Diversity of central nervous system manifestations in Sjogren's Disease: a case-based review.

Abstract

Sjogren's disease (SjD) is a chronic and disabling autoimmune disease, predominantly characterized by dryness of the mouth and eyes, resulting from lymphocytic infiltration of exocrine glands. While these are the most prominent symptoms, extra-glandular manifestations are also common. Studies suggest that up to 70% of SjD patients experience neurological symptoms, which interestingly often precede the hallmark dryness. Although every structure of the nervous system can be affected, disorders of peripheral nervous system (PNS) are more common than central nervous system (CNS) involvement. The CNS manifestations can range from subtle to severe, with some patients experiencing a rapid progression of symptoms. The literature documents cases where patients initially diagnosed with Creutzfeldt-Jakob disease, neurosarcoidosis, temporary hemiplegia, neuromyelitis optica, or epilepsy were ultimately diagnosed as having SjD. Here, we present five SjD patient cases, each with a different manifestation of CNS involvement, along with an overview of the current understanding of CNS disorders in the course of the disease. In four cases, the neurological manifestations appeared before the onset of sicca symptoms. In one patient, diagnosis was delayed by 15 years due to an atypical presentation. After receiving immunosuppression, all patients experienced notable alleviation of their symptoms.