BACKGROUND: Primary central nervous system vasculitis (PCNSV) is a rare and poorly described vascular inflammatory condition confined within the central nervous system (CNS). Typical presentations of PCNSV include headache, hemiparesis, cerebral edema, and altered cognition. Vasculitic processes in the spinal cord are exceedingly rare. Although intracranial vasculitis has been associated with certain systemic inflammatory disorders and lymphomas, the etiology of spinal cord vasculitis is unclear. We report the first case of a patient in whom spinal subdural hematomas were the first presenting signs of PCNSV. CASE DESCRIPTION: A 69-year-old woman presented with sudden-onset severe back pain and paraplegia following an acute hypertensive event. Multiple spinal subdural hematomas were noted on magnetic resonance imaging, and emergent laminectomies were performed for evacuation. Vasculitic abnormalities confined to the CNS were noted intraoperatively and on imaging, leading to the unique diagnosis. CONCLUSIONS: This previously unreported etiology of nontraumatic acute spinal subdural hematomas suggests that spinal cord vasculitis as part of PCNSV should be included in the differential diagnosis.
BACKGROUND:Primary central nervous system vasculitis (PCNSV) is a rare and poorly described vascular inflammatory condition confined within the central nervous system (CNS). Typical presentations of PCNSV include headache, hemiparesis, cerebral edema, and altered cognition. Vasculitic processes in the spinal cord are exceedingly rare. Although intracranial vasculitis has been associated with certain systemic inflammatory disorders and lymphomas, the etiology of spinal cord vasculitis is unclear. We report the first case of a patient in whom spinal subdural hematomas were the first presenting signs of PCNSV. CASE DESCRIPTION: A 69-year-old woman presented with sudden-onset severe back pain and paraplegia following an acute hypertensive event. Multiple spinal subdural hematomas were noted on magnetic resonance imaging, and emergent laminectomies were performed for evacuation. Vasculitic abnormalities confined to the CNS were noted intraoperatively and on imaging, leading to the unique diagnosis. CONCLUSIONS: This previously unreported etiology of nontraumatic acute spinal subdural hematomas suggests that spinal cord vasculitis as part of PCNSV should be included in the differential diagnosis.