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A 64-year-old man with xerostomia and xerophthalmia presented with numbness over the right chin and cheek, progressing over 18 months. He chewed food exclusively on the left side, as he would bite his tongue chewing on the right. On examination, there was multimodality trigeminal sensory loss in the right maxillary (V2) and mandibular (V3) distributions, with weakness and wasting of the right masseter and temporalis muscles ( figure 1 A). Forehead sensation (V1) and corneal reflex were normal. MR scan of head showed atrophy of all the right-sided muscles of mastication ( figure 2 ); there was abnormal gadolinium enhancement of the right trigeminal nerve, including its maxillary ( figure 3 A) and mandibular divisions ( figure 4 ). While some enhancement of V2 and V3 is normal, the degree and asymmetry of enhancement was obviously abnormal ( figures 3 and 4 ). The increased enhancement was limited to the nerves themselves, with no appreciable abnormal dural-based enhancement. The serum antinuclear antibodies-Sjögren's syndrome A (anti-Ro/SSA) and B (anti-La/SSB)-were elevated (>8.0 units; normal range, <1 unit). The Rose Bengal test confirmed keratoconjunctivitis sicca.
We diagnosed a right trigeminal neuropathy due to Sjögren's syndrome. Three months following treatment with oral prednisone 60 mg daily, there was almost complete resolution of trigeminal nerve enhancement...