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 CASE REPORT
Year : 2012  |  Volume : 58  |  Issue : 1  |  Page : 57-59

Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome


Department of Neurology, Athens National University, "Aeginition" Hospital, Athens, Greece

Correspondence Address:
E Andreadou
Department of Neurology, Athens National University, "Aeginition" Hospital, Athens
Greece
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.93254

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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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Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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