Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic autoimmune neuropathy. It encompasses a range of diseases that present with sensorimotor disturbances and electrophysiological demyelination.
Autoantibodies against the Ranvier node can be detected in about 10% of patients fulfilling the CIDP criteria. These antibodies typically target nodal-paranodal cell-adhesion molecules Contactin-1 (CNTN1), Neurofascin 155 (NF155), 186 (NF186), 140 (NF140), or Contactin-associated protein 1 (Caspr1). Patients carrying these autoantibodies are classified as having autoimmune nodopathy – and often exhibit more aggressive symptoms that may require an altered therapeutic approach.
Patients with antibodies against NF155 often have an earlier age of onset with a fast progression involving ataxia and tremors.
These patients often have a rapid clinical progression, sometimes combined with other systematic autoimmune diseases.
Caspr1-positive patients often have ataxia and are often initially diagnosed with GBS. Around half of the patients are in severe pain. They typically have poor response to immunoglobulin treatment but good response to rituximab.
The combination of these antibodies has been seen in cases of acute neuropathy – with symptoms similar to Guillain-Barre Syndrome (GBS). Although there may be an initial improvement in symptoms, this type of pan-neurofascin neuropathy can progress rapidly.
Wieslab Diagnostic Services can help in the diagnosis of autoimmune nodopathy. We offer a panel as well as individual tests for:
Our normal turn-around time is one week, but we also offer results within 48 hours.
See our panels