Sjogren's myelitis presenting as hemicord syndrome

Lee, Andrew; Pande, Ira; Gran, Bruno

Abstract

We present a case where a 63-year-old right-handed man who presented with a 6-month history of progressive asymmetrical sensorimotor symptoms in lower limbs. This was associated with concomitant rash on the lower limbs, and mild sicca symptoms. MRI spine showed focal T2 hyperintensity in the left hemicord at C3-4 level. Skin biopsy of the rash revealed urticarial vasculitis, and lip biopsy revealed lymphocytic sialadenitis. Initial anti-Ro antibody was negative, but subsequent Ro52 antibody testing returned positive. There was also matched serum and cerebrospinal fluid oligoclonal bands. He was subsequently diagnosed as Sjogren's myelitis and treated with intravenous methylprednisolone, then transitioned to a steroid sparing agent. This case highlights the difficulties in reaching a rheumatological diagnosis in the early stages with typical negative antibodies, and shows a rare neurological manifestation of a systemic rheumatological condition. [Abstract copyright: © Royal College of Physicians 2023. All rights reserved.]

• Journal Article Type: Article
• Acceptance Date: Jul 1, 2023
• Online Publication Date: Jul 31, 2023
• Publication Date: 2023-07
• Deposit Date: Jun 15, 2024
• Publicly Available Date: Jun 19, 2024
• Journal: Clinical Medicine
• Print ISSN: 1470-2118
• Electronic ISSN: 1473-4893
• Publisher: Royal College of Physicians
• Peer Reviewed: Peer Reviewed
• Volume: 23
• Issue: 4
• Pages: 423-424
• DOI: https://doi.org/10.7861/clinmed.2023-0158
• Keywords: Sjogren's syndrome, hemicord syndrome, myelitis
• Public URL: https://nottingham-repository.worktribe.com/output/24414939
• Publisher URL: https://www.sciencedirect.com/science/article/pii/S1470211824045949?via%3Dihub