Associated with a spectrum of extra glandular manifestations, primary Sjögren syndrome (pSS) is a chronic autoimmune disorder affecting primarily exocrine glands, causing xerophthalmia and xerostomia. First described by Sjögren in 1933 followed by Jönköping in 1935 , it has been well established that central and peripheral nervous systems involvement is one of the most serious complications of pSS. Based on many studies, 1.8%-70% of pSS patients may suffer from neurological manifestations [2-17]. Different study designs, selection bias depending on rheumatology or neurology specialty conducting the study, and degree of investigation on patients’ signs and symptoms may contribute to this significant disparity. Studies with lower prevalence focused on the general population [2,3 and 5], while the higher prevalence series are based on patients who present with neurological symptoms [16,17]. Indeed, the definition and classification criteria of pSS have been changed frequently making it difficult to compare different cohorts [18,19].