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J Neurol Neurosurg Psychiatry 1997;63:376-378 doi:10.1136/jnnp.63.3.376
  • Short report

Sjögren’s syndrome in patients with chronic idiopathic axonal polyneuropathy

  1. G W van Dijka,
  2. N C Notermansa,
  3. L Katerb,
  4. A A Kruizeb,
  5. W H J P Linssenc,
  6. J H J Wokkea
  1. aDepartments of Neurology, bRheumatology and Clinical Immunology, University Hospital, Utrecht, The Netherlands, cDepartment of Neurology, St Lucas-Andreas Hospital, Amsterdam, The Netherlands
  1. Dr GW van Dijk, Department of Neuromuscular Diseases, University Hospital, Utrecht, PO Box 85500, C03.236, 3508 GA Utrecht, The Netherlands.
  • Received 21 January 1997
  • Revised 19 March 1997
  • Accepted 3 April 1997

Abstract

OBJECTIVE To assess the presence of symptoms and signs of Sjögren’s syndrome in patients with otherwise idiopathic axonal polyneuropathy and to develop guidelines for the diagnostic approach with respect to Sjögren’s syndrome in these patients.

METHODS Sixty five patients with axonal polyneuropathy in whom an aetiological diagnosis could not be made underwent (1) a standard interview focusing on ocular and oral sicca symptoms, (2) physical examination, (3) tests for objective assessment of keratoconjunctivitis sicca, (4) extensive serological investigations, and (5) a sublabial salivary gland biopsy.

RESULTS In forty nine patients a sublabial salivary gland (SSG) biopsy was performed, thereby completing the whole investigation for Sjögren’s syndrome. Three of these 49 patients (all women) had an SSG biopsy specimen suggestive of Sjögren’s syndrome, which, in combination with other symptoms and signs, led to a diagnosis of primary Sjögren’s syndrome.

CONCLUSIONS None of the three patients with primary Sjögren’s syndrome had spontaneously complained about sicca symptoms and the clinical neurological picture of them did not differ from the other patients in the study. Therefore, in patients with chronic idiopathic axonal polyneuropathy, especially in women, a systematic investigation for Sjögren’s syndrome should be done, because the presence of Sjögren’s syndrome may have implications for treatment and justifies a clinical follow up on a regular base.

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