A wide range of central and peripheral nervous system disorders occur in patients with primary Sjögren's syndrome (pSS), although the true prevalence is an aspect which has been and remains controversial. Under-recognition of pSS and lack of consensus regarding criteria contribute to the uncertainty regarding the extent of neuropsychiatric involvement. A relatively high rate of affective and cognitive symptoms, as well as abnormal fatigue and poorly characterized pain, are features of pSS that contribute to diminishing health quality in the pSS population. This article describes the neurologic complications and controversies that surround the neurologic syndromes associated with pSS and reviews the current literature on potential immunopathogenetic mechanisms and therapy. © 2008 Elsevier Inc. All rights reserved.
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