Hamilton S R, Smith C H, Lessell S
Division of Neurology, University of Washington, Seattle.
J Clin Neuroophthalmol. 1993 Jun;13(2):127-34.
We evaluated 3 patients with biopsy-proven hypertrophic cranial pachymeningitis apparently unrelated to any systemic disease. Each patient had chronic headache, cranial neuropathy, an elevated ESR, and a mild CSF pleocytosis. Neuro-ophthalmic findings included bilateral sixth nerve palsies in two patients and the third had bilateral optic neuropathies. MR imaging revealed thickened dura that enhanced with Gd-DTPA administration. Histologic examination showed thickened, fibrotic dura with a sterile, chronic, nongranulomatous inflammation. The response to treatment was variable with corticosteroids, immunosuppressive drugs, or radiation. The distinctive MR appearance should help physicians recognize this rarely reported disease.
我们评估了3例经活检证实为肥厚性硬脑膜炎且显然与任何全身性疾病无关的患者。每位患者均有慢性头痛、颅神经病变、血沉升高及脑脊液轻度细胞增多。神经眼科检查结果包括2例患者出现双侧展神经麻痹，第3例患者出现双侧视神经病变。磁共振成像显示硬脑膜增厚，注射钆喷酸葡胺后强化。组织学检查显示硬脑膜增厚、纤维化，伴有无菌性、慢性、非肉芽肿性炎症。使用皮质类固醇、免疫抑制药物或放疗治疗的反应各不相同。这种独特的磁共振表现应有助于医生识别这种鲜有报道的疾病。
