Teaching NeuroImage: Sjögren Syndrome Presenting With Craniospinal Hypertrophic Pachymeningitis

A 66-year-old female patient presented with neck pain for 3 years, moderately severe headache for 1 year, and dry eyes and mouth for 6 months.Examination revealed restricted neck movements and a positive Schirmer test.MRI showed craniospinal hypertrophic pachymeningitis, causing cervicodorsal spinal cord compression (Figure).She underwent C7-T1 laminectomy with excision of the spinal dural thickening.Histopathology revealed fibrohyalinized tissue with a lymphoplasmacytic infiltrate, suggesting an inflammatory etiology.IgG4-related disease, sarcoidosis, granulomatosis with polyangiitis, tuberculosis, and fungal infection were excluded by unremarkable serum IgG4, ANCA, and ACE levels, normal CSF studies, and a normal CT of the chest, abdomen, and pelvis.A strongly positive anti-Ro-52 antibody and lip biopsy showing inflammatory lymphocytic exudate confirmed the diagnosis of Sjgren syndrome.She improved significantly on oral steroids and mycophenolate mofetil over 6 months.Although rare, Sjgren syndrome may present as hypertrophic