Zielke Tara, Kim Miri, Simon Joshua E, Borys Ewa, Prabhu Vikram C, Pappu Suguna
Pathology, Loyola University Medical Center, Maywood, Illinois.
Departments of Neurological Surgery and.
J Neurosurg Case Lessons. 2021 May 24;1(21):CASE20168. doi: 10.3171/CASE20168.
Hypertrophic cranial pachymeningitis is a rare inflammatory disorder characterized by thickening of the dura mater and multiple cranial neuropathies. Although an infectious etiology may be present, often no specific cause is discovered.
The authors described a 71-year-old man with progressive right eye vision loss, ptosis, and complete ophthalmoplegia with imaging findings suggestive of hypertrophic cranial pachymeningitis. Extensive studies, including cerebrospinal fluid studies, showed negative results. Blood serum, cell-free evaluation, and paraffin-embedded dural tissue testing had positive results for which allowed treatment tailored to the organism and a salutary clinical outcome.
The constellation of neurological and radiological findings may make a diagnosis difficult in an inflammatory setting. The most precise methodology for establishing a diagnosis involves sampling the dura and testing it for infectious pathology. However, if results are inconclusive, further cell-free serum sampling with next-generation sequencing is a viable option for identifying pathogens with infectious concerns. This case highlighted the importance of multimodality studies for identifying a targetable pathogen.
肥厚性硬脑膜炎是一种罕见的炎症性疾病，其特征为硬脑膜增厚和多发脑神经病变。虽然可能存在感染性病因，但通常无法发现具体病因。
作者描述了一名71岁男性，出现进行性右眼视力丧失、上睑下垂和完全性眼肌麻痹，影像学检查结果提示肥厚性硬脑膜炎。包括脑脊液检查在内的广泛检查结果均为阴性。血清、无细胞评估及石蜡包埋硬脑膜组织检测结果呈阳性，据此进行了针对性治疗，临床预后良好。
在炎症背景下，神经学和影像学检查结果可能难以做出诊断。最精确的诊断方法是对硬脑膜进行采样并检测其是否存在感染性病变。然而，如果结果不明确，进一步进行无细胞血清采样并结合下一代测序是识别有感染疑虑病原体的可行选择。该病例凸显了多模态研究对于识别可靶向病原体的重要性。
