IDIOPATHIC AXONAL NEUROPATHY AND PRIMARY SJöGREN'S SYNDROME

Abstract

Objective To assess the diagnostic outcome of patients with an idiopathic neuropathy that were referred to a specialist Sjogren9s clinic. Background In about 15% of patients with a chronic axonal polyneuropathy a cause cannot be found following standard investigations. Primary Sjogren9s 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 Sjogren9s 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 Schirmer9s 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.