Case Report: We report the case of a 64-year-old woman presenting with subacute-onset headache, nausea, vomiting and diplopia. There was mild encephalopathy and partial left 3rd nerve palsy on examination. She had a history of Sjogren’s syndrome comprising sicca-symptoms and stable interstitial lung disease, and was anti-Ro and anti-La antibody positive. She never required immunomodulatory treatment.
MRI-brain showed multiple T2-hyperintense lesions with surrounding oedema in the cerebellum, surrounding the fourth ventricle, and in the brainstem. The lesions showed marked ring-enhancement on post-gadolinium T1 sequences. Although the imaging appearances were suggestive of multiple cerebral metastases, extensive investigation for primary malignancy including CT-TAP, PET-CT, tumour markers and detailed skin examination, and lung biopsy was unyielding. Aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibody were negative. CSF had a white-cell-count of 149 and protein of 2.68g/l. A broad infective screen in CSF and serum was negative. Ultimately, brain biopsy of one of the cerebellar lesions revealed focal atrophy and hyalinisation, chronic T-lymphocyte and macrophage infiltrate of the leptomeninges and parenchyma, without diagnostic features of vasculitis or evidence of malignancy.
She was diagnosed with CNS Sjogren’s Syndrome and improved clinically following steroids with only a mild residual third nerve palsy. There was almost complete resolution radiologically on her 3-month follow-up imaging.