The Neuropathy of Sjögren's Syndrome

SUMMARY

Ten patients had Sjögren's syndrome and peripheral or trigeminal neuropathy, or both. The neurologic features of other connective tissue diseases are reviewed for comparison. The peripheral neuropathy in Sjögren's syndrome was predominately sensory and characteristically mild, distal, and symmetrical. Spinal fluid examinations were usually normal. The neuropathy in Sjögren's syndrome was indistinguishable from that seen in rheumatoid arthritis. Initiation or manipulation of corticoid dosage was not related to the onset of neuropathy. The therapeutic response to steroids was unpredictable. A sensory trigeminal neuropathy occurred in four patients. It was characterized by numbness, hypesthesia, and tingling paresthesias. The divisional distribution of sensory deficit favored a peripheral rather than a central lesion or an entrapment neuropathy. The presence of neuropathy correlated strongly with vasculitis in the nerves and muscle. The vascular inflammation was felt to be causally related to the pathogenesis of the neuropathy.