Nodo-Paranodopathy: Beyond the Demyelinating and Axonal Antibody-Mediated Neuropathies
Partha S Ray*
Indian Medical Association (IMA), National Professor in Neurology, India
*Corresponding Author: Prof. Dr. Partha Ray, Indian Medical Association (IMA), National Professor in Neurology, India.
Received: February 14, 2022 Published: March 24, 2022
Abstract
In the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo paranodopathies’. These patients present with clinical features not usually seen with the most common immune mediated neuropathies, GuillainBarre syndrome and chronic inflammatory demyelinating polyneuropathy. They respond poorly to conventionally used immunomodulatory therapies. Emerging evidence that these are pathologically distinct diseases has further prompted the use of more targeted treatment, such as the B cell depleting monoclonal antibody rituximab, which has been reported to significantly improve functional outcomes in this subset of patients. It is now apparent that antibodies directed against several region-specific cell adhesion molecules, including neurofascin, contactin and contactin-associated protein, can be linked to phenotypically distinct peripheral neuropathies. Importantly, the immunological characteristics of these antibodies facilitate the prediction of treatment. Electro- clinico- pathological diagnostic criteria of these acquired immune mediated neuropathies thus need to be reclassified in the light of this disease entity of ‘nodo-paranodopathies’. In this review the readership is directed to explore the presence of these specific antibodies that cause this “nodoparanodopathy” immune phenotype that has a different therapy paradigm and prognosis compared to the conventional acute and chronic neuropathy syndromes that we know of so very well.
Keywords: Guillain-barre syndrome, paranode node, CIDP; Node of Ranvier, Neurofascin, Contactin contactin-associated protein 1
Citation: Ray PS. “Nodo-Paranodopathy: Beyond the Demyelinating and Axonal Antibody-Mediated Neuropathies”. SVOA Neurology 3:2 (2022) Pages 72-79.
