Abstract
AbstractAutoimmune nodopathy (AN) are demyelinating pathologies of the peripheral nervous system which have the particularity of having a pathophysiology mediated by antibodies mainly of IgG4 isotype. Specific autoantibodies, the acute or subacute onset of neuropathy, the poor response to intravenous immunoglobulins and the improvement after rituximab led to exclude AN from the group of chronic inflammatory demyelinating polyradiculoneuropathies. Currently, 4 auto-antibodies targeting axo-glial proteins at the nodes of Ranvier and paranodes have been identified: anti-pan-neurofascin (anti-pan-Nfasc) antibodies which recognize all neurofascin isoforms, anti-neurofascin 155 (anti-Nfasc155), anti-contactin 1 (anti-CNTNI) and anti-contactin associated protein 1 (anti-CASPRI). Currently, in France, the identification of these antibodies is processed by flow cytometry on transfected HEK cells in the Immunology laboratory at the hospital La Conception in Marseille. As AN can cause severe disability and sometimes be life-threatening, their diagnosis should be done as soon as possible in order to start promptly the treatment.
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