Ghilain S, Delreux V, Kevers L, Sindic C J, Mathurin P, Laterre E C
Cliniques Saint-Luc (UCL), Bruxelles.
Acta Neurol Belg. 1988 Mar-Apr;88(2):91-100.
Otitis media in a 43 year-old male patient was followed by signs of chronic meningitis with persistent headaches, moderate pleiomorphic lymphocytosis of CSF without hypoglycorrhachia and unilateral impairment of cranial nerves XI to II. These signs were accompanied by widespread inflammation with general deterioration of the patient's condition. After a time the tentorium of cerebellum showed thickening and significant contrast uptake in conjunction with necrotizing granulomatous inflammation, indicating tentorial pachymeningitis. Swift and complete remission was obtained with a corticoid-cyclophosphamide combination. After reviewing the other possible causes of chronic meningitis, the authors proposed a diagnosis of Wegener's disease of auricular origin in view of the compatibility of all the data. A few cases of cranial pachymeningitis of indeterminate origin are described in the literature. Only this patient was treated with a corticoid-cyclophosphamide combination. The remarkable efficacy of this double therapy confirms the diagnostic hypothesis of Wegener's granulomatosis.
一名43岁男性患者患中耳炎后，出现慢性脑膜炎症状，伴有持续性头痛、脑脊液中度多形性淋巴细胞增多但无脑脊液低糖、以及第XI至II对颅神经单侧受损。这些症状伴有广泛炎症，患者病情总体恶化。一段时间后，小脑幕增厚并伴有明显的造影剂摄取，同时存在坏死性肉芽肿性炎症，提示小脑幕硬脑膜炎。使用皮质类固醇 - 环磷酰胺联合治疗后迅速实现了完全缓解。在回顾了慢性脑膜炎的其他可能病因后，鉴于所有数据的一致性，作者提出了耳源性韦格纳病的诊断。文献中描述了几例病因不明的颅硬脑膜炎病例。只有该患者接受了皮质类固醇 - 环磷酰胺联合治疗。这种双重疗法的显著疗效证实了韦格纳肉芽肿病的诊断假设。
