Paraneoplastic Optic Neuropathy Associated With Purkinje Cell Antibody-2 in a Patient With Small Cell Lung Cancer

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Title Paraneoplastic Optic Neuropathy Associated With Purkinje Cell Antibody-2 in a Patient With Small Cell Lung Cancer
Creator Jonathan A. Micieli, MD, CM, Edward A. Margolin, MD
Affiliation Department of Ophthalmology and Vision Science, University of Toronto, Toronto, Ontario, Canada
Subject Female; Humans; Lung Neoplasms; Middle Aged; Ophthalmoscopy; Optic Nerve Diseases; Paraneoplastic Syndromes, Ocular; Small Cell Lung Carcinoma
Abstract Although still rarely diagnosed, amyloid light chain (AL) amyloidosis is the most common form of systemic amyloidosis. It is characterized by misfolded monoclonal immunoglobulin light chain fragments that accumulate extracellularly as amyloid fibrils, with consequent organ dysfunction. We report 2 such cases where initial symptoms and signs were identical to and mistaken for those of giant cell arteritis, associated with polymyalgia rheumatica. Neither patient responded to high-dose corticosteroids; instead, their temporal artery biopsies revealed amyloid deposits and other investigations confirmed a diagnosis of systemic AL amyloidosis. Review of the literature reveals similar cases of diagnostic confusion spanning 75 years. We have summarized the findings and learning points from cases reported in the past 30 years and highlight the need for increased awareness and investigation of this underrecognized syndrome.
OCR Text Show
Publisher Lippincott, Williams & Wilkins
Date 2017-03
Type Text
Language eng
Rights Management © North American Neuro-Ophthalmology Society
Publication Type Journal Article
ARK ark:/87278/s6xt01b2
Setname ehsl_novel_jno
Date Created 2018-07-25
Date Modified 2020-03-18
ID 1353379
Reference URL https://collections.lib.utah.edu/ark:/87278/s6xt01b2
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