PDF(Pubmed)
Abstract：
BACKGROUND: Idiopathic hypertrophic pachymeningitis (IHP) is a rare neurological disorder without a definite etiology. Diagnosis is mainly based on exclusion of other etiologies.
METHODS: A 41-year-old male patient presented with insidious onset headache of 3-month duration.
METHODS: Contrast-enhanced brain magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement over bilateral cerebral hemispheres and the tentorium cerebelli. Lumbar puncture showed increased pressure, lymphocytic pleocytosis, and elevated protein level with normal glucose concentration. Blood tests detected elevated erythrocyte sedimentation rate (ESR) and C-reactive protein. Pathological examination of the dura mater from the right frontal convexity disclosed coarse collagenous deposition with focal lymphoid aggregation. After malignancy and infectious etiologies were excluded, a diagnosis of IHP was made.
METHODS: Oral prednisolone and azathioprine followed by methotrexate were administered.
RESULTS: During the 7-year follow-up period, although the patient was not totally headache-free, medical therapy significantly reduced the severity of headache. Follow-up MRI studies showed a reduction in meningeal enhancement and serial ESR measurements revealed a trend of improvement.
CONCLUSIONS: Methotrexate therapy may be considered in cases of steroid-resistant IHP. In addition to clinical evaluation, serial ESR testing may be considered to guide the treatment strategy and assess the response to therapy.
METHODS: A 41-year-old male patient presented with insidious onset headache of 3-month duration.
METHODS: Contrast-enhanced brain magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement over bilateral cerebral hemispheres and the tentorium cerebelli. Lumbar puncture showed increased pressure, lymphocytic pleocytosis, and elevated protein level with normal glucose concentration. Blood tests detected elevated erythrocyte sedimentation rate (ESR) and C-reactive protein. Pathological examination of the dura mater from the right frontal convexity disclosed coarse collagenous deposition with focal lymphoid aggregation. After malignancy and infectious etiologies were excluded, a diagnosis of IHP was made.
METHODS: Oral prednisolone and azathioprine followed by methotrexate were administered.
RESULTS: During the 7-year follow-up period, although the patient was not totally headache-free, medical therapy significantly reduced the severity of headache. Follow-up MRI studies showed a reduction in meningeal enhancement and serial ESR measurements revealed a trend of improvement.
CONCLUSIONS: Methotrexate therapy may be considered in cases of steroid-resistant IHP. In addition to clinical evaluation, serial ESR testing may be considered to guide the treatment strategy and assess the response to therapy.
摘要：
背景：特发性肥厚性硬脑膜炎（IHP）是一种罕见的神经系统疾病，没有明确的病因。诊断主要基于排除其他病因。
方法：一名41岁男性患者表现为持续3个月的隐匿性头痛。
方法：对比增强脑磁共振成像（MRI）显示双侧大脑半球和小脑幕的弥漫性硬脑膜增强。腰椎穿刺显示压力增加,淋巴细胞增多症，和升高的蛋白质水平与正常的葡萄糖浓度。血液检查发现红细胞沉降率（ESR）和C反应蛋白升高。从右额叶凸面对硬脑膜进行的病理检查显示，粗糙的胶原沉积具有局灶性淋巴聚集。在排除恶性肿瘤和感染性病因后，进行了IHP诊断。
方法：口服泼尼松龙和硫唑嘌呤，然后甲氨蝶呤。
结果：在7年的随访期间，虽然病人并非完全没有头痛,药物治疗可显着降低头痛的严重程度。后续的MRI研究显示脑膜增强减少，连续的ESR测量显示出改善的趋势。
结论：在激素抵抗的IHP病例中可以考虑甲氨蝶呤治疗。除了临床评估，可考虑进行系列ESR检测以指导治疗策略和评估治疗反应.
方法：一名41岁男性患者表现为持续3个月的隐匿性头痛。
方法：对比增强脑磁共振成像（MRI）显示双侧大脑半球和小脑幕的弥漫性硬脑膜增强。腰椎穿刺显示压力增加,淋巴细胞增多症，和升高的蛋白质水平与正常的葡萄糖浓度。血液检查发现红细胞沉降率（ESR）和C反应蛋白升高。从右额叶凸面对硬脑膜进行的病理检查显示，粗糙的胶原沉积具有局灶性淋巴聚集。在排除恶性肿瘤和感染性病因后，进行了IHP诊断。
方法：口服泼尼松龙和硫唑嘌呤，然后甲氨蝶呤。
结果：在7年的随访期间，虽然病人并非完全没有头痛,药物治疗可显着降低头痛的严重程度。后续的MRI研究显示脑膜增强减少，连续的ESR测量显示出改善的趋势。
结论：在激素抵抗的IHP病例中可以考虑甲氨蝶呤治疗。除了临床评估，可考虑进行系列ESR检测以指导治疗策略和评估治疗反应.
