[Acute axonal polyneuropathy and Gougerot-Sjögren syndrome].

Abstract

INTRODUCTION:

Gougerot-Sjögren syndrome (GSS) is an autoimmune disease characterized by a lymphocytic infiltration and destruction of saliva and lachrymal glands. About 20% of patients develop a neurological involvement.

CASE REPORT:

A 29-year-old woman was admitted with a dysautonomic and sensorial neuropathy. Clinical manifestations (dry syndrome and positive Schirmer's test), blood (anti-SSA and anti-SSB antibodies) and histological (chronic sialadenitis) tests linked this neuropathy to a primary GSS. The characteristics of this case were acute onset (within a few days), severe clinical presentation (continued confinement to bed and malnutrition) and non-responsiveness to all treatments (intravenous immunoglobulins, corticosteroids, plasmapheresis, rituximab).

CONCLUSION:

This case illustrates various clinical signs and inconstant responsiveness to treatment of neuropathy associated with primary GSS.

• Authors: N-Y Mapoure, R Ali-Ahmad, L Aubert, C Fernandez, N Schleinitz, F Macia
• Journal: Revue neurologique (Rev Neurol (Paris)) 2011 Jun-Jul Vol. 167 Issue 6-7 Pg. 530-2 ISSN: 0035-3787 [Print] France
• Vernacular Title: Polyneuropathie axonale aiguë et syndrome de Gougerot-Sjögren.
• PMID: 21492890 (Publication Type: Case Reports, English Abstract, Journal Article)
• Copyright: Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Chemical References

• Immunoglobulins, Intravenous
• Immunosuppressive Agents

Topics

• Adult
• Axons (pathology)
• Female
• Humans
• Immunoglobulins, Intravenous
• Immunosuppressive Agents (therapeutic use)
• Polyneuropathies (complications, pathology)
• Sjogren's Syndrome (complications, pathology)
• Sural Nerve (pathology)
• Treatment Failure
• Urinary Bladder, Neurogenic (etiology)