Neurol Neuroimmunol Neuroinflamm. 2021 Jun 2;8(5):e1022.doi: 10.1212/NXI.0000000000001022. Print 2021 Jul.

Spencer K Hutto ¹, Mary D Maher ², Eli M Miloslavsky ², Nagagopal Venna ²

Author information

• ¹From the Division of Neuroimmunology and Neuroinfectious Diseases (S.K.H., N.V.), Department of Neurology, Massachusetts General Hospital, Boston; Division of Neuroradiology (M.D.M), Department of Radiology, Massachusetts General Hospital, Boston; and Division of Rheumatology, Allergy and Immunology (E.M.M), Department of Medicine, Massachusetts General Hospital, Boston. shutto@mgh.harvard.edu.
• ²From the Division of Neuroimmunology and Neuroinfectious Diseases (S.K.H., N.V.), Department of Neurology, Massachusetts General Hospital, Boston; Division of Neuroradiology (M.D.M), Department of Radiology, Massachusetts General Hospital, Boston; and Division of Rheumatology, Allergy and Immunology (E.M.M), Department of Medicine, Massachusetts General Hospital, Boston.

Abstract

Objectives: To review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment.

Methods: We review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed.

Results: Only 2 cases of ulcerative colitis and 5 cases of RP were found in association with pachymeningitis; there were no cases in association with CD. Our patient presented with symptoms isolated to a steroid-responsive headache in the setting of normal neurologic and rheumatologic examinations. Her preceding history was notable for long-standing CD and increasingly active symptoms referable to RP. Focal nodular pachymeningitis was seen overlying the left hemisphere on brain MRI. An extensive serum and CSF workup and body fluorodeoxyglucose-PET scan failed to identify an alternative etiology beyond her underlying autoimmune inflammatory disorders. After adding prednisone and adalimumab to her preexisting treatment of methotrexate, she responded dramatically both clinically and radiographically.

Conclusions: Although exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.