Forty consecutive patients with primary Sjögren's syndrome (SS) were prospectively evaluated for evidence of neurologic manifestations of the disease. All patients had primary SS diagnosed on the basis of typical sicca symptomatology, objective documentation of keratoconjunctivitis sicca (KCS) and/or xerostomia, and a positive minor labial salivary gland biopsy. None had criteria that would classify him/her as systemic lupus. Evaluation included a detailed neurologic history and physical examination and nerve-conduction studies of all four extremities. Ten patients had a mild sensory or mixed neuropathy of the glove-stocking pattern, and six of them had abnormal nerve conduction studies. Two of those, plus one more, had trigeminal neuropathy. Two more patients had abnormal electrophysiologic studies, one of them with absence of Achilles tendon reflexes as well. None of the above patients volunteered complaints related to these findings. Only one patient with severe primary SS, manifested by cryoglobulinemia, vasculitis and glomerulonephritis, presented with mononeuritis multiplex, that partially responded to intravenous cyclophosphamide and high-dose steroids. Central nervous system (CNS) disease was not detected in any of our patients, suggesting that it is rather rare, whereas peripheral nervous system (PNS) involvement is relatively common and benign in the majority of primary SS individuals.
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