Since 2013, a number of new antibodies to nodal and paranodal antigens have been identified in a small proportion of patients with chronic inflammatory demyelinating polyneuropathy (CIDP).
Anti neurofascin 155 antibodies have been found in patients sharing a phenotype consisting of distal weakness, tremor and ataxia as well as immunoglobulin resistance.
Anti contactin 1 antibodies have been reported with variable phenotypes and poor immunoglobulin response.
Anti CASPR1 antibodies have been reported in patients with acute onset, monophasic course and pain.
These new forms of CIDP are generally resistant to conventional therapies and maty benefit from rituximab. Consideration of testing is helpful in case of refractoriness to first-line agents.
Key points:
- New paranodal antibodies have been discovered in the last few years in a minority of cases of CIDP
- Clinical features may be of distal predominance and ataxia although more data is needed for firm conclusions
- Refractoriness to conventional therapies may raise the need for testing for these antibodies in view of the therapeutic implications.
Refereces:
Querol at al. Nat Rev Neurol 2017.