Azeroual A, Benomar A, Harmouche H, Daoudi N, Benhaddou E H Ait, Yahyaoui M, Maaouni A
Service de Neurologie B et de Neurogénétique, hôpital des spécialités de Rabat, Maroc; Service de Médecine Interne, Centre Hospitalier Ibn Sina, Rabat, Maroc.
Rev Neurol (Paris). 2007 Dec;163(12):1242-5. doi: 10.1016/S0035-3787(07)78411-4.
Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.
原发性干燥综合征是一种伴有多种腺体外并发症的自身免疫性外分泌病。20%的病例有中枢神经表现。小脑综合征在干燥综合征中不常见,但可在诊断后出现,或很少作为该病的首发症状。我们报告一例54岁男性患者,其出现伴有口腔干燥和干眼的小脑综合征。经副唾液腺活检确诊为原发性干燥综合征。患者接受了口服和静脉注射皮质类固醇治疗,并每月联合使用环磷酰胺。结果良好。