Service de Neurologie, Hôpital Fondation Adolphe de Rothschild, Paris, France.
Service de Radiologie, Hôpital Fondation Adolphe de Rothschild, Paris, France.
J Neuroimmunol. 2021 Nov 15;360:577717. doi: 10.1016/j.jneuroim.2021.577717. Epub 2021 Sep 10.
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.
IgG4 相关疾病（IgG4-RD）是一种多系统纤维炎症性疾病；这是一种具有挑战性的诊断，需要临床病理相关性。我们报告了一位年轻女性，表现为颅神经麻痹。检查发现硬脑膜增厚、脑静脉血栓形成（CVT）和乳突破坏性病变。我们通过乳突切除术诊断 IgG4-RD。因此，对无症状、很少受影响的器官（如乳突）进行活检可以满足所有组织病理学标准。在神经脑膜表现中，CVT 可能继发于硬脑膜增厚的局部炎症环境。由于我们的患者在一年前有深静脉血栓形成，我们讨论了 IgG4-RD 患者血栓形成的风险可能更高。
