P264 Sjögren’s syndrome presenting with myelitis and without SICCA symptoms

Abstract

Sjogren’s syndrome (SS) is a chronic inflammatory disorder of exocrine glands with autoimmune etiology. Sjogren’s syndrome affect the nervous system in approximately 20% of cases. The prevalence of peripheral and central neurological manifestations in Sjogren syndrome are about 15% and 5% respectively. Neurological symptoms may occur before the onset of SICCA symptoms (dry eyes and dry mouth). Anti-Ro/SSA and anti-La/SSB antibodies are present 50–70% of patients with Sjogren syndrome. Patients with SS that have neurological symptoms, only 21% have anti-SSA or anti-SSB antibodies. In this article, we report a patient with SS, presenting as myelitis without any SICCA symptoms and without any Anti-Ro/SSA and anti-La/SSB antibodies in the sera of patient. Most common peripheral nervous system (PNS) presentations are axonal polyneuropathies (distal axonal sensory and sensorimotor), sensory ganglionopathy, motor neuropathy, small fiber neuropathy, multiple mononeuritis, trigeminal and other cranial neuropathies, autonomic neuropathies, demyelinating polyradiculoneuropathy. The sensory neuropathies constitute the most frequent PNS complication. Central nervous system (CNS) manifestations of Sjogren’s syndrome may affect spinal cord, brain stem, optic nerves, cerebellum, and cerebral hemispheres. CNS involvement is much less common than peripheral nervous system involvement. Transverse myelitis is an inflammatory disorder of the spinal cord that presents acutely. The presence of sensorial axonal polyneuropathy and longitudinally extensive transverse myelitis that spans the servical and thoracic spinal cord led us thinking about SS. The positive Schirmer’ test and lip biopsy supported the diagnosis of SS. So, it is important to consider SS in differential diagnosis even if there is no SICCA syndrome or even if autoantibodies are negative.