Rapidly progressive seronegative immune-mediated neuropathies responded to “Remove and suppress” therapy with plasma exchange and B-cell suppression therapy

Abstract

Immune-mediated peripheral neuropathies are heterogeneous group of disorders due to the present of autoantibodies against peripheral nerve molecules located in node of Ranvier such as gangliosides and cell adhesion proteins or myelin components of peripheral nerves. Although the exact aetiology and pathophysiological mechanisms involved are not fully understood, both humoral and cellular immunity are likely playing a role in their pathogenesis. A proportion of patients present with clinical phenotype of rapidly progressive neuropathy refractory to conventional therapies but are lacking in identifiable or detectable antibodies. This makes diagnosis and treatment decision challenging.

We illustrate a patient with rapidly progressive seronegative immune-mediated neuropathy resembling autoimmune nodopathy (AN) associated with atypical features (prominent ataxia) and cranial involvement (facial and oropharyngeal weakness, dysgeusia), refractory to conventional therapies (IV immunoglobulin and corticosteroids) but responded to ‘remove and suppress’ treatment regime using therapeutic plasma exchange (TPE), followed by long-term B-cell suppressive therapy.