Pseudotumoral brain lesion as the presenting feature of primary Sjögren's syndrome.

Background: The frequency and type of central nervous system involvement in primary Sjögren's syndrome (pSS) remain controversial. Brain magnetic resonance imaging (MRI) abnormalities in pSS are usually discrete hyperintense areas in the white matter. Tumefactive brain lesions have been rarely reported.

Case Report: We describe a 31-year-old woman who exhibited transcortical motor aphasia, hemiparesis and partial motor seizures as the initial manifestation of pSS. Brain MRI revealed a large frontoparietal lesion extending into the corpus callosum. The patient had spontaneous recovery and developed sicca symptoms 6 months after onset. Primary SS was diagnosed on the basis of clinical features, abnormal Schirmer test findings, high levels of anti-La/SSB antibodies and positive salivary gland biopsy results.

Conclusion: The present case suggests that a pseudotumoral brain lesion can occur as an initial symptom of pSS.