Primary Sjogren’s Syndrome with Renal Tubular Acidosis with Central Pontine Myelinolysis - A rare case report

A middle aged female patient, a case of Primary Sjogren's Syndrome with renal tubular acidosis as revealed by severe hypokalemia along with normal anion gap, metabolic acidosis and acidic urinary pH had brain stem lesion which presented as quadriplegia, dysphagia and dysarthria. Laboratory tests revealed that anti-nuclear antibodies (ANA) and anti Ro/SSA antibodies were positive. MRI showed hyper intense lesion in T2W images in middle pons, typical characteristic of central pontine myelenolysis. So, patient was diagnosed as Primary Sjogren's Syndrome with renal tubular acidosis with central pontine myelinolysis. She recovered with correction of hypokalemia, intravenous methyl prednisolone and cyclophosphamide.