Atypical Neurologic Complications in Patients with Primary Sjogren's Syndrome: Report of 4 Cases

Background: Neurologic involvement occurs in approximately 25% of patients with primary Sjogren's syndrome. Manifestations are diverse and can affect the entire neuroaxis. Central nervous system dysfunction involves the brain as well as the spinal cord and may recur over time. Due to a variety of presentations, Sjogren's syndrome with neurologic involvement may be difficult to diagnose. Methods: We report 4 cases of patients with primary Sjogren's syndrome who presented with atypical neurologic manifestations. Results: The first case describes a patient with a pseudotumoral lesion. The second patient was a 54-year-old woman suffering from a multiple mononeuropathy. The third case describes a 66-year-old man whose primary Sjogren's syndrome presented as progressive multiple sclerosis, and the fourth case reports a 57-year-old woman patient suffering from myelitis along with progressive cognitive disorders. Conclusions: Neurologic impairment in Sjogren's syndrome is probably underestimated and the diagnosis is often delayed. Primary Sjogren's syndrome should be suspected in patients presenting with atypical clinical and radiologic neurologic manifestations. (C) 2011 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 40:338-342