Abstract
A 60 y.o. female was investigated for progressive distal paresthesias, which had started a few years before. Neurological examination revealed mild sensory ataxia, areflexia in lower limbs with normal motor function. Routine laboratory tests were unremarkable; autoantibodies to non‐organ‐specific, extractable nuclear and cytoplasmic antigens were absent and CSF was normal; neurophysiologic findings showed absent sural sensory responses. Sural nerve biopsy revealed a complete depletion of large and small myelinated fibers without aspects of regeneration; no vascular or perivascular inflammation nor changes suggestive of vasculitis were detected. Salivary gland biopsy showed focal lymphocytic infiltrates. Late systemic manifestations included dry skin, xerophtalmia and arthralgias. Severe sensory neuropathy is rarely associated with Sjögren syndrome. It occurs more frequently in female patients and its major pathologic process is believed to be a dorsal root ganglionitis.
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