A Rare Case of Cerebral Venous Thrombosis Revealing a Primary Sjögren's Syndrome

Abstract

Sjögren's syndrome, formerly known as Gougerot-Sjögren syndrome, is an autoimmune disease with a predilection for exocrine glands, earning it the nickname of exocrinopathy or autoimmune epithelitis of the exocrine glands. It is a rare disease that predominantly affects females, with a sex ratio of 9 women to 1 man and an incidence peak around the age of 50 years. Sjögren's syndrome can be primary when isolated, or secondary when associated with another autoimmune disease which the most common is Rheumatoid Arthritis but also Lupus Erythematosus and Scleroderma. The triad defining the disease includes dryness syndrome, pain, and fatigue [1]. Cerebral thrombophlebitis corresponds to the blockage of a vein around the brain by a blood clot, initially causing few symptoms but eventually leading to persistent headaches that worsen progressively, possibly accompanied by vomiting or epileptic seizures [2]. We report the case of a patient who presented with cerebral thrombophlebitis revealing primary Sjögren's syndrome.