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Case Reports in Medicine
Volume 2010, Article ID 542925, 3 pages
http://dx.doi.org/10.1155/2010/542925
Case Report

Peripheral Neuropathy and VIth Nerve Palsy Related to Randall Disease Successfully Treated by High-Dose Melphalan, Autologous Blood Stem Cell Transplantation, and VIth Nerve Decompression Surgery

1Service de Gériatrie, Hôpital Jean Minjoz, CHU, Boulevard Fleming, 25000 Besançon Cedex, France
2Service de Médecine Interne Gériatrie, Hôpital de Champmaillot, CHU BP 87909, 2 rue Jules Violle, 21079 Dijon Cedex, France
3INSERM/U887 Motricité-Plasticité: Performance, Dysfonctionnement, Vieillissement et Technologies d'optimisation, Université de Bourgogne, BP 27877, 21078 Dijon Cedex, France
4Service de Médecine Interne, Hôpital Jean Minjoz, CHU, Boulevard Fleming, 25000 Besançon Cedex, France

Received 11 September 2010; Revised 14 November 2010; Accepted 17 November 2010

Academic Editor: Shaji Kumar

Copyright © 2010 C. Foguem et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Randall disease is an unusual cause of extraocular motor nerve (VI) palsy. A 35-year-old woman was hospitalized for sicca syndrome. The physical examination showed general weakness, weight loss, diplopia related to a left VIth nerve palsy, hypertrophy of the submandibular salivary glands, and peripheral neuropathy. The biological screening revealed renal insufficiency, serum monoclonal kappa light chain immunoglobulin, urinary monoclonal kappa light chain immunoglobulin, albuminuria, and Bence-Jones proteinuria. Bone marrow biopsy revealed medullar plasma cell infiltration. Immunofixation associated with electron microscopy analysis of the salivary glands showed deposits of kappa light chains. Randall disease was diagnosed. The patient received high-dose melphalan followed by autostem cell transplantation which led to rapid remission. Indeed, at the 2-month followup assessment, the submandibular salivary gland hypertrophy and renal insufficiency had disappeared, and the peripheral neuropathy, proteinuria, and serum monoclonal light chain had decreased significantly. The persistent diplopia was treated with nerve decompression surgery of the left extraocular motor nerve. Cranial nerve complications of Randall disease deserve to be recognized.