Department of Neurology, Kokura Memorial Hospital, Kitakyushu, Japan
Department of Neurosurgery, Kokura Memorial Hospital, Kitakyushu, Japan.
BMJ Case Rep. 2021 Sep 20;14(9):e243395. doi: 10.1136/bcr-2021-243395.
The most common neurological manifestation of eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is mononeuritis multiplex caused by small-vessel vasculitis. In contrast, central nervous system involvement is rare. Among EGPA-associated central nervous system disorders, there are only a few reported cases of hypertrophic pachymeningitis (HP). Here, we report a patient with EGPA with headache and ophthalmoplegia who presented with HP and had a dural biopsy. The biopsy specimen showed lymphocytic inflammatory cell infiltration without EGPA-specific findings, that is, eosinophilic infiltration, granuloma or angiitis. To the best of our knowledge, there are no previous reports of EGPA-associated HP pathology. Here, we report the first case presentation of a patient with EGPA-associated HP with pathological findings.
嗜酸性肉芽肿性多血管炎（EGPA），以前称为变应性肉芽肿性血管炎，最常见的神经系统表现是小血管炎引起的多发性单神经炎。相比之下，中枢神经系统受累较为罕见。在 EGPA 相关的中枢神经系统疾病中，仅有少数肥厚性硬脑膜炎（HP）的病例报道。在此，我们报告一例 EGPA 伴头痛和眼肌麻痹的患者，表现为 HP，并进行了硬脑膜活检。活检标本显示淋巴细胞炎性细胞浸润，无 EGPA 特异性表现，即嗜酸性粒细胞浸润、肉芽肿或血管炎。据我们所知，以前没有 EGPA 相关 HP 病理学的报道。在此，我们报告首例 EGPA 相关 HP 的患者病例，并提供了病理学发现。
