Multiple Ring-Enhancing Cerebral Lesions in Central Nervous System Sjogren’s Syndrome
Grainne Mulkerrin1, Patrick Cullinane2, Sarah Mangan3, Alan Beausang4, Sean O'Riordan5
1Royal College of Physicians Ireland, 2SVUH, 3St Vincents University Hospital, 4Beaumont Hospital, 5St. Vincent's University Hosp
Objective:
NA 
Background:
Background: CNS-involvement of Sjogren’s Syndrome is rare. It can affect any part of the CNS but has a predilection for the spinal cord and optic nerves. Imaging would be consistent with demyelination, without specific features outside of what can be expected in other CNS demyelinating disorders.
Design/Methods:
NA 
Results:

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.

Conclusions:
Conclusion: This case highlights that Sjogren’s Syndrome should be considered as a potentially treatable differential diagnosis of multiple ring-enhancing brain lesions. Neuromyelitis optica and MOGAD should be carefully excluded; given the association with Sjogren’s Syndrome and similar imaging features, respectively.   
10.1212/WNL.0000000000212344
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.