title: [Review of the recent literature on peripheral neuropathies: therapeutic advances].
abstract: Improvement of therapeutic strategies for peripheral neuropathies requires multicentric clinical trials. For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a randomized controlled multicentric study compared IgIV to pulses of methylprednisolone (MP) given for 6 months. The primary endpoint was treatment discontinuation due to inefficacy or intolerance; 45 patients were enrolled: more patients had interrupted MP than IVIg, usually because of inefficacy. A multicentric randomized clinical trial (PREDICT) evaluated long-term remission of CIDP after short-term corticosteroid therapy (pulses of dexamethasone or prednisolone); 39 patients were enrolled: 26% achieved cure or remission, a relapse occurred in 50% after a delay of 11 to 17 months. Differential diagnosis was identified in 58% of patients who had not responded to any therapy. For refractory CIDP, a retrospective study showed the possibility of functional improvement in 24% of cases after adjunction of an immunomodulatory agent; cyclosporine was associated with the highest rate of adverse events or side effects. In familial amyloidotic polyneuropathy, a multicentric controlled study against placebo with tafamidis, an akinetic stabilizer of transthyretin (TTR) 20mg/d, in early stage of Val30MetTTR showed efficiency in the evaluable group and led to marketing authorization by the EMA in stage 1 to slow the progression of the neuropathy. A Cochrane database system review showed that there are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome, for neuropathies with anti-MAG antibodies, or multifocal motor neuropathy on which to base practice. This review underlines the usefulness of multicentric randomized trials to assess treatments in peripheral neuropathies.
authors.affiliation: Inserm U 788, service de neurologie, centre de référence neuropathies amyloïdes familiales et autres neuropathies périphériques rares (NNERF), université Paris Sud, CHU Bicêtre, AP-HP, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre cedex, France. Electronic address: david.adams@bct.aphp.fr.
authors.forename: D
authors.initials: D
authors.lastname: Adams
mesh_terms: D006801:Humans; D007167:Immunotherapy; D016878:POEMS Syndrome / Q000188:drug therapy; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000628:therapy*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000188:drug therapy
keywords: Corticosteroids; Dexaméthasone; Familial amyloid polyneuropathy; IgIV; Immunomodulateurs; Immunomodulators; Neuropathies amyloïdes héréditaires; PIDC; POEMS syndrome; Refractory; Syndrome de POEMS; Tafamidis