Roongpiboonsopit Duangnapa, Phanthumchinda Kammant
J Med Assoc Thai. 2014 Apr;97(4):374-80.
Idiopathic Hypertrophic Pachymeningitis (IHP) is a rare chronic inflammatory disorder of the dura. Classic clinical symptoms include headaches and cranial neuropathy. Because of scarce clinical data from Thailand, the present study aimed to determine the clinical features, neuroimaging findings, natural histories, therapeutic options, and outcomes for treatment of IHP in a tertiary care center.
A retrospective study was carried out on all adult IHP patients hospitalized at King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between January 2000 and November 2011. Diagnostic criteria included 1) clinical symptom compatibility with IHP, 2) neuroimaging to reveal enhanced hypertrophic dura compatible with clinical syndrome, and 3) ruled out secondary causes of LHP, using appropriate clinical profiles and investigations including tissue biopsy.
Thirty-two patients were enrolled with 21 females and 11 males, mean age of 49.03 +/- 16.12 years. The two most common symptoms were headache (93.8%) and diplopia (43.8%). The most common neurological finding was multiple cranial neuropathies (84.4%). Cranial nerve III was affected in 56.3% of the patients, followed by other cranial nerves including CN VI, IV, V and II. Headache without a neurological deficit was observed in 12.5% of the cases. Focal and diffuse enhanced thickening of the dura were observed in 96.9% and 3.1% of the cases respectively. Focal thickening in the supratentorium included the cavernous sinus, orbital apex, sphenoid wing, and superior orbital fissure. Focal thickening in the infratentorium included the falx cerebelli, the dura at the base of the skull, Meckel's cave, and foramen magnum. CSF examination showed lymphocyte pleocytosis with a slight increase in CSF proteins. Headache subsided in all of the patients after treatment with corticosteroid In relapsing and recurrent patients, a combined treatment of steroids and azathioprine was prescribed. With the combined treatment, clinical complete recovery, relapsing and recurrence were detected in 40%, 40% and 20% of the cases respectively. All relapsing and recurrence were due to rapid tapering of for early discontinuation of the steroids treatment. Only one patient had a spontaneous remission.
The most common clinical manifestations of IHP were headache and multiple cranial nerve involvement. Almost all of the patients had good initial response to steroid therapy. Relapse or recurrence was usually caused by rapid tapering off or early discontinuation of the steroid treatment. Long-term treatment with combined immunosuppression may be necessary in some cases.
特发性肥厚性硬脑膜炎（IHP）是一种罕见的硬脑膜慢性炎症性疾病。典型的临床症状包括头痛和颅神经病变。由于泰国的临床数据稀缺，本研究旨在确定一家三级医疗中心中IHP患者的临床特征、神经影像学表现、自然病程、治疗选择及治疗结果。
对2000年1月至2011年11月期间在泰国曼谷朱拉隆功国王纪念医院住院的所有成年IHP患者进行回顾性研究。诊断标准包括：1）临床症状与IHP相符；2）神经影像学显示与临床综合征相符的硬脑膜肥厚强化；3）通过适当的临床资料和检查（包括组织活检）排除LHP的继发原因。
共纳入32例患者，其中女性21例，男性11例，平均年龄49.03±16.12岁。最常见的两种症状是头痛（93.8%）和复视（43.8%）。最常见的神经系统表现是多发性颅神经病变（84.4%）。56.3%的患者动眼神经（颅神经III）受累，其次是其他颅神经，包括展神经（CN VI）、滑车神经（IV）、三叉神经（V）和视神经（II）。12.5%的病例出现无神经功能缺损的头痛。分别有96.9%和3.1%的病例观察到硬脑膜局灶性和弥漫性强化增厚。幕上的局灶性增厚包括海绵窦、眶尖、蝶骨翼和眶上裂。幕下的局灶性增厚包括小脑镰、颅底硬脑膜、梅克尔腔和枕骨大孔。脑脊液检查显示淋巴细胞增多，脑脊液蛋白略有升高。所有患者经皮质类固醇治疗后头痛缓解。对于复发和复发病例，采用类固醇和硫唑嘌呤联合治疗。联合治疗后，分别有40%、40%和20%的病例实现临床完全缓解、复发和再复发。所有复发和再复发均归因于类固醇治疗快速减量或过早停药。只有1例患者自发缓解。
IHP最常见的临床表现是头痛和多发性颅神经受累。几乎所有患者对类固醇治疗初始反应良好。复发或再复发通常是由于类固醇治疗快速减量或过早停药所致。在某些情况下可能需要长期联合免疫抑制治疗。
