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IDIOPATHIC AXONAL NEUROPATHY AND PRIMARY SJöGREN'S SYNDROME
  1. Etienne Laverse1,
  2. Amal Samaraweera1,
  3. Wan-Fai Ng2,
  4. James Miller1
  1. 1Royal Victoria Infirmary, Newcastle
  2. 2Freeman Hospital, Newcastle

Abstract

Objective To assess the diagnostic outcome of patients with an idiopathic neuropathy that were referred to a specialist Sjögren's clinic.

Background In about 15% of patients with a chronic axonal polyneuropathy a cause cannot be found following standard investigations. Primary Sjögren's Syndrome (PSS) has been associated with a variety of peripheral neuropathies, particularly dorsal root ganglioneuronopathy and small fibre neuropathy.

Method We retrospectively reviewed the clinicopathological records of thirty consecutive patients with undiagnosed neuropathies referred from a specialist peripheral nerve clinic to a regional Sjögren's clinic for further investigation.

Results Ten (33%) of the thirty patients were confirmed on lip biopsy to have PSS. 9/10 patients had a sensory/sensorimotor axonal neuropathy and 1/10 had a dorsal root ganglioneuronopathy. Three cases (30%) were positive for anti-nuclear antibodies at titres of 1:160 but all were negative for anti Ro/La antibodies. 4/10 had a normal Schirmer's test.

Six (60%) of the PSS cases had dysaesthetic symptoms and four (40%) complained of ataxia. Eight (80%) patients had distal sensory impairment of pinprick and/or vibration with absent ankle jerks.

Conclusions PSS is a common association in otherwise undiagnosed sensory axonal neuropathy. Salivary gland biopsy should be considered in all patients with an unidentified cause for sensory neuropathy and sicca symptoms.

  • EPILEPSY

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