Hypertrophic spinal pachymeningitis caused by ANCA-associated vasculitis revealed by 18F-FDG PET/CT

Abstract

Rationale:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT.Patient concerns:A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago.Diagnosis:Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis.Interventions:The patient was treated with a higher dose of prednisone and cyclophosphamide.Outcomes:After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord.Lessons:18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.