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Sjögren's syndrome: an unusual cause of Bell's palsy
  1. M HADITHI,
  2. F STAM,
  3. A J M DONKER
  1. Department of Internal Medicine
  2. Department of Rheumatology
  3. Academic Hospital Vrije Universiteit
  4. Amsterdam, The Netherlands
  1. m.hadithi{at}azvu.nl
  1. B A C DIJKMANS
  1. Department of Internal Medicine
  2. Department of Rheumatology
  3. Academic Hospital Vrije Universiteit
  4. Amsterdam, The Netherlands
  1. m.hadithi{at}azvu.nl

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The most common form of facial paralysis is idiopathic—that is, Bell's palsy. Sjögren's syndrome (SS), a chronic inflammatory disorder characterised by lymphocytic infiltration of exocrine glands resulting in the so called “sicca complex”, is a rare secondary cause of this self limiting illness. Primary SS includes mostly peripheral, and to a lesser extent cranial, autonomic neuropathy and central nervous system involvement.1 A patient with unilateral facial palsy, autoimmune hypothyroidism, and Sjögren's syndrome is presented.

A 41 year old woman developed right sided facial numbness, described as “dentist anaesthesia for tooth extraction”. One day later she had a reduced sense of taste and right facial weakness. General physical examination was not remarkable. Neurological examination showed anisocoria, peripheral right sided facial paresis, reduced sense of taste on the right half of the tongue, and dysaesthesia in the region of the second segment of the right trigeminal nerve.

Although the erythrocyte sedimentation rate (ESR) was 30 mm/1st h, routine laboratory investigations were normal. Liquor examination, including IgG, IgG index, and oligoclonal or extra bands on electrophoresis, was not abnormal. Screening tests for herpes simplex and varicella virus, syphilis, and borrelia in the serum and in the liquor were negative. Magnetic resonance imaging (MRI) of the brain showed no abnormalities. Nerve conduction studies showed peripheral facial paresis. She recovered spontaneously from her symptoms within several days. Conversely, during follow up the raised ESR persisted.

Review of her medical history uncovered complaints of burning eyes and dry mouth, slight weight gain, and cold intolerance. There was no history of arthralgias or skin lesions. She denied using any drugs previously. A strongly positive anti-extranuclear antigen (ENA)/SS-A antibodies test was shown on further investigation, with negative tests for antinuclear factor/anti-nDNA antibodies/rheumatoid factor and RNP/SS-B/Sm antibodies. Rose-Bengal staining showed corneal punctate lesions (van Bijsterveld score 6). Lower labial biopsy showed histopathological findings matching the diagnosis of SS with a focus score of one lymphocyte focus for 4 mm2 salivary gland tissue. Additionally, thyroid function tests showed a raised thyroid stimulating hormone (11 mU/l), low free thyroxine 4 (13.0 pmol/l) with positive antithyroid microsomal antibodies and negative antithyroglobulin antibodies.

The clinical, serological, and histopathological manifestations fulfilled the European study group criteria for the diagnosis of SS. The patient was treated with artificial tears and thyroxine supplements that returned her thyroid function tests to normal.

Prevalence of neuropathy in patients with SS ranges from 10 to 50%.2 Polyneuropathy can be the first clinical manifestation of SS and may even precede sicca symptoms in 40% of patients.3 However, less frequently, cranial neuropathy can occur with a predisposition to involvement of the trigeminal nerve.4 The vasculitic damage to vaso nervorum documented by pathological studies is associated with a higher incidence of serum anti-SS-A (Ro) antibodies.5 The association of SS with autoimmune thyroid disease (AITD) is well recognised.6 7AITD and SS share similarities in the immunopathology in addition to their genetic linkage to the HLA-DR3/DR4 alleles.7 Only nine cases of facial nerve involvement associated with SS have been described previously.1 8-10

This case illustrates how facial palsy disclosed the primary SS as an underlying systemic disorder. To our knowledge the combination Bell's palsy as presenting feature in a patient with SS, and hypothyroidism secondary to AITD has not been reported hitherto.

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