Encephalopathy in Sjogren’s Syndrome - A Distinct Neurological Presentation

Sjogren’s syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma infiltration of exocrine glands, resulting in dry mouth and kerato-conjunctivitis sicca. The clinical symptoms may range from mucosal dryness to more systematic complaints. Sjogren's syndrome (SS) may be complicated by neurological manifestations.1 We report a 36-year-old woman presenting with quadriparesis, started as lower limb weakness which progressed to the upper limbs, having Guillain-Barre syndrome like pattern. MRI brain showed altered signal intensity in the brainstem, predominantly centered in the pons labelled as autoimmune brainstem encephalitis with distal RTA. With differentials as clippers, autoimmune disease and de-mylinating disease patient underwent an Autoimmune panel which showed Antinuclear Antibody showing speckled SSA/RO pattern and antibodies to SSB/LA were positive suggestive of Sjogren’s syndrome. The diagnosis was confirmed with a minor salivary gland biopsy from the lower lip which was definitive of Sjogren’s syndrome. Patient showed excellent improvement on pulsed steroid therapy. Therefore, this case illustrates a rare, heralding manifestation of Sjogren’s syndrome as brainstem encephalitis with definitely diagnosis based on minor salivary gland biopsy showing complete recovery on early detection and management.