Trigeminal Neuralgia as an Initial Presentation of Systemic Autoimmune Diseases: A Case Series

BACKGROUND: Trigeminal neuralgia, also known as tic doloureaux, refers to a sudden onset of unilateral or bilateral facial numbness with or without pain or paraesthesia. Trigeminal neuralgia is rare in connective tissue diseases (CTD); however, it is the most common neurologic manifestation of mixed connective tissue (MCTD) and maybe the only presenting symptom in various CTDs. PATIENTS AND METHODS: Here, we describe a series of four cases of various autoimmune connective tissue diseases where trigeminal neuralgia was the presenting complaint. The first 2 cases were MCTD patients, and the 3rd case was a patient with diffuse cutaneous systemic sclerosis (SSc) and the 4th case had overlap syndrome (primary Sjogren’s syndrome with SSc). The relevant literature describing trigeminal neuralgia in CTD was reviewed. The authors performed a systematic search of patients with Trigeminal neuralgia and Connective tissue diseases in PubMed, Scopus from January 1970 until July 2022. RESULTS: All our cases had trigeminal neuralgia as presenting symptom which suggests that trigeminal neuralgia may be one of the presenting symptoms of several systemic autoimmune diseases that often cause a significant delay in diagnosis and treatment. We selected 15 records for the literature review. CONCLUSION: Any patient who presents with trigeminal neuralgia which responds poorly to medical management should be properly examined for underlying primary systemic autoimmune diseases.