Hatano N, Behari S, Nagatani T, Kimura M, Ooka K, Saito K, Yoshida J
Department of Neurosurgery, Nagoya University School of Medicine, Japan.
Neurosurgery. 1999 Dec;45(6):1336-42; discussion 1342-4. doi: 10.1097/00006123-199912000-00014.
Idiopathic hypertrophic cranial pachymeningitis is a rare disease, of undetermined pathogenesis, that is characterized by inflammation and fibrosis of the dura mater.
We encountered six patients with idiopathic hypertrophic cranial pachymeningitis and analyzed their clinical presentations, radiological findings, and treatment.
In the six patients, the main manifestations were cranial nerve palsies and headache. Three associations were present, namely optic neuropathy, Tolosa-Hunt syndrome, and diabetes insipidus. Gadolinium-enhanced magnetic resonance imaging was diagnostic, showing intense dural enhancement in a linear or nodular pattern. The responses to corticosteroid therapy were better for patients who exhibited linear, rather than nodular, dural enhancement. For one patient, surgical decompression of the superior orbital fissure provided lasting relief. The course of the disease followed one of three patterns, i.e., sustained remission, relapse with corticosteroid independence, or relapse with corticosteroid dependence. Pulse corticosteroid therapy provided significant relief, while reducing the daily corticosteroid requirement and avoiding side effects, for a corticosteroid-dependent relapsing patient.
Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression.
特发性肥厚性硬脑膜炎是一种罕见疾病，发病机制不明，其特征为硬脑膜炎症和纤维化。
我们收治了6例特发性肥厚性硬脑膜炎患者，并分析了他们的临床表现、影像学检查结果及治疗情况。
6例患者的主要表现为脑神经麻痹和头痛。存在3种关联情况，即视神经病变、托洛萨-亨特综合征和尿崩症。钆增强磁共振成像具有诊断价值，显示硬脑膜呈线状或结节状强化。对于硬脑膜呈线状而非结节状强化的患者，皮质类固醇治疗反应更佳。1例患者行眶上裂减压手术获得持久缓解。疾病进程呈现3种模式之一，即持续缓解、不依赖皮质类固醇复发或依赖皮质类固醇复发。对于依赖皮质类固醇复发的患者，脉冲皮质类固醇治疗显著缓解了症状，同时减少了每日皮质类固醇用量并避免了副作用。
特发性肥厚性硬脑膜炎临床病程多样。在疾病活动期，通过每日给予皮质类固醇、脉冲皮质类固醇治疗或手术减压来预防不可逆性脑神经病变很重要。
