Department of Neurology, The First Affiliated Hospital of China Medical University, Shenyang, China.
BMC Neurol. 2024 Aug 13;24(1):283. doi: 10.1186/s12883-024-03785-y.
Hypertrophic pachymeningitis (HP) is a rare disorder marked by thickening of the dura mater due to diverse etiologies. MPO-ANCA-positive HP represents a variant of AAV confined to the central nervous system, distinguished by the presence of serum MPO antibodies. Distinguishing HP triggered by MPO-ANCA from other causes can be challenging.In this study, we present two cases of MPO-ANCA-positive HP initially misdiagnosed as intracranial infections. Case 1 underwent surgery for chronic suppurative otitis media, with histopathological findings revealing inflammatory changes without definitive suppuration. He was presumed to have a secondary intracranial infection resulting from the surgery. However, his condition deteriorated despite two weeks of antibiotic and antiviral treatment. Case 2 presented with headache and was initially suspected of having intracranial Brucellosis given his serum Brucella positivity. Despite treatment for brucellosis, his symptoms persisted, and he developed visual and hearing impairments. Both patients were ultimately diagnosed with MPO-ANCA-positive HP, exhibiting serum MPO antibody positivity. Their symptoms showed improvement with glucocorticoid and immunosuppressive therapy.Based on these observations, we propose that MPO-ANCA-positive HP may initially present as intracranial infection. For HP patients presenting with headache, mastoiditis, otitis media, and visual loss, it is imperative to conduct ANCA antibody-related tests to enhance diagnostic precision.
肥厚性硬脑膜炎（Hypertrophic pachymeningitis，HP）是一种罕见疾病，其特征为硬脑膜因多种病因而增厚。抗髓过氧化物酶-抗中性粒细胞胞质抗体（Myeloperoxidase-antineutrophil cytoplasmic antibody，MPO-ANCA）阳性 HP 是局限于中枢神经系统的抗中性粒细胞胞质抗体相关性血管炎（Anti-neutrophil cytoplasmic antibody-associated vasculitis，AAV）的一种变体，其特征为血清 MPO 抗体阳性。区分 MPO-ANCA 阳性 HP 与其他病因具有一定挑战性。本研究报告了两例最初误诊为颅内感染的 MPO-ANCA 阳性 HP 病例。病例 1 因慢性化脓性中耳炎而行手术治疗，组织病理学检查发现存在炎症改变，但无明确的化脓灶。考虑到该患者为手术引起的继发性颅内感染。然而，尽管接受了两周的抗生素和抗病毒治疗，他的病情仍在恶化。病例 2 因头痛就诊，最初怀疑为颅内布氏杆菌病，因为其血清布氏杆菌阳性。尽管针对布氏杆菌病进行了治疗，但他的症状仍持续存在，并出现了视力和听力受损。两名患者最终均被诊断为 MPO-ANCA 阳性 HP，且血清 MPO 抗体阳性。他们的症状在接受糖皮质激素和免疫抑制剂治疗后得到改善。基于这些观察结果，我们提出 MPO-ANCA 阳性 HP 可能最初表现为颅内感染。对于以头痛、乳突炎、中耳炎和视力丧失为表现的 HP 患者，必须进行抗中性粒细胞胞质抗体相关检查以提高诊断准确性。
