Chapter

Pathogenesis

Robert B. Darnell and Jerome B. Posner

in Paraneoplastic Syndromes

Published on behalf of Oxford University Press

Published in print April 2010 | ISBN: 9780199772735
Published online April 2013 | e-ISBN: 9780199322916 | DOI: http://dx.doi.org/10.1093/med/9780199772735.003.003

Series: Contemporary Neurology Series

Pathogenesis

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Despite years of effort, we do not fully understand the pathogenesis of most paraneoplastic syndromes, especially those affecting the central nervous system (CNS). In large part, this relates to the fact that investigators have been unable to generate animal models that recapitulate these disorders. Exceptions are myasthenia gravis, the Lambert-Eaton myasthenic syndrome (LEMS), and an autonomic neuropathy associated with neuronal acetylcholine receptor antibodies (see Chapter 8). Animal (and clinical) findings indicate that these disorders are largely mediated by antibodies, with the help of T-cells and cytokines.1,2 Other disorders that are probably, at least in part, also antibody mediated include stiff person syndrome (Chapter 6), limbic encephalopathy (LE) associated with anti-NMDA receptor and some other antibodies reactive with antigens found on the surface of neurons or their processes (see Chapter 13), and carcinoma-associated retinopathy (Chapter 7). In these disorders, the paraneoplastic antibodies target readily accessible cell-surface receptors The pathogenesis of the many neurologic paraneoplastic syndromes whose antigens are found inside the cell is still unknown. Over the years, investigators have proposed several potential pathogenetic mechanisms (Table 3–1).

Chapter.  11846 words.  Illustrated.

Subjects: Neurology

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