El Aoud S, Frikha F, Ben Salah R, Snoussi M, Loukil H, Bahloul Z
Department of Internal Medicine, Hedi Chaker Hospital.
Reumatismo. 2013 Dec 18;65(5):248-52. doi: 10.4081/reumatismo.2013.248.
Pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. Here, we report the case of a 60-year old man who had a progressive sensorineural hearing loss, visual disturbance and others cranial nerve involvement with an accompanying headache over several months. Brain magnetic resonance imaging showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody titers. After empirical steroid and cyclophosphamide therapy, the neurological problems were partially improved. Therefore, in the case of atypical sensorineural hearing loss accompanied by cranial nerve palsy or headache, pachymeningitis should be considered in the differential diagnosis.
硬脑膜炎是一种进展性疾病，由于炎症、肿瘤或自身免疫性疾病导致硬脑膜弥漫性增厚，但大多数病例为特发性。在此，我们报告一例60岁男性患者，该患者在数月内出现进行性感音神经性听力损失、视觉障碍及其他颅神经受累，并伴有头痛。脑部磁共振成像显示硬脑膜弥漫性增厚，给予钆剂后强化明显，这与硬脑膜炎相符。基于血清髓过氧化物酶-抗中性粒细胞胞浆抗体滴度升高，推测其与自身免疫发病机制有关。经过经验性的类固醇和环磷酰胺治疗后，神经问题得到部分改善。因此，对于伴有颅神经麻痹或头痛的非典型感音神经性听力损失病例，在鉴别诊断时应考虑硬脑膜炎。
