Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
School of Public Health, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Biomed Res Int. 2020 Aug 14;2020:2926419. doi: 10.1155/2020/2926419. eCollection 2020.
Hypertrophic pachymeningitis (HP) is generally regarded as a rare inflammatory disease, which results in a diffuse thickening of the dura mater. We retrospectively collected data from patients with HP.
A total of 16 patients with HP were included in our study. The clinical features, laboratory evaluation, imaging findings, treatment, and outcome were reviewed.
Of the 16 cases, half were male, with a mean age of 52.6 ± 13.2 years. The mean duration from onset to diagnosis was 8.6 months. The most frequent presenting symptoms in HP cases were a recurrently chronic headache (81.3%) and multiple cranial nerve injury (50%). Antineutrophil cytoplasmic antibody- (ANCA-) related HP was found in 5 cases and IgG4-related HP in 1 case. The intracranial pressure was elevated in 4 cases. The cerebrospinal fluid (CSF) had lymphocytosis in 5 cases and increased protein in 12 cases. Immunoglobulins (IgG, IgA, and IgM) and protein showed linear relationships in the CSF. On magnetic resonance imaging (MRI), localized or diffuse dura maters were thickened in all cases. HP combined with subacute subdural hemorrhage or hypertrophic spinal pachymeningitis was also observed in individual cases. Biopsy of the dura mater in one case showed amounts of inflammatory cells infiltrating, with an increased percentage of IgG4-positive plasma cells. Of all cases referring to glucocorticoid treatment, the symptoms have improved significantly in 10 cases. In other 6 cases, mycophenolate mofetil or azathioprine was added. All patients showed clinical improvement at the follow-up visits.
The clinical characters of HP are chronic onset, recurrently chronic headache, and multiple cranial nerves paralysis. Inflammatory changes in CSF caused by intrathecal synthesis of immunoglobulin, characteristic dural enhancement on MRI, and pathologic biopsy are all helpful for diagnosis. The addition of immunosuppressant, especially mycophenolate mofetil, is a good choice for steroid-resistance HP.
肥厚性硬脑膜炎（HP）一般被认为是一种罕见的炎症性疾病，导致硬脑膜弥漫性增厚。我们回顾性地收集了 HP 患者的数据。
本研究共纳入 16 例 HP 患者。回顾分析了其临床特征、实验室评估、影像学表现、治疗及转归。
16 例患者中，男性占一半，平均年龄为 52.6±13.2 岁。从发病到诊断的平均时间为 8.6 个月。HP 患者最常见的首发症状为反复发作的慢性头痛（81.3%）和多颅神经损伤（50%）。抗中性粒细胞胞质抗体（ANCA）相关 HP 发现 5 例，IgG4 相关 HP 发现 1 例。4 例颅内压升高。5 例脑脊液（CSF）中有淋巴细胞增多，12 例 CSF 蛋白增加。CSF 中的免疫球蛋白（IgG、IgA 和 IgM）和蛋白呈线性关系。磁共振成像（MRI）显示所有病例均有局限性或弥漫性硬脑膜增厚。个别病例还观察到 HP 合并亚急性硬膜下血肿或肥厚性脊髓硬脑膜炎。1 例硬脑膜活检显示有大量炎性细胞浸润，IgG4 阳性浆细胞比例增加。所有接受糖皮质激素治疗的病例中，10 例症状明显改善。另外 6 例加用霉酚酸酯或硫唑嘌呤。所有患者在随访时均显示出临床改善。
HP 的临床特征为慢性起病、反复发作性慢性头痛和多颅神经麻痹。CSF 中免疫球蛋白的鞘内合成引起的炎症改变、MRI 上特征性硬脑膜增强以及病理活检均有助于诊断。添加免疫抑制剂，特别是霉酚酸酯，是治疗激素抵抗性 HP 的良好选择。
