Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) that is refractory to standard treatment.MethodWestern BlotResultReported as negative or positive.InterpretationContactin-1 (CNTN1) is a paranodal protein that is essential for the function of the node of Ranvier. IgG autoantibodies against CNTN1 can be detected in about 5% of patients with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). This subgroup is characterized by higher age at onset and a subacute aggressive GBS-like course with axonal damage, motor weakness and poor response to treatment with intravenous immunoglobulin (IVIg). Some of these patients respond to treatment with steroids or plasmapheresis. It has been reported that treatment resistant cases have been responsive to treatment with rituximab (Querol L et al., 2015).
These autoantibodies are dominated by IgG4 subclass (Vural A et al., 2018).ReferencesQuerol L et al., Neurol. Neuroimmunol. Neuroinflamm. 2015 (PMID 26401517)
Delmont E et al., Brain 2017: 140; 1851–1858
Vural A et al., Front. Immunol., 14 May 2018