title: Connective tissue disorders: systemic lupus erythematosus, Sjögren's syndrome, and scleroderma.
abstract: Connective tissue disorders are systemic, autoimmune, multiorgan diseases in which the central and peripheral nervous systems are frequently involved. The objective of this chapter is to describe the neurological manifestations of three of the most common systemic autoimmune disorders: systemic lupus erythematosus (SLE), scleroderma, and Sjögren's syndrome (SS). In SLE the neuropsychiatric manifestations involve mainly the central nervous system (CNS), including cognitive dysfunction, headache, psychosis and mood changes, seizures, cerebrovascular disease, and myelopathy. Peripheral nervous system (PNS) manifestations are less common and include polyneuropathies as well as mononeuropathies and acute inflammatory demyelinating polyneuropathy. Antiphospholipid syndrome (APLS) is relatively common and should be searched for whenever focal neurological symptoms occur. In scleroderma the PNS is more commonly involved; symptoms include polyneuropathies, entrapment neuropathies, and mononeuropathies (mostly cranial neuropathies or mononeuritis multiplex). Additionally autonomic involvement occurs and myopathies are relatively common. In SS the PNS is similarly involved with several types of polyneuropathies, mononeuropathies, and autonomic dysfunction. Also common are myelopathies and aseptic meningitides. These and other, less common manifestations, as well as the diagnostic procedures and the therapeutic approaches, will be dealt with in this chapter.
authors.affiliation: Department of Neurology, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv and Neurology Unit, Hasharon Hospital, Rabin Medical Center, Petah Tikva, Israel. Electronic address: jonathans@clalit.org.il.
authors.forename: Jonathan Y
authors.initials: JY
authors.lastname: Streifler
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv and Rheumatology Unit, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel.
authors.forename: Yair
authors.initials: Y
authors.lastname: Molad
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D009422:Nervous System Diseases / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications; D012859:Sjogren's Syndrome / Q000150:complications
keywords: Connective tissue disorders; Sjögren’s syndrome; antiphospholipid syndrome; multisystem rheumatic disorders; neuropsychiatric manifestations; peripheral nervous system manifestations; scleroderma; systemic autoimmune disorders; systemic lupus erythematosus