[Acute axonal polyneuropathy and Gougerot-Sjögren syndrome].


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.

DOI: 10.1016/j.neurol.2010.10.009

Cite this paper

@article{Mapoure2011AcuteAP, title={[Acute axonal polyneuropathy and Gougerot-Sj{\"{o}gren syndrome].}, author={N Y Mapoure and R Ali-Ahmad and Laurence Aubert and Carlos Hern{\'a}ndez Fern{\'a}ndez and Nicolas Schleinitz and Fr{\'e}d{\'e}ric Macia}, journal={Revue neurologique}, year={2011}, volume={167 6-7}, pages={530-2} }