Sjogren’s myelitis presenting as hemicord syndrome

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.