Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine.
Department of Neurology, Tohoku University Graduate School of Medicine.
Tohoku J Exp Med. 2022 Mar;256(3):241-247. doi: 10.1620/tjem.256.241.
Hypertrophic pachymeningitis (HP) presents with thickening of the dura mater in the cerebrum and spine, and its symptoms vary depending on the affected location. The association of HP with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recognized, and most cases are complicated by granulomatosis with polyangiitis. We report the case of a 47-year-old man who presented with HP upon relapse of eosinophilic granulomatosis with polyangiitis (EGPA), with literature review. He presented with disturbance of consciousness, and magnetic resonance imaging (MRI) revealed thickening of the dura mater around the left parietal lobe. Although myeloperoxidase (MPO)-ANCA was positive on EGPA diagnosis, the elevation of MPO-ANCA was not documented at the onset of HP. Brain perfusion scintigraphy showed an increase in blood flow in the left parietal lobe and temporal lobe, and electroencephalogram (EEG) revealed slow waves in the left parietal lobe. He was treated with a high dose of corticosteroid and rituximab, and the slow waves on EEG and brain perfusion were normalized. Although the most frequent symptom of HP is headache, disturbance of consciousness can be the manifestation of HP, and inflammation of HP could affect the cerebral parenchyma, which can be documented as abnormal EEG and perfusion scintigraphy. Literature review revealed that most of the HP in EGPA developed when EGPA relapsed, and was observed in patients with MPO-ANCA positivity. HP develops without evidence of other clinical features of EGPA; therefore, adequate imaging, including contrast-enhanced MRI, is necessary. Rituximab may be effective for treating HP complicated with EGPA.
肥厚性硬脑膜炎（HP）表现为大脑和脊柱脑膜增厚，其症状因受影响部位而异。现已认识到 HP 与抗中性粒细胞胞质抗体（ANCA）相关性血管炎有关，大多数病例并发肉芽肿性多血管炎。我们报告了一例 47 岁男性，在嗜酸性肉芽肿性多血管炎（EGPA）复发时出现 HP，并进行了文献复习。他表现为意识障碍，磁共振成像（MRI）显示左顶叶周围脑膜增厚。虽然 EGPA 诊断时 MPO-ANCA 阳性，但 HP 发病时未记录到 MPO-ANCA 升高。脑灌注闪烁显像显示左顶叶和颞叶血流量增加，脑电图（EEG）显示左顶叶慢波。他接受了大剂量皮质类固醇和利妥昔单抗治疗，EEG 和脑灌注的慢波恢复正常。虽然 HP 最常见的症状是头痛，但意识障碍也可能是 HP 的表现，HP 的炎症可能影响脑实质，表现为异常 EEG 和灌注闪烁显像。文献复习显示，EGPA 中大多数 HP 在 EGPA 复发时发生，且见于 MPO-ANCA 阳性患者。HP 在无 EGPA 其他临床特征的情况下发生；因此，需要进行充分的影像学检查，包括增强 MRI。利妥昔单抗可能对治疗伴有 EGPA 的 HP 有效。
