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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。
Objective To retrospectively analyze the clinical and cerebrospinal fluid (CSF) cytology data of Nerve Behcet’s syndrome (NBS) to investigate the clinical and cytological features of the disease. Methods A total of 27 inpatients with NBS at Peking Union Medical College Hospital from 1991 to 2010 were retrospectively analyzed. The clinical manifestations, neuroimaging and cytological features of CSF were analyzed. Results Among the 27 patients, there were 19 cases of acute neurological symptoms, 6 cases of subacute disease and 2 cases of chronic disease. Clinical manifestations: headache in 9 cases, hemiplegia in 8 cases, diplopia in 6 cases, 5 cases of partial sensory dysfunction, 5 cases of disturbance of consciousness, 3 cases of aphasia, 3 cases of seizures, mental disorders, 6 cases of intellectual decline, paraplegia in 2 cases, Ataxia in 2 cases, choking, swallowing difficulties in 2 cases, Parkinson’s syndrome in 1 case. There were 16 cases of MRI abnormalities, multiple low or low signal of T1, high signal of T2, focal distribution in basal ganglia, brainstem and thalamus, all of which were enhanced by enhanced MRI in 9 cases. Lesions. Magnetic resonance venography (MRV): 1 case met the superior sagittal sinus thrombosis. CSF examination: 19 cases of lumbar puncture CSF, including 8 cases of elevated pressure, 17 cases of protein increased (0.48 ~ 1.08g / L); CSF cytology (sedimentation pool method, Mai - lattice - Ji staining) Of the 11 cases, 7 cases showed lymphocyte-predominant inflammatory reaction, of which 6 cases were accompanied by an increased proportion of neutrophils and 3 cases were mixed lymphocytic and neutrophilic inflammatory diseases One case had neutrophilic inflammatory reaction, eight cases showed activated lymphocytes and four cases showed plasma cells. Conclusion The clinical manifestations of NBS are diverse. The changes of neuroimaging are mainly affected by the structure of the proximal midline, such as basal ganglia, brainstem, and thalamus. CSF cytology is characterized by inflammatory reaction. The type of CSF cytological inflammatory response, particularly the increased proportion of CSF neutrophils, has implications for the diagnosis of NBS.