以无菌性脑膜炎为首发症状的组织细胞坏死性淋巴结炎一例

A Case of Kikuchi-Fujimoto's Disease with Aseptic Meningitis as Initial Manifestations

组织细胞坏死性淋巴结炎(KFD)临床以急性或亚急性颈部淋巴结肿痛、发热和粒细胞减少为特征,常合并淋巴结外表现,合并神经系统损害非常少见,以无菌性脑膜炎为首发症状者更为罕见。本研究报道1例年轻女性患者以高热、头痛和四肢抽搐入院,入院后逐渐出现颈部淋巴结肿痛,脑脊液检查以淋巴细胞为主的细胞数目增多。颅脑磁共振成像示:部分软脑膜强化、颅脑静脉成像未见异常,诊断考虑"KFD合并病毒性脑膜炎"。予以地塞米松和阿昔洛韦治疗1个月后症状消失并痊愈。对于伴有淋巴结肿痛的脑膜炎患者应考虑到KFD的可能性,尽早行淋巴结活检可及早明确诊断,避免过度治疗。另外,KFD可伴发复发性无菌性脑膜炎或系统性红斑狼疮等,应注意随访。

Kikuchi Fujimoto disease （KFD） , or histiocytic necrotizing lymphadenitis, is a benign and self- limiting condition characterized by the enlarged cervical lymph nodes, fever and neutropenia. KFD has various extranodal manifestations, including skin lesions, gastrointestinal symptoms or splenomegaly. Neurological complications, including aseptic meningitis are not common. Initial aseptic meningitis associated with KFD is an extremely rare condition. We reported a 22 year - old young woman presenting convulsion of limbs, fever and headache preceding cervical lymphadenopathy. Lymphocyte - dominant pleocyto- sis was observed in cerebrospinal fluid. Brain magnetic resonance imaging showed intensified part of piamater. Dexamethasone and acyclovir were given and showed certain effect. Acute cerebellar symptoms and cervical lymphadenopathy gradually disappeared within one month. A diagnosis of KFD should be considered when enlarged cervical lymph nodes are observed in patients with a- septic meningitis. Fine- needle aspiration and excision biopsy should be undertaken. Hormone and antiviral therapy are effective therapeutic methods. KFD is occasionally accompanied with recurrent meningitis and systemic lupus eythematosus. Follow - up should also be conducted.