We review the concept fo Monoclonal Gammopathy emphasizing that the so called benign forms, with or without polyneuropathy can have an unpredictable evolution. Polyneuropathies associated to monoclonal gammopathy-IgM, IgG, IgA, Waldestrom's disease, Multiple myeloma, Osteosclerotic myeloma (POEMS), Amyloidosis and Cryoglobulinemias are reviewed. They generally are solely sensory, sensorimotor or rarely, solely motor. The importance of neuro-physiological studies and nerve biopsy is emphasized. For progressive forms, treatment with plasmapheresis, immunotherapy, corticoids or intravenous gammaglobulin is suggested.
Download full-text PDF |
Source |
---|
Rev Neurol (Paris)
October 2014
Neurology Department, University hospital, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France. Electronic address:
In most dysimmune neuropathies, historically the microscopical lesions were described prior to immunological studies. The latter along with neuropathological studies have found some immune, albeit incomplete, explanations of the mechanisms of these lesions which we will describe in two main syndromes: the primitive auto-immune inflammatory peripheral polyneuropathies (GBS and CIDP) and polyneuropathies induced by a monoclonal dysglobulinemia. In some patients who have to be discussed case by case pathology (nerve biopsy) will confirm the diagnosis, may help to delineate the molecular anomalies and identify lesional mechanisms.
View Article and Find Full Text PDFBrain Nerve
February 2011
Kitakyushu Yahata-Higashi Hospital, Fukuoka, Japan.
In 1971, the author reported an autopsy case of a 48-year-old Japanese man with polyneuropathy, skin hyperpigmentation, diabetes mellitus, and monoclonal gammopathy. Previously, a total of 2 cases of solitary myeloma accompanied by polyneuropathy and endocrinological disorders have been reported by Fukase et al in 1968 and by Shimomori and Kusumoto in 1970 in Japan. The author's case is the first reported non-myeloma case where polyneuropathy associated with dermatoendocrionological changes and dysglobulinemia was observed.
View Article and Find Full Text PDFJ Neuropathol Exp Neurol
August 2009
Centre de Référence National Neuropathies Périphériques Rares, Neurology Department, University Hospital, Limoges Cedex, France.
Indications for nerve biopsy have decreased during the last 20 years. For the most part, this is a result of progress in the application of molecular biologic diagnostic testing for genetic peripheral neuropathies (PNs) and the increasing use of skin biopsy. The latter is primarily used to evaluate small-fiber PN, although it rarely discloses the specific etiology of a PN.
View Article and Find Full Text PDFMuscle Nerve
July 2008
Department of Neurology and Centre National de Référence Neuropathies Périphériques Rares, University Hospital, 2 Avenue Martin Luthert King, Limoges Cedex, France.
There are several pathogenic mechanisms of peripheral nerve involvement in patients with monoclonal dysglobulinemia. Intranervous proliferation of malignant cells, immunoglobulin, or amyloid deposits in the endoneurial space can only be determined by examination of nerve biopsy specimens. We present clinical, electrophysiological, and histological data from seven patients whose polyneuropathy was induced by immunoglobulin deposits in the endoneurial space.
View Article and Find Full Text PDFJ Neuropathol Exp Neurol
May 2005
Department of Neurology, University Hospital, 2 Avenue Martin Luther King, 87042 Limoges, France.
There are many potential mechanisms of peripheral nerve impairment by a monoclonal IgG dysglobulinemia. In this study, using electron microscopy, we observed widening of the myelin lamellae comparable to that commonly described in IgM neuropathies with antimyelin-associated glycoprotein activity. Such features have yet to be described in IgG neuropathies.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!