channels. Peripheral nerve lymphoma (NL) is a multifocal painful neuropathy that causes endoneurial inflammatory demyelination: primary NL is less severe than secondary NL, which occurs after remission, suggesting that nerve may be considered a "safe lymphoma haven". MULTIPLE SCLEROSIS (MS): Biotin in progressive forms of MS and daclizumab in relapsing-remitting forms appear to be promising treatments. In case of failure of current first-line and/or second-line therapeutics, alemtuzumab may be an interesting alternative treatment. Teriflunomide, dimethyl fumarate and fingolimod are oral treatments with confirmed efficacy and acceptable safety. Besides vitamin D insufficiency and smoking, which are confirmed risk factors for the disease, testosterone insufficiency (in males) and obesity are emerging risk factors, which could also be corrected.

Inherited peripheral neuropathies due to mitochondrial disorders.

Mitochondrial disorders (MIDs) are frequently responsible for neuropathies with variable severity. Mitochondrial diseases causing peripheral neuropathies (PNP) may be due to mutations of mitochondrial DNA (mtDNA), as is the case in MERRF and MELAS syndromes, or to mutations of nuclear genes. Secondary abnormalities of mtDNA (such as multiple deletions of muscle mtDNA) may result from mitochondrial disorders due to mutations in nuclear genes involved in mtDNA maintenance. This is the case in several syndromes caused by impaired mtDNA maintenance, such as Sensory Ataxic Neuropathy, Dysarthria and Ophthalmoplegia (SANDO) due to recessive mutations in the POLG gene, which encodes the catalytic subunit of mtDNA polymerase (DNA polymerase gamma), or Mitochondrial Neuro-Gastro-Intestinal Encephalomyopathy (MNGIE), due to recessive mutations in the TYMP gene, which encodes thymidine phosphorylase. The last years have seen a growing list of evidence demonstrating that mitochondrial bioenergetics and dynamics might be dysfunctional in axonal Charcot-Marie-Tooth disease (CMT2), and these mechanisms might present a common link between dissimilar CMT2-causing genes.

[Photobiomodulation in the prevention and the management of side effects of cancer treatments: Bases, results and perspectives]. / Photobiomodulation dans la prévention et la prise en charge des effets secondaires des traitements anticancéreux : bases, bilan et perspectives.

BACKGROUND: Assess the current and potential indications of photobiomodulation (PBM) therapy and their level of evidence in the prevention or treatment of side effects related to oncology treatments (radiation therapy, and to a minimal extent favored and hematopoietic stem cell transplants). And report on the recommended modalities (parameters and doses) of PBM therapy. MATERIALS AND METHODS: The Embase, Medline/PubMed, Cochrane, EBSCO, Scopus, and LILACS databases were systematically reviewed to include and analyze publications of clinical studies that evaluated PBM in the prevention or management side effects related to cancer treatments. The keywords used were "photobiomodulation"; "low level laser therapy"; "acute oral mucositis"; "acute dysphagia"; "acute radiation dermatitis"; "lymphedema"; "xerostomia"; "dysgeusia"; "hyposalivation"; "lockjaw"; "bone necrosis"; "osteoradionecrosis"; "radiation induced fibrosis"; "voice and speech alterations"; "palmar-plantar erythrodysesthesia"; "graft versus host disease"; "peripheral neuropathy"; "chemotherapy induced alopecia". Prospective studies were included, while retrospective cohorts and non-original articles were excluded from the analysis. RESULTS: PBM in the red or infrared spectrum has been shown to be effective in randomized controlled trials in the prevention and management of certain complications related to radiotherapy, in particular acute mucositis, epitheliitis and upper limb lymphedema. The level of evidence associated with PBM was heterogeneous, but overall remained moderate. The main limitations were the diversity and the lack of precision of the treatment protocols which could compromise the efficiency and the reproducibility of the results of the PBM. For other effects related to chemo/radiation therapy (dysgeusia, osteonecrosis, peripheral neuropathy, alopecia, palmar-plantar erythrodysaesthesia) and haematopoietic stem cell transplantation (graft versus host disease), treatment with PBM suffers from a lack of studies or limited studies at the origin of a weakened level of proof. However, based on these results, it was possible to establish safe practice parameters and doses of PBM. CONCLUSION: Published data suggest that PBM could therefore be considered as supportive care in its own right for patients treated with radiation, chemotherapy, immunotherapy, hormone therapy or targeted therapies, whether in clinical practice or clinical trials. therapies. However, until solid data have been published on its long-term safety, the use of PBM should be considered with caution and within the recommended parameters and doses, particularly when practiced in areas of known or possible tumours. In this case, the patient should be informed of the theoretical benefits and risks of PBM in order to obtain informed consent before treatment.

[2012 literature review on peripheral neuropathies: immune neuropathies (treatments excluded)]. / Revue de la littérature 2012 sur les neuropathies périphériques : neuropathies dysimmunitaires (à l'exclusion du traitement).

In 2012, interest remains high in the field of dysimmune neuropathies, chiefly concerning Guillain-Barré syndrome (GBS). The pathophysiological mechanisms are now better known but electrophysiological criteria should be updated. The risk of GBS in H1N1 vaccination is now well evaluated. Nerve ultrasonography provides new prospects for diagnosis and follow-up of dysimmune neuropathies but cannot substitute for electrophysiology. This paper aims to present some noteworthy articles published in 2012 in the field of dysimmune neuropathies.

Neuropathies périphériques associées aux syndromes lymphoprolifératifs : spectre clinique et démarche diagnostique.

Lymphoproliferative syndromes (multiple myeloma, Waldenström's disease, chronic lymphocytic leukemia, lymphomas) may be associated with peripheral neuropathies. The mechanism can be dysimmune, associated or not with monoclonal gammopathies; paraneoplastic; infiltrative; or more commonly, iatrogenic or due to vitamin deficiency. The diagnosis can be complex, especially when the neuropathy is the presenting manifestation, requiring a close cooperation between internists and neurologists. The positive diagnosis of the neuropathy is based on a systematic electro-clinical investigation, which specifies the topography and the mechanism of the nerve damage, sometimes reinforced by imaging examinations, in particular, nerve and/or plexus MRI. The imputability of the neuropathy to a lymphoproliferative syndrome is based on a set of arguments including the clinical context (B signs, tumour syndrome), first-line laboratory tests (hemogram, protein electrophoresis, viral serologies, complement), auto-antibodies discussed according to the neuropathy (anti-MAG, anti-gangliosides) and sometimes more invasive examinations (bone marrow or neuro-muscular biopsies).

[POEMS syndrome: Diagnosis, stratification, treatments]. / Syndrome POEMS : diagnostic, prise en charge et traitements.

POEMS syndrome is a rare form of B-cell dyscrasia with multiple clinical signs including the acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It is a paraneoplastic syndrome due to an underlying plasma cell disorder belonging to the monoclonal gammopathies of clinical significance (MGCS). The major criteria for this syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), and the presence of Castleman's disease. Minor features include organomegaly, endocrinopathy, skin changes, papilledema, extravascular volume over-load, and thrombocytosis. The diagnosis of POEMS syndrome requires three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. VEGF plays a major role in the disease although anti-VEGF treatments have been disappointing. Risk stratification is based on clinical phenotype rather than specific molecular markers. Depending on bone marrow involvement and the number of sclerotic bone lesions, first line therapy should be irradiation or systemic therapy. For patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing irradiation therapy should receive antiplasma cell systemic therapy, the most effective being high dose chemotherapy with autologous stem cell transplantation. Lenalidomide seems to have a high efficacy with manageable toxicity. Thalidomide and proteasome inhibitors like bortezomib are also effective, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy.

[Peripheral neuropathies: Diagnostic strategy]. / Neuropathies périphériques : démarche diagnostique.

Diagnosing a peripheral neuropathy is sometimes challenging, as the causes are diverse and the clinical pictures heterogeneous. Overall, diagnosing a patient with peripheral neuropathy will require some knowledge in almost every field of medicine. Therefore, it appears crucial to adopt a diagnostic strategy that is based on solid clinical and neurophysiological grounds. The present paper describes a three-step diagnostic strategy: (1) to delineate a clinico-pathologic entity from clinical and electrodiagnostic findings; (2) to propose a list of plausible causes based on step one, history and clinical context; (3) to use appropriate workup in order to determine the cause or mechanism of the neuropathy. The three steps of this diagnostic strategy necessitate a high level of expertise and interaction between physicians is highly desirable. Finally, an aggressive course and a severe impairment should lead to relentlessly look for a curable cause.

Somatosensory evoked potentials in the assessment of peripheral neuropathies: Commented results of a survey among French-speaking practitioners and recommendations for practice.

BACKGROUND: Somatosensory evoked potentials (SSEPs) are increasingly performed for the assessment of peripheral neuropathies, but no practical guidelines have yet been established in this specific application. STUDY AIM: To determine the relevant indication criteria and optimal technical parameters for SSEP recording in peripheral neuropathy investigation. METHODS: A survey was conducted among the French-speaking practitioners with experience of SSEP recording in the context of peripheral neuropathies. The results of the survey were analyzed and discussed to provide recommendations for practice. RESULTS: SSEPs appear to be a second-line test when electroneuromyographic investigation is not sufficiently conclusive, providing complementary and valuable information on central and proximal peripheral conduction in the somatosensory pathways. CONCLUSIONS: Guidelines for a standardized recording protocol, including the various parameters to be measured, are proposed. CLINICAL RELEVANCE: We hope that these proposals will help to recognize the value of this technique in peripheral neuropathy assessment in clinical practice.