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.     

Lymphomes T périphériques : diagnostic et prise en charge

Peripheral T cell lymphomas are rare malignancies with aggressive course, with several different subtype described in the 2016 WHO classification. Their distribution across the world is heterogenous, with marked difference between Western and Asian country. Their clinical presentation often comprise extra-nodal involvement, B symptoms and immune system disorder which can lead to wrong diagnosis orientation. Make a right diagnosis need a experienced pathologist in close collaboration with clinical datas. Peripheral T cell lymphomas are in general associated with poor prognosis when treated with anthracyclines-based regimen, and several studies and trials focused on the use of intensified regimen or novel targeted agents, whose proper indication still remain to be clarified.     

Dysfonctionnement nasal chronique et obstruction des voies aériennes périphériques

Allergic rhinitis often occur at the same time with many upper and lower airway diseases in which there is asthma. Many patients without clinical asthma may have an asymptomatic bronchial hyperreactivity and an allergic rhinitis at the same time. An overview of these actual knowledges is done including clinical, respiratory functional explorations, therapeutic studies, as well as those observed in chronic nasal dysfunctions (allergic or non allergic eosinophilic rhinitis, nasal polyposis, chronic rhinitis).Objectives. – The aim of this study is to determine bronchial hyperresponsiveness of a chronic nasal dysfunction population having allergic or non allergic rhinitis.Material and methods. – Retrospective study of respiratory functional explorations (using a methacholine provocation test) among non asthmatic patients consulting for rhinology-allergy diseases but with broncho-pulmonary symptomatology.Results. – It has been observed an allergic rhinitis population without upper bronchial hyperreactivity but with a small airway obstruction revealed or worsened during the methacholine bronchoprovocation test.Conclusion. – Chronic nasal dysfunction and small airway obstruction are correlated. This association shows an airway inflammation from the nose through the small bronchi. As upper bronchial hyperreactivity can precede asthma, a small airway obstruction could precede a bronchial hyperreactivity. Nasal chronic dysfunction treatment could act upon small airway obstruction, or central bronchial hyperreactivity or asthma appearance.     

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).     

La paralysie faciale périphérique a frigore

Idiopathic peripheral facial palsy, also named Bell's palsy, is the most common cause of peripheral facial palsy in adults. Although it is considered as a benign condition, its social and psychological impact can be dramatic, especially in the case of incomplete recovery. The main pathophysiological hypothesis is the reactivation of HSV 1 virus in the geniculate ganglia, leading to nerve edema and its compression through the petrosal bone. Patients experience an acute (less than 24 hours) motor deficit involving ipsilateral muscles of the upper and lower face and reaching its peak within the first three days. Frequently, symptoms are preceded or accompanied by retro-auricular pain and/or ipsilateral face numbness. Diagnosis is usually clinical but one should look for negative signs to eliminate central facial palsy or peripheral facial palsy secondary to infectious, neoplastic or autoimmune diseases. About 75% of the patients will experience spontaneous full recovery, this rate can be improved with oral corticotherapy when introduced within the first 72 hours. To date, no benefit has been demonstrated by adding an antiviral treatment. Hemifacial spasms (involuntary muscles contractions of the hemiface) or syncinesia (involuntary muscles contractions elicited by voluntary ones, due to aberrant reinnervation) may complicate the disease's course. Electroneuromyography can be useful at different stages: it can first reveal the early conduction bloc, then estimate the axonal loss, then bring evidence of the reinnervation process and, lastly, help for the diagnosis of complications.     

Neuropathies périphériques au cours des maladies de système : partie II (vascularites)

Primary systemic vasculitides, mainly of the small and medium-sized vessels, are frequently associated with peripheral neuropathies. When the disease is already known, the appearance of a neuropathy should suggest a specific injury, especially when associated with other systemic manifestations. Conversely, when neuropathy is inaugural, close collaboration between neurologists and internists is necessary to reach a diagnosis. A standardized electro-clinical investigation specifying the topography, the evolution and the mechanism of the nerve damage enables the positive diagnosis of the neuropathy. Several elements orient the etiological diagnosis and allow to eliminate the main differential diagnosis: non systemic vasculitic neuropathy. The existence of associated systemic manifestations (glomerular or vascular nephropathy, interstitial lung disease, intra-alveolar hemorrhage, ENT involvement…), biological markers (ANCA, cryoglobulinemia, rheumatoid factor), and invasive examinations allowing histological analysis (neuromuscular biopsy) are all useful tools for.     

Neuropathies périphériques au cours des maladies de système : partie I (connectivites et granulomatoses)

Systemic diseases (connective disease, granulomatosis) may be associated with peripheral neuropathies. The diagnosis can be complex when the neuropathy is the presenting manifestation of the disease, requiring close collaboration between neurologists and internists. Conversely, when the systemic disease is already known, the main question remaining is its imputability in the neuropathy. Regardless of the situation, the positive diagnosis of neuropathy is based on a systematic and rigorous electro-clinical investigation, specifying the topography, the evolution and the mechanism of the nerve damage. Certain imaging examinations, such as nerve and/or plexus MRI, or other more invasive examinations (skin biopsy, neuromuscular biopsy) enable to specify the topography and the mechanism of the injury. The imputability of the neuropathy in the course of a known systemic disease is based mainly on its electro-clinical pattern, on which the alternatives diagnoses depend. In the case of an inaugural neuropathy, a set of arguments orients the diagnosis, including the underlying terrain (young subject), possible associated systemic manifestations (inflammatory arthralgias, polyadenopathy), results of first-line laboratory tests (lymphopenia, hyper-gammaglobulinemia, hypocomplementemia), autoantibodies (antinuclear, anti-native DNA, anti-SSA/B) and sometimes invasive examinations (neuromuscular biopsy).     

Hyperplasie hypophysaire secondaire à une hypothyroïdie périphérique

Introduction

Pituitary hyperplasia due to primary hypothyroidism is rare and underdiagnosed.

Thrombolyse des artériopathies des membres

Thrombolytic therapy has been employed for 40 years, by systemic infusion, in the treatment of acute artery occlusions, and since 1971 by local infusion. Several randomised studies published during the 1990's have compared thrombolysis to surgery; they showed the benefits of the thrombolytic therapy, together with, however, the risks and potential complications. The use of thrombolysis combined with endovascular treatment of arterial lesions by recanalisation, thrombo-aspiration, angioplasty with or without endoprosthesis, has allowed defining new therapeutic strategies. The aim of the present chapter is to update one of our previous works by the identification of those new treatment modalities that have been used during the last decade, the presentation of the mechanism of action of standard thrombolytic drugs, streptokinase (SK) and urokinase (UK) utilised by systemic infusion, local fusion, and during per-operative procedures, and finally modern thrombolytic treatments from the plasminogen activator (tPA) to the staphylokinase. A European consensus has allowed to precise indications, contraindications and complications of such treatment (TASC). Recommendation # 59 concludes that there is no more indication for the systemic treatment of acute arterial occlusions with currently available thrombolytic drugs. The contraindications published in 1998 are actually widely known. Current indication remains local thrombolysis. The procedure duration, in addition to the associate risk of complications, is not always compatible with the emergency pattern of the revascularisation necessitated by some acute ischemias. Intra operative treatment is useful in by-pass occlusions. Despite insufficient published data, the combination of surgery and thrombolysis allows reducing both the dose and the duration of the procedure, which can be very important in severe cases of acute ischemia.     

Carrefour des spécialités : autogreffe de cellules souches hématopoïétiques périphériques pour la prise en charge thérapeutique des polyradiculonévrites inflammatoires démyélinisantes chroniques

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a dysimmune neuropathy with sensory and/or motor symptoms due to destruction of the myelin sheat secondary to an auto-immune attack. A quarter to a third of patients do not respond to immunomodulatory first line recommended therapies. No second line treatment has shown its effectiveness with a sufficient level of evidence. Autologous hematopoietic stem cell transplantation (AHSCT) is a promising therapy for autoimmune disease, especially for CIDP in recent works. We present in this article an update on the diagnosis of CIDP, its conventional treatments as well as the results of AHSCT in this indication, which was the subject of French recommendations under the aegis of the SFGMTC and neuromuscular disease french faculty (FILNEMUS) as a third line therapy after failure of two first-line and one second-line treatments.