Teaching NeuroImages: Primary Sjögren syndrome presenting as isolated lesion of medulla oblongata.

A 21-year-old woman presented with 2 weeks of vomiting and a brief episode of sudden loss of consciousness. On examination, she had slurred speech and dysphagia. Fluid-attenuated inversion recovery and T2-weighted MRI of the brain revealed whitematter hyperintensity within the medulla oblongata (figure 1, A–C). Biopsy of the salivary gland was diagnostic of Sjögren syndrome (SS) (figure 2). After multimodal therapy, lesions of medulla oblongata improved (figure 1D), and the patient largely returned to normal. Primary SS is characterized by chronic inflammation of exocrine glands. Involvement of CNS has been reported occasionally.