Pearls & Oy-sters: Breaking Bad CIDP

Autoimmune nodopathy is a rare, debilitating, demyelinating polyneuropathy triggered by autoantibodies targeting peripheral nerve nodal and paranodal proteins including neurofascin-155 (NF155), contactin-1 (CNTN1), contactin-associated protein-1 (CASPR1), and neurofascin-186/140 (NF186/NF140).c Early testing for nodal and paranodal autoantibodies is crucial in refractory chronic inflammatory demyelinating polyneuropathy (CIDP) because autoimmune nodopathy does not respond robustly to first-line immunomodulatory treatments but improves with B-cell depletion. Oy-sters cClinical features of a demyelinating neuropathy that should raise suspicion for an underlying autoimmune nodopathy include tremors, severe sensory ataxia, cranial nerve involvement, and pain.c Severely elevated CSF protein or nephrotic syndrome in CIDP warrants antibody testing for an underlying autoimmune nodopathy.