[25例脑实质神经-白塞病临床特点分析]。

Y Li, Q Shi, Y Lin, M Y Liu, J Q Liu
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摘要

目的:回顾性分析以头晕为主的脑实质性神经白塞病(P-NBD)的临床资料、影像学特征、治疗及转归。方法:对2010年至2022年中国人民解放军总医院第一医疗中心神经内科收治的25例确诊为P-NBD患者的临床资料进行横断面研究。人口年龄中位数为37岁(范围:17-85岁)。回顾性分析临床资料,包括性别、发病年龄、病程、临床表现、血清免疫指标、脑脊液常规生化及细胞因子水平、颅脑及脊髓磁共振成像(MRI)表现、治疗及转归。结果:患者以男性居多(16例;(64.0%),平均发病年龄(28±14)岁(4 ~ 58岁),病程为急性或亚急性。发热是最常见的临床表现,主诉头晕也不少见(8/25例)。80.0%(20/25)患者血清免疫指标补体(C3、C4)、红细胞沉降率、白细胞介素-1 (IL-1)、IL-6、IL-8、肿瘤坏死因子- α异常。16/25行腰椎穿刺检查的患者中,大多数颅内压正常,脑脊液白细胞计数和蛋白升高[中位值分别为44 (15-380)×106/L和0.73 (0.49-2.81)g/L]。在接受脑脊液细胞因子检测的5例患者中,4例患者结果异常;其中,IL-6水平升高最为常见,其次是IL-1和IL-8。颅脑MRI最常见的受累部位是脑干和基底节区(分别为60.0%),其次是白质(48.0%)和皮层(44.0%)。病灶强化9例(36.0%),肿块样病变6例(24.0%)。3例(12.0%)患者有脊髓病变,最常见的是胸脊髓。所有患者均接受免疫干预治疗;在随访期间,大多数患者预后良好。结论:P-NBD是一种多系统受累、临床表现多样的自身免疫性疾病。头晕的症状并不罕见,很容易被忽视。早期免疫治疗很重要,可以改善这些患者的预后。
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[Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet's disease].

Objective: To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. Methods: This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. Results: The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×106/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. Conclusions: P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.

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