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A Population-Based Study

Lasse G. Gøransson, MD; Anita Herigstad, MD; Anne B. Tjensvoll, MD; Erna Harboe, MD; Svein I. Mellgren, MD, PhD; Roald Omdal, MD, PhD

Arch Neurol. 2006;63:1612-1615.

Background  Neurological manifestations appear to be frequently involved in patients with primary Sjögren syndrome (PSS).

Objective  To investigate the involvement of the peripheral nervous system, including small-diameter nerve fibers, in an unselected cohort of patients who fulfilled the new international criteria for PSS.

Design  Cross-sectional study.

Setting  Stavanger University Hospital.

Patients  Sixty-two patients with PSS (mean ± SD age, 57.1 ± 14.6 years).

Interventions  Clinical neurologic examinations, conventional nerve conduction studies, and skin punch biopsies.

Main Outcome Measures  Signs of large-diameter and small-diameter peripheral nerve fiber neuropathy as determined by clinical examination, nerve conduction studies, and densities of intraepidermal nerve fibers in skin punch biopsy specimens.

Results  Seventeen patients (27%) were diagnosed as having neuropathy after clinical examination. The results of nerve conduction studies were abnormal in 34 patients (55%): 19 patients (31%) had motor neuropathy, 8 (13%) had sensory neuropathy, and 7 (11%) had sensorimotor neuropathy. Two patients had intraepidermal nerve fiber densities less than 3.4 fibers per millimeter, fitting the morphologic criteria for small-diameter nerve fiber neuropathy.

Conclusions  Peripheral neuropathy occurs in a large proportion of patients with PSS, in most cases as a subclinical demyelinating neuropathy. Small-diameter nerve fiber neuropathy is not a frequent finding in these patients.

Author Affilations: Clinical Immunology Unit, Department of Internal Medicine (Drs Gøransson, Harboe, and Omdal), Department of Clinical Neurophysiology (Dr Herigstad), and Department of Neurology (Dr Tjensvoll), Stavanger University Hospital, Stavanger; Institute of Internal Medicine, University of Bergen, Bergen (Drs Gøransson and Omdal); Institute of Clinical Medicine, University of Tromsø, Tromsø (Dr Mellgren); and Department of Neurology, University Hospital of North Norway, Tromsø (Dr Mellgren), Norway.