Abstract
Conventional treatment options, including corticosteroids, intravenous immunoglobulin, or plasma exchange, often fail to treat dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and monoclonal gammopathy with its subtypes. Therefore, a significant percentage of patients require adjunctive immunosuppressive therapies. Considering that even immunosuppressive agents often are ineffective and/or associated with significant toxicities, the need for the development of safe and effective new treatment options is rising. Currently, several monoclonal antibodies (MAbs) have been tested in open-label small-sized studies or even in single cases so as to establish future directions in the therapy of diseases of the peripheral nervous system (PNS). Rituximab, an MAb targeting against the B cell surface membrane protein CD20, is the most widely used and promising MAb for the treatment of dysimmune neuropathies, especially for those in which immunoglobulin M (IgM) autoantibodies are pathogenetically involved. The efficacy of alemtuzumab, bevacizumab, and etanercept to treat various forms of dysimmune neuropathies is currently under investigation. This review looks critically at recent developments in molecularly targeted therapies for dysimmune neuropathies and also highlights areas of future research to pursue.
Highlights
The armamentarium of conventional treatment options for diseases of the peripheral nervous system (PNS), especially for dysimmune neuropathies, include the administration of corticosteroids, plasmapheresis, long term intermittent intravenous immunoglobulin (IVIg) infusion, and immunosuppressive agents
Search Strategy and Selection Criteria References for this review were identified by searches of PubMed from 2000 until December 2008 with the terms “dysimmune neuropathy,” “treatment of dysimmune neuropathy,” “monoclonal antibodies for diseases of the peripheral nervous system,” “monoclonal antibodies and dysimmune neuropathy,” “molecularly-targeted treatment for dysimmune neuropathy,” “rituximab for Address correspondence and reprint requests to Andreas A Argyriou, Department of Neurology, Saint Andrew’s State General Hospital of Patras, 26335, Patras, Greece
This study revealed a lack of rituximab efficacy for Chronic Idiopathic Demyelinating Polyradiculopathy (CIDP) patients, since the primary endpoint was not reached
Summary
The armamentarium of conventional treatment options for diseases of the peripheral nervous system (PNS), especially for dysimmune neuropathies, include the administration of corticosteroids, plasmapheresis, long term intermittent intravenous immunoglobulin (IVIg) infusion, and immunosuppressive agents. The efficacy of these treatment approaches is usually short lasting or associated with adverse events, mainly because of the clinical heterogeneity and the huge variability of treatment responses [1]. Recent advances in the thorough understanding of the complex immunological pathogenesis of dysimmune neuropathies or nerve root syndromes have led to the arousal of rationale applications of new molecularly targeted treatment options, especially for disorders that are resistant to conventional treatment options. Recent developments in molecularly targeted therapies for dysimmune neuropathies are evaluated critically. To the best of my knowledge, this is the first review article in the topic
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