Jimenez-Caballero P E, Diamantopoulos-Fernandez J, Camacho-Castaneda I
Hospital Virgen de la Salud, Complejo Hospitalario de Toledo, 45005 Toledo, Espana.
Rev Neurol. 2006;43(8):470-5.
Hypertrophic pachymeningitis is an infrequent disease that is characterised by inflammatory hypertrophy of the dura mater. There are cranial, spinal and craniospinal forms of the disease. They may be due to underlying infectious, autoimmune or neoplastic processes, although most of the cases reported in recent years have no base pathology and are known as idiopathic hypertrophic pachymeningitis. The ideal treatment is unknown, but most cases usually respond well to therapy with corticoids.
We report two cases of cranial forms, one idiopathic with typical clinical features consisting in cranial polyneuropathy and good response to corticoids, and another case secondary to rheumatoid arthritis that began with epileptic seizures. Both had a parenchymatous oedema. The two forms of spinal pachymeningitis presented as progressive paraparesis that evolved well after surgical removal of the lesion.
On observing pachymeningitis in neuroimaging tests it becomes necessary to conduct a comprehensive aetiological study in search of infectious, autoimmune and neoplastic diseases. In the cranial forms there may be parenchymatous oedema, which would explain the epileptic seizures and the cognitive deterioration of our patients. Response to corticoids is spectacular, although it is often necessary to continue to administer them for prolonged periods of time.
肥厚性硬脑膜炎是一种罕见疾病，其特征为硬脑膜的炎性肥厚。该疾病有颅型、脊髓型和颅脊髓型。它们可能由潜在的感染、自身免疫或肿瘤性病变引起，尽管近年来报道的大多数病例没有基础病理，被称为特发性肥厚性硬脑膜炎。理想的治疗方法尚不清楚，但大多数病例通常对皮质类固醇治疗反应良好。
我们报告两例颅型病例，一例为特发性，具有典型临床特征，包括颅多神经病且对皮质类固醇反应良好，另一例继发于类风湿性关节炎，以癫痫发作为首发症状。两例均有实质水肿。两种脊髓型硬脑膜炎均表现为进行性截瘫，病变手术切除后病情恢复良好。
在神经影像学检查中观察到硬脑膜炎时，有必要进行全面的病因学研究，以寻找感染性、自身免疫性和肿瘤性疾病。在颅型病例中可能存在实质水肿，这可以解释我们患者的癫痫发作和认知功能减退。对皮质类固醇的反应显著，尽管通常需要长时间持续给药。
