Summary
A 56-year-old woman suffering from primary Sjögren's syndrome showed a distal, symmetric, predominantly sensory neuropathy of the lower limbs, with slow nerve conduction velocity and raised CSF protein.
Sural nerve biopsy demonstrated severe loss of myelinated fibers with increased collagen, prominent remyelination on teased fibers, and onion bulb formation on electron microscopy.
On the basis of the findings of hypertrophic neuropathy (not previously reported in this disease) and of raised CSF protein, the polyneuropathy of Sjögren's syndrome is compared with chronic relapsing polyneuropathy and a possible role of immunologic mechanisms is suggested.
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Marbini, A., Gemignani, F., Manganelli, P. et al. Hypertrophic neuropathy in Sjögren's syndrome. Acta Neuropathol 57, 309–312 (1982). https://doi.org/10.1007/BF00692189
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DOI: https://doi.org/10.1007/BF00692189