IgG4-related pachymeningitis and mastoiditis, associated with cerebral venous thrombosis: A case report

• Published in: Journal of neuroimmunology 2021-11, Vol.360, p.577717-577717, Article 577717
• Main Authors: Nilles, Christelle, Poillon, Guillaume, Deschamps, Lydia, Daval, Mary, Diallo, Ibrahima Mariam, Deschamps, Romain, Boudot de la Motte, Marine
• Format: Article
• Language: English
• Subjects: Abducens Nerve Diseases - etiology; Adrenal Cortex Hormones - therapeutic use; Adult; Cerebral venous thrombosis; Classification criteria; Clinico-pathologic confirmation; Dabigatran - therapeutic use; Female; Humans; IgG4-related disease; Immunoglobulin G4-Related Disease - blood; Immunoglobulin G4-Related Disease - complications; Inflammatory pseudotumor; Lateral Sinus Thrombosis - etiology; Lung - diagnostic imaging; Magnetic Resonance Imaging; Mastoidectomy; Mastoiditis - diagnostic imaging; Mastoiditis - drug therapy; Mastoiditis - etiology; Mastoiditis - surgery; Meningitis - diagnostic imaging; Meningitis - drug therapy; Meningitis - etiology; Neuroimaging; Rituximab - therapeutic use; Skull base; Thrombophilia - drug therapy; Tomography, X-Ray Computed; Venous Thrombosis - etiology
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2021.577717

IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition; this can be a challenging diagnosis that requires clinico-pathologic correlation. We report a young woman, presenting with cranial nerve palsy. The work-up revealed pachymeningitis, cerebral venous thrombosis (CVT), and a destructive lesion in the mastoid. We diagnosed IgG4-RD through mastoidectomy. Thus, a biopsy of asymptomatic, infrequently affected organs, like the mastoid, can meet all histopathological criteria. In neuro-meningeal presentations, CVT may be secondary to the local inflammatory environment of pachymeningitis. Since our patient had a deep vein thrombosis one year prior, we discuss a possible higher risk of thrombosis in IgG4-RD patients. [Display omitted] •IgG4-RD is a multisystem disorder that requires clinico-pathologic confirmation.•IgG4-RD's diagnosis may be aided by biopsy of clinically uninvolved areas.•In IgG4-RD, cerebral venous thrombosis may be secondary to the local inflammation.