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| CASE REPORT |
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| Year : 2012 | Volume
: 58
| Issue : 1 | Page : 57-59 |
Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome
E Andreadou, V Zouvelou, N Karandreas, C Kilidireas
Department of Neurology, Athens National University, "Aeginition" Hospital, Athens, Greece
Correspondence Address:
E Andreadou
Department of Neurology, Athens National University, "Aeginition" Hospital, Athens
Greece
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0022-3859.93254
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.
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