Purpose of Review

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.

Recent Findings

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.

Summary

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.

中文翻译：

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头痛医生的特发性肥厚性颅脑膜炎的最新进展。

审查目的

我们旨在审查特发性肥厚性颅脑膜炎（IHCP），描述常见的头部疼痛模式和与疾病相关的特征，根据最近发布的《国际头痛分类3》建议对头痛症状进行潜在分类，探讨与之相关的病理生理学并伴有IHCP病例，并指出该疾病的常见治疗方法。

最近的发现

提示IHCP的一个子集是IgG4相关的自身免疫性疾病。发现患有IHCP的患者脑脊液（CSF）蛋白升高和淋巴细胞性细胞增多。皮质类固醇是治疗的支柱。其他的免疫抑制剂和类固醇保护剂作为附加疗法可能在治疗仅对皮质类固醇难治的病例中有用。

概要

IHCP的临床表现取决于炎性病变的位置和相邻神经系统结构的压迫。头痛和颅神经功能丧失是肥厚性颅脑膜炎的最常见表现。IHCP可能导致几种头痛诊断。d增强MRI是诊断的标准成像方式。尽管对病理生理学了解甚少，但许多肥厚性臀肌炎（HP）病例被认为与炎症性疾病密切相关。先前被认为是特发性的HP病例可能具有IgG4病理生理学。脑脊液和血清学研究是有帮助的。治疗涉及免疫抑制剂。神经影像学，测定，测试，