神经白塞综合征的临床和脑脊液细胞学特点

目的回顾性分析神经白塞综合征(NBS)临床和脑脊液(CSF)细胞学资料,探讨该病的临床和CSF细胞学特点。方法回顾1991-2010年间北京协和医院NBS住院患者共27例,分析其临床表现、神经影像学和CSF细胞学特点。结果 27例患者中,神经科症状急性病程19例,亚急性病程6例,慢性病程2例。临床表现:头痛9例,偏瘫8例,复视6例,偏身感觉障碍5例,意识障碍5例,失语3例,癫痫发作3例,精神行为异常、智能减退6例,截瘫2例,共济失调2例,呛咳、吞咽困难2例,帕金森综合征1例。头MRI异常16例,多发片状T1低或较低信号,T2高信号,病灶分布于基底节、脑干、丘脑等处,9例行增强MRI均有强化;2例颈段脊髓MRI可见强化病灶。磁共振静脉成像(MRV):1例符合上矢状窦血栓形成。CSF检查:19例行腰穿查CSF,其中8例压力升高,17例蛋白升高(0.48～1.08g/L);行CSF细胞学检查(沉淀池法制片,迈-格-姬染色)的11例中7例表现为淋巴细胞为主的炎性反应,其中6例伴有中性粒细胞比例升高,余4例中3例为淋巴细胞与中性粒细胞为主的混合性炎性反应,1例为中性粒细胞性炎性反应,8例可见激活淋巴细胞,4例可见浆细胞。结论 NBS临床表现多样,神经影像学改变以基底节、脑干和丘脑等邻近中线的结构受累为主,CSF细胞学呈炎性反应表现。CSF细胞学炎性反应类型,特别是CSF中性粒细胞比例升高对NBS的诊断具有一定意义。

A clinical and cerebrospinal fluid cytology study of neuro-Behcet syndrome

Objective To demonstrate the clinical and cerebrospinal fluid(CSF) cytological characteristics of neuro-Behcet syndrome(NBS),we retrospectively reviewed the clinical data and CSF cytology of patients with NBS in Peking Union Medical College Hospital(PUMCH).Methods We described 27 hospitalized patients with NBS and reviewed their clinical data,neuroimaging findings and CSF cytology.Conventional cytology of CSF was performed with May-Grunwald-Giemsa stain.The relevant literatures on the subject were reviewed.Results The acute onset of neurological symptoms were found in 19,subacute in 6 and chronic in 2 cases.Headache was present in 9 cases,hemiplegia in 8 cases,double vision in 6 cases,consciousness impairment in 5 cases,dysphasia in 3 cases,seizure in 3 cases,mental and behavior disorders in 6 cases,paraplegia in 2 cases,ataxia in 2 cases,bulbar palsy in 2 cases and parkinsonism in 1 case.The common abnormal findings in MR studies were located in brain stem,basal ganglion and thalamus with low or equal signals on T1 and high signals on T2 weighted sequences.All 9 cases who undertook contrasted MR studies showed enhanced lesions.Intracranial venous thrombosis was confirmed by MRV in 1 case.Lumbar puncture was performed in 19 cases with elevated open pressure in 8 cases and increased protein concentration in 17 cases(0.48~1.08 g/L).CSF cells increased in 11 cases (11~48)×106/L].CSF cytology studies of 11 patients demonstrated inflammatory reactions,including lymphocyte-predominant inflammation in 7 cases with mild increase of neutrophil percentage in 6 cases,lymphocyte-neutrophil inflammation in 3 cases and neutrophil predominant inflammation in 1 case.Neutrophils were present in 10 cases totally.Activated lymphocytes were present in 10 cases and plasmacytes in 4 cases.Conclusions NBS may be present with various clinical manifestations,the most common neuroimaging finding are the CNS midline structure involvement including brainstem,basal ganglion and thalamus.The CSF cytological findings,typically the presentation of neutrophils in CSF,indicate the vasculitic nature of NBS and are important in the diagnosis of NBS.