title: Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome.
abstract: Clinical involvement of the peripheral nervous system in the calcinosis cutis, raynaud's phenomenon, esophageal dismotility, sclerodactyly and telangiectasia (CREST) variant of systemic sclerosis occurs infrequently and is characterized by axonal degeneration due to necrotizing vasculitis. We report a female patient with a known history of CREST syndrome, which developed a slowly progressive, distal symmetric demyelinating sensorimotor polyneuropathy (PN), with tremor and ataxia as prominent features, compatible with anti-myelin associated glycoprotein (MAG) PN. The diagnosis of PN was established by the presence of monoclonal immunoglobulin M anti-MAG antibodies (Thin-Layer Chromatography, Western Blot and enzyme-linked immunoabsorbent assay). Given the evidence that in CREST activation of T-helper cells is observed and that anti-MAG antibodies, despite the fact that they are T-cell-independent, may be influenced by an increase in T-helper function, the coexistence of these two rare autoimmune disorders in the same patient may not be incidental but related to the underlying immunological mechanisms involved.
authors.affiliation: Department of Neurology, Athens National University, Aeginition Hospital, Athens, Greece. eandread@med.uoa.gr
authors.forename: E
authors.initials: E
authors.lastname: Andreadou
authors.forename: V
authors.initials: V
authors.lastname: Zouvelou
authors.forename: N
authors.initials: N
authors.lastname: Karandreas
authors.forename: C
authors.initials: C
authors.lastname: Kilidireas
mesh_terms: D000368:Aged; D000888:Antibodies, Anti-Idiotypic; D001259:Ataxia / Q000209:etiology; D001323:Autoantibodies / Q000032:analysis*; D015153:Blotting, Western; D017675:CREST Syndrome / Q000150:complications* / Q000276:immunology; D002855:Chromatography, Thin Layer; D003520:Cyclophosphamide / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D011115:Polyneuropathies / Q000150:complications / Q000276:immunology*; D016896:Treatment Outcome; D014202:Tremor / Q000209:etiology
chemical_list: D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents; D018992:Myelin-Associated Glycoprotein; C044203:anti-IgM; D003520:Cyclophosphamide