• Anne-Laure Fauchais, Laurent Magy, and Elisabeth Vidal.
• Limoges University Hospital, Internal Medicine Unit, 87042 Limoges, France. anne-laure.fauchais@chu-limoges.fr
• Presse Med. 2012 Sep 1; 41 (9 Pt 2): e485-93.

AbstractPrimary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. Clinically significant neurologic manifestations affect approximately 20% of patients and may be the first manifestation of the disease in at least 25% of the cases. The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability.Copyright © 2012 Elsevier Masson SAS. All rights reserved.

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