title: Central and peripheral neurological complications of primary Sjögren's syndrome.
abstract: Primary 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.
authors: Limoges University Hospital, Internal Medicine Unit, 87042 Limoges, France. anne-laure.fauchais@chu-limoges.fr
authors: Anne-Laure
authors: AL
authors: Fauchais
authors: Laurent
authors: L
authors: Magy
authors: Elisabeth
authors: E
authors: Vidal
mesh_terms: D020274:Autoimmune Diseases of the Nervous System / Q000209:etiology*; D001927:Brain Diseases / Q000209:etiology; D002493:Central Nervous System Diseases / Q000209:etiology*; D006801:Humans; D020422:Mononeuropathies / Q000209:etiology; D009046:Motor Neurons / Q000473:pathology; D009103:Multiple Sclerosis / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D011115:Polyneuropathies / Q000209:etiology; D011984:Sensory Receptor Cells / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications*; D013118:Spinal Cord Diseases / Q000209:etiology; D014657:Vasculitis / Q000209:etiology