Collard M, Sellal F, Hirsch E, Mutschler V, Marescaux C
Clinique Neurologique, Strasbourg.
Rev Neurol (Paris). 1991;147(5):403-5.
A 33 year-old Sephardic Jewish man with familial mediterranean fever (FMF), presented during a 7 year period, 6 episodes of aseptic meningitis, improving within less than 24 h after spinal tap. Cerebrospinal fluid analysis showed a mixed leucocytic pleocytosis ranging from 100 to 1,000 cell/mm3. Spinal fluid cultures for bacteria, viruses and viral antibodies were always negative. Our case supports other reports showing that recurrent aseptic meningitis, although rare, may occur in FMF. It usually responds to treatment with colchicine, like other manifestations of the disease. FMF meningitis has been compared to Mollaret's meningitis whose cause is undetermined. However, Mollaret's meningitis, unlike FMF, is sporadic and ubiquitous, is not transmitted genetically and affects men and women equally. Moreover, in Mollaret's meningitis transient neurological abnormalities, such as signs of encephalitis have often been reported: polyserositis or associated amylosis are absent, there is no biological inflammatory syndrome, and in 65% of the patients the CSF contains specific large mononuclear-derived cells called endothelial cells. Such abnormalities have not been described in FMF.
一名33岁患有家族性地中海热(FMF)的西班牙裔犹太男子,在7年期间出现了6次无菌性脑膜炎发作,在腰椎穿刺后不到24小时内病情好转。脑脊液分析显示白细胞混合性增多,范围为100至1000个细胞/mm³。脑脊液细菌、病毒培养及病毒抗体检测均为阴性。我们的病例支持其他报告,表明复发性无菌性脑膜炎虽然罕见,但可能发生在FMF中。它通常像该疾病的其他表现一样,对秋水仙碱治疗有反应。FMF脑膜炎已与病因未明的莫拉雷脑膜炎相比较。然而,与FMF不同,莫拉雷脑膜炎是散发性且普遍存在的,不具有遗传性,对男性和女性的影响相同。此外,在莫拉雷脑膜炎中,经常报告有短暂的神经功能异常,如脑炎体征:不存在多浆膜炎或相关淀粉样变,没有生物炎症综合征,并且65%的患者脑脊液中含有特定的大单核细胞衍生细胞,称为内皮细胞。这些异常在FMF中尚未见描述。