Department of Neurology, University of Michigan, 1914 Taubman Center, 1500 E. Medical Center Dr. SPC 5316, Ann Arbor, MI, 48109-5316, USA.
Department of Anesthesiology, University of Michigan, Ann Arbor, MI, USA.
Curr Pain Headache Rep. 2020 Aug 17;24(10):57. doi: 10.1007/s11916-020-00893-5.
We aim to review idiopathic hypertrophic cranial pachymeninigitis (IHCP), describe common head pain patterns and features associated with the disorder, suggest potential classification of head pain syndromes based on the recently published International Classification of Headache Disorders-3, explore pathophysiology found to be associated with cases of IHCP, and indicate common treatment for the disorder.
It is suggested that a subset of IHCP is an IgG4-related autoimmune disorder. Patients with IHCP were found to have elevated cerebrospinal fluid (CSF) protein and lymphocytic pleocytosis. Corticosteroids are a mainstay of treatment. Other immunosuppressive agents and steroid sparing agents as add-on therapy may have utility in the treatment of cases refractory to corticosteroids alone. Clinical manifestations of IHCP depend upon the location of the inflammatory lesions and compression of the adjacent nervous system structures. Headache and loss of cranial nerve function were the most common presenting features of hypertrophic cranial pachymeninigitis. Several headache diagnoses may result from IHCP. Gadolinium-enhanced MRI is the standard imaging modality for diagnosing. Although the pathophysiology is poorly understood, many cases of hypertrophic pachymeninigitis (HP) are thought to be closely related to inflammatory disorders. Cases of HP previously thought to be idiopathic may have IgG4 pathophysiology. CSF and serological studies are helpful. Treatment involves immunosuppressive agents. Advancement in neuroimaging, assays, tests, and further delineation of inflammatory disorders affecting the nervous system may provide further insight to the etiology of cases of HP previously considered and diagnosed as idiopathic.
我们旨在回顾特发性肥厚性颅底脑膜炎（IHCP），描述与该疾病相关的常见头痛模式和特征，根据最近发布的《国际头痛疾病分类-3》建议基于头痛综合征的潜在分类，探讨与 IHCP 相关的发现的病理生理学，并指出该疾病的常见治疗方法。
提示 IHCP 的一个亚组是 IgG4 相关自身免疫性疾病。IHCP 患者的脑脊液（CSF）蛋白和淋巴细胞增多。皮质类固醇是治疗的主要药物。其他免疫抑制剂和类固醇节约剂作为附加治疗可能对单独使用皮质类固醇治疗无效的病例有用。IHCP 的临床表现取决于炎症病变的位置和邻近神经系统结构的受压情况。头痛和颅神经功能丧失是肥厚性颅底脑膜炎的最常见表现。几种头痛诊断可能与 IHCP 有关。钆增强 MRI 是诊断的标准影像学方法。尽管病理生理学尚不清楚，但许多肥厚性脑膜炎（HP）病例被认为与炎症性疾病密切相关。以前被认为是特发性的 HP 病例可能具有 IgG4 病理生理学。CSF 和血清学研究有帮助。治疗包括免疫抑制剂。神经影像学、检测、试验的进展以及对影响神经系统的炎症性疾病的进一步描述可能会进一步了解以前被认为是特发性的 HP 病例的病因。
