Central nervous system (CNS) manifestations of primary Sjögren's syndrome: an overview

Central nervous system (CNS) complications occur in approximately 25% of patients with primary Sjögren's syndrome (CNS-SS) seen at our institution. We describe herein the clinical, neurodiagnostic, and immunopathologic features of CNS-SS. The neurologic manifestations can be protean and affect the entire neuroaxis. Characteristically, CNS-SS is multifocal, recurrent, and progressive resulting in cumulative neurologic impairment over time. In addition to specific neurologic deficits, more subtle abnormalities of personality and mentation may occur. Whereas, computed axial tomography of the head and cerebral angiography are relatively insensitive techniques in detecting abnormalities, brain magnetic resonance imaging shows multiple small areas of increased intensity in two thirds of CNS-SS patients. Electrophysiologic studies or cerebrospinal fluid analyses are abnormal in two thirds of CNS-SS patients. Preliminary histopathologic studies show several types of CNS inflammatory involvement suggesting an immunologically mediated autoimmune disorder.