Trigeminal sensory neuropathy associated with connective tissue diseases

We reviewed the clinical and laboratory features of 81 patients who had trigeminal sensory neuropathy (TSN) and a connective tissue disease (CTD). The neuropathy developed before the symptoms of CTD in 6/81 patients (7%), and in 38/81 patients (47%) TSN and CTD were diagnosed concurrently. The most frequently associated CTDs were undifferentiated connective tissue disease (38/81, 47%), mixed connective tissue disease (21/81, 26%), and scleroderma (15/81, 19%). Of 66 patients followed for more than 1 year (median, 5 years; range, 1 to 26 years), 8/66 patients (12%) had mild improvement and 2/66 (3%) had marked improvement of numbness; no patient had complete return of sensation. The facial numbness was frequently associated with moderate to severe facial pain that was usually resistant to pharmacologic therapy. None of the patients developed clinical or laboratory evidence of systemic vasculitis. The etiology of this cranial sensory neuropathy remains obscure.