Intracranial Hypertension as a rare presentation of Sjögren's Syndrome: a case report

Introduction. A rare extraglandular manifestation of Sjögren's syndrome is presented, mimicking idiopathic intracranial hypertension. Case Report. A young eutrophic female, complained of persistent headache, followed by diplopia and bilateral sixth nerve paresis. Neuroimaging investigation showed classic signs of intracranial hypertension. As the patient had a normal weight, prompted evaluation of secondary causes, which revealed positive anti-SSA/Ro and anti-La/SSB antibodies. A salivary gland biopsy later confirmed the diagnosis of Sjögren's syndrome. Xerostomia and dysphagia developed weeks later. Conclusion. This case report is important to raise awareness about an atypical neurological manifestation of Sjögren's syndrome.