Monoclonal gammopathy of undeterminated significance and endoneurial IgG deposition

Content Provider	Semantic Scholar
Author	Mathis, Stéphane Franques, J. Thomas Richard, Laurence Vallat, J. -M.
Copyright Year	2016
Abstract	BACKGROUND Monoclonal gammopathy of undeterminated significance is the most common form of plasma cell dyscrasia, usually considered as benign. In rare cases it may have a malignant course, sometimes limited to an organ such as peripheral nerves. METHODS We describe clinical, electrophysiological and pathological findings in a patient presenting a immunoglobulin G (IgG) paraproteinemic polyneuropathy clinically mimicking a chronic inflammatory demyelinating polyneuropathy. RESULTS Immuno-electron microscopy (immune-EM) demonstrated that the widenings of the myelin lamellae resulted from the infiltration of IgG between a significant number of myelin lamellae (with absence of inflammatory cells in the epineurium, endoneurium, and perineurium, and the lack signs of vasculitis). This patient was finally treated successfully with lenalidomide then mycophenolate mofetil. CONCLUSIONS In polyneuropathies associated to a monoclonal gammopathy, a nerve biopsy may clinch the diagnosis. Immuno-EM may be required to determine the role of the pathological immunoglobulin in the destruction of the peripheral nerve parenchyma. Diagnosis of such a direct involvement of peripheral nerve can endorse more aggressive treatment of real efficiency.
Starting Page	102
Ending Page	110
Page Count	9
File Format	PDF HTM / HTML
PubMed reference number	27603395
Alternate Webpage(s)	https://doi.org/10.1097/MD.0000000000004807
DOI	10.1097/md.0000000000004807
Journal	Medicine
Volume Number	95
Language	English
Access Restriction	Open
Subject Keyword	Abducens Nerve Diseases Daclizumab Demyelinating peripheral neuropathy Electron Microscopy Monoclonal Gammapathies Monoclonal Gammopathy of Undetermined Significance Myelin Associated Glycoprotein Parenchyma Patients Perineurium Peripheral Nerves Plasma Cell Neoplasm Polyneuropathy Polyradiculoneuropathy, Chronic Inflammatory Demyelinating Vasculitis abnormal cellular structure or growth lenalidomide mycophenolate mofetil trastuzumab
Content Type	Text
Resource Type	Article