Peripheral neuropathy in primary Sjögren's syndrome

Abstract

Sjögren's syndrome (dryness of eyes, mouth, and other mucous membranes) may be associated with disease of joints, blood, internal organs, skin, and central and peripheral nervous systems. We reviewed 33 cases of primary Sjögren's syndrome and peripheral neuropathy evaluated by neurologic examinations and EMG at the Mayo Clinic from 1976 to 1988, and studied sural nerve biopsy specimens in 11 of them. Symmetric sensorimotor polyneuropathy occurred most frequently, followed by symmetric sensory neuropathy. Autonomic neuropathy, mononeuropathy, or cranial neuropathy (especially trigeminal neuropathy) was superimposed on generalized neuropathy in approximately one-fourth of patients. The course generally was slowly progressive, except for a few patients who may have improved with prednisone therapy. Although spinal ganglion involvement might have accounted for some of the clinical and neurophysiologic findings, we found evidence that necrotizing vasculitis was involved in fiber degeneration. All nerve biopsies revealed perivascular inflammatory infiltrates and other vessel abnormalities, which were diagnostic in two cases and strongly suggestive of necrotizing vasculitis in six cases. Axonal degeneration predominated over demyelination and sometimes was focal or multifocal. In neuropathy of unknown cause, particularly if it is sensory, autonomic, or involves trigeminal nerve, consider Sjögren's syndrome.

Neurological involvement in primary Sjogren's syndrome

“…The current study agrees with those of Deland et al, Mellgren et al and Lafitte et al, who described many cases (between 47-53%), of pSS with neurologic manifestations as presenting symptoms [11][12][13] . The absence of sicca symptoms in 46% of our patients at the onset of neurologic signs, especially in cases of CNS involvement, emphasizes the difficulty of the diagnosis and the utility of systematically screening for SS in patients with neurologic symptoms such as sensory motor axonal polyneuropathy, pure sensory neuropathy, cranial nerve involvement (especially cochlear and trigeminal nerves), acute or chronic myelopathy, or multifocal CNS involvement with onset after 50 years of age 2,9 .…”

“…Our series include the main types of neuropathy already described in pSS. Small fiber neuropathy (SFN) is a neurological manifestation of pSS recently describe and the most published series there is no reference to this manifestation 12,13 . SFN is a polyneuropathy selectively involving small--diameter myelinated (A-delta) and non-myelinated (C) nerve fibers and leaving large myelinated fibers relatively unaffected.…”

Motor Dominant Neuropathy in Sjoegren's Syndrome: Report of Two Cases.

“…Wereport here severe axonal and acute motor neuropathy in SS (the first case). According to previous reports, the typical features of peripheral nerve biopsies in SS include the presence of mononuclear inflammatory cells in and around small epineural blood vessels, and a decrease in the mean density of myelinated fibers (6,7). However, some nerve biopsied reports of SS neuropathy failed to reveal either vasculitis or lymphocytic infiltration, though remyelination was observed (8,9).…”

Ocular Manifestations of Sjögren Syndrome

“…Symptoms of dry eye have been reported to follow or occur concurrently with clinical manifestations of PN in some of these patients [16,17]. Although symptoms of corneal dysesthesia have been generally considered as secondary to dry eye even without corneal epitheliopathy, the dry eye could be caused by the immunopathic pathogenesis of corneal neuropathy in early SS [18].…”