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

•Some patients with progressive neuropathy are refractory to conventional therapies.•Lacking of identifiable antibodies makes diagnosis and treatment challenging.•Alternative options are therapeutic plasma exchange and B-cell suppressive therapy. Immune-mediated peripheral neuropathies are heterogen...

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Bibliographic Details
Published in:	Brain disorders Vol. 14; p. 100148
Main Authors:	Hung, Stefanie Kar Yan, Yeap, Daniel Tze Wei, Karunakaran, Thanusha, Krishnan, Dhayalen, Tee, Sow Kuan, Hiew, Fu Liong
Format:	Journal Article
Language:	English
Published:	Elsevier B.V 01-06-2024 Elsevier
Subjects:	Autoimmune nodopathies B-cell suppression Chronic inflammatory demyelinating polyneuropathy Seronegative immune-mediated neuropathy Therapeutic plasma exchange ACD ONLS MRI Autoimmune nodopathies B-cell suppression IVIg CIDP Seronegative immune-mediated neuropathy CSF and RODS Therapeutic plasma exchange Chronic inflammatory demyelinating polyneuropathy EMG MRC NCS
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Summary:	•Some patients with progressive neuropathy are refractory to conventional therapies.•Lacking of identifiable antibodies makes diagnosis and treatment challenging.•Alternative options are therapeutic plasma exchange and B-cell suppressive therapy. 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.
ISSN:	2666-4593 2666-4593
DOI:	10.1016/j.dscb.2024.100148