Yao Yajun, Xu Yun, Li Xindi, Song Tian, Xu Wangshu, Duan Yunyun, Liu Yaou, Zhang Xinghu, Tian De-Cai
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China.
Departments of Radiology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China.
Mult Scler Relat Disord. 2022 Oct;66:104026. doi: 10.1016/j.msard.2022.104026. Epub 2022 Jul 5.
Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease that causes focal or diffuse thickening of the dura mater. However, longitudinal follow up studies are still lacking for these patients.
To investigate the clinical characteristics, neuroimaging findings, treatment response and outcome of IHP.
A retrospective case series of 30 patients admitted Beijing Tiantan Hospital were screened via Hospital Information System from January 1st, 2011, to January 31st, 2021. All patients' clinical symptoms, imaging, and treatment response were collected via a standardized form. We compared the effects of high-dose and low-dose corticosteroids on headache, impaired vision, and MRI remission during acute onset. The effects of different immunosuppressants on preventing relapses were also compared.
Headache (93.3%) and multiple cranial neuropathy (66.7%) were the most common symptoms of IHP. Cerebral spinal fluid test showed that protein levels were elevated in 17 (56.7%) patients, and white blood cells were increased in seven patients. MRI demonstrated that diffuse (60%) and focal (40%) enhancement occurred in the dura mater, especially in the tentorium cerebellum (80%). High-dose and low-dose corticosteroids reduced headache and dural enhancement during the acute phase. The high dose corticosteroid significantly relieved the headache than the low dose group (p = 0.041). Patients treated with mycophenolate mofetil and cyclophosphamide might achieve longer remission (months, p = 0.428).
Headache and multiple cranial neuropathy are the most common clinical manifestations of IHP. In this study, almost all patients had a good initial response to corticosteroid therapy during the acute phase. Mycophenolate mofetil and cyclosporine may be effective for preventing relapses.
特发性肥厚性硬脑膜炎（IHP）是一种罕见的炎症性疾病，可导致硬脑膜局灶性或弥漫性增厚。然而，对这些患者仍缺乏纵向随访研究。
探讨IHP的临床特征、神经影像学表现、治疗反应及预后。
通过医院信息系统对2011年1月1日至2021年1月31日在北京天坛医院住院的30例患者进行回顾性病例系列研究。通过标准化表格收集所有患者的临床症状、影像学检查及治疗反应。比较高剂量和低剂量皮质类固醇对急性发作期头痛、视力障碍和MRI缓解的影响。还比较了不同免疫抑制剂对预防复发的效果。
头痛（93.3%）和多发性颅神经病变（66.7%）是IHP最常见的症状。脑脊液检查显示，17例（56.7%）患者蛋白水平升高，7例患者白细胞增多。MRI显示硬脑膜出现弥漫性（60%）和局灶性（40%）强化，尤其是小脑幕（80%）。高剂量和低剂量皮质类固醇在急性期均可减轻头痛和硬脑膜强化。高剂量皮质类固醇比低剂量组更能显著缓解头痛（p = 0.041）。接受霉酚酸酯和环磷酰胺治疗的患者可能获得更长时间的缓解（月，p = 0.428）。
头痛和多发性颅神经病变是IHP最常见的临床表现。在本研究中，几乎所有患者在急性期对皮质类固醇治疗均有良好的初始反应。霉酚酸酯和环孢素可能对预防复发有效。
