title: [Acute axonal polyneuropathy and Gougerot-Sjögren syndrome].
abstract: BACKGROUND
  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.
  
  
  METHODS
  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).
  
  
  CONCLUSIONS
  This case illustrates various clinical signs and inconstant responsiveness to treatment of neuropathy associated with primary GSS.
authors.affiliation: Service de Neurologie, Hôpital Font-Pré, 1208 avenue Colonel-Picot, 83100 Toulon, France.
authors.forename: N-Y
authors.initials: NY
authors.lastname: Mapoure
authors.forename: R
authors.initials: R
authors.lastname: Ali-Ahmad
authors.forename: L
authors.initials: L
authors.lastname: Aubert
authors.forename: C
authors.initials: C
authors.lastname: Fernandez
authors.forename: N
authors.initials: N
authors.lastname: Schleinitz
authors.forename: F
authors.initials: F
authors.lastname: Macia
mesh_terms: D000328:Adult; D001369:Axons / Q000473:pathology*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011115:Polyneuropathies / Q000150:complications / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000150:complications / Q000473:pathology*; D013497:Sural Nerve / Q000473:pathology; D017211:Treatment Failure; D001750:Urinary Bladder, Neurogenic / Q000209:etiology
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents