Masson C, Hénin D, Hauw J J, Rey A, Raverdy P, Masson M
Service de Neurologie, Hôpital Beaujon, Clichy, France.
Neurology. 1993 Jul;43(7):1329-34. doi: 10.1212/wnl.43.7.1329.
We report seven patients with cranial pachymeningitis of unknown origin in whom the main clinical features were headaches, ataxia, and cranial nerve palsies. CSF showed inflammatory changes. CT and MRI showed thickening of the falx and of the tentorium. The clinical course was chronic. Four patients improved with prednisolone but became steroid-dependent: in two cases, radiotherapy had no lasting improvement and in one, azathioprine permitted a reduction of the corticosteroids. Five patients had biopsy of the tentorium cerebelli or of the temporal dura mater. In two cases, autopsy revealed extensive pachymeningitis without parenchymal changes. In all instances, microscopic examination of the dura mater showed a cellular infiltrate of polymorphic cells; there were no epithelioid granulomas. Review of the literature discloses seven similar cases. We discuss the relationship of these lesions with inflammatory meningeal masses, the focal pachymeningitis of the Tolosa-Hunt syndrome, and multifocal fibrosis.
我们报告了7例病因不明的颅骨硬脑膜炎患者，其主要临床特征为头痛、共济失调和颅神经麻痹。脑脊液显示有炎症改变。CT和MRI显示大脑镰和小脑幕增厚。临床病程为慢性。4例患者使用泼尼松龙后病情改善，但出现了类固醇依赖：2例患者放疗后无持久改善，1例患者使用硫唑嘌呤后可减少皮质类固醇用量。5例患者对小脑幕或颞部硬脑膜进行了活检。2例患者尸检显示广泛硬脑膜炎，无实质改变。在所有病例中，硬脑膜的显微镜检查显示有多形细胞的细胞浸润；无上皮样肉芽肿。文献回顾发现7例类似病例。我们讨论了这些病变与炎性脑膜肿块、托洛萨-亨特综合征的局限性硬脑膜炎以及多灶性纤维化的关系。
