Connective tissue disorders. systemic lupus erythematosus, Sjögren's syndrome, and scleroderma.

Jonathan Y. Streifler*, Yair Molad

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review


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.

Original languageEnglish
Title of host publicationHandbook of Clinical Neurology
PublisherElsevier B.V.
Number of pages11
StatePublished - 2014

Publication series

NameHandbook of Clinical Neurology
ISSN (Print)0072-9752


  • Antiphospholipid syndrome
  • Connective tissue disorders
  • Multisystem rheumatic disorders
  • Neuropsychiatric manifestations
  • Peripheral nervous system manifestations
  • Scleroderma
  • Sjögren's syndrome
  • Systemic autoimmune disorders
  • Systemic lupus erythematosus


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