文摘
Over the last five years, the management of peripheral neuropathies has become structured by the publication of recognized diagnostic criteria for inflammatory neuropathies and the elaboration of a function score, the R-ODS, used to evaluate the progression of these neuropathies. The concept of nodo-paranodopathy has enriched the concept of peripheral neuropathies, over-riding the classical mechanisms of axonal and demyelinating mechanisms. The structures of the nodes of Ranvier, gangliosides, contractin and neurofascin are preferential targets for auto-antibodies responsible for dysimmune neuropathies. Concerning treatments, immunosuppressors have demonstrated their efficacy for the treatment of anti-MAG neuropathies. Corticosteroid treatments are also in the limelight, demonstrating a different response profile than intravenous immunoglobulins for CIDP. But the most remarkable therapeutic progress has been made in the domain of hereditary neuropathies. The first trial versus placebo produced positive results in CMT1a. Finally, the era of genetic therapy appears to have come to fruition with the interfering RNA trial for familial amyloid neuropathies.
