脑实质型神经白塞病25例患者临床特征分析

Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet′s disease

目的对脑实质型神经白塞病(P-NBD)患者临床资料、影像学特征,治疗及预后等方面进行回顾性研究,并重点关注其头晕主诉。方法横断面研究。共纳入2010年至2022年于解放军总医院第一医学中心神经内科就诊并最终确诊为P-NBD的25例患者临床资料。人群年龄中位数为37(17~85)岁,回顾性分析性别、起病年龄、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、头颅和脊髓磁共振、治疗和转归等临床资料。结果(1)本组男性居多,16例(64.0%),平均发病年龄为4~58(28±14)岁,呈急性或亚急性病程。发热是最常见的临床表现,头晕(32.0%)主诉不少见。(2)80.0%(20/25)患者血清免疫指标异常,包括补体(C3和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。16例患者行腰椎穿刺检查,多数颅内压正常、脑脊液白细胞数和蛋白升高[中位数分别为44(15~380)×106/L和0.73(0.49~2.81)g/L]。5例行脑脊液细胞因子检查,4例结果异常,以IL-6升高为著,其次为IL-1、IL-8。(3)头颅磁共振最常受累部位为脑干和基底节,各有15例(60.0%),其次为白质[12例(48.0%)]和皮质[11例(44.0%)]。9例(36.0%)患者病灶伴强化,6例(24.0%)患者为团块样病灶。3例(12.0%)患者脊髓受累,以胸髓多见。(4)所有患者均采用免疫干预治疗,随访结局普遍良好。结论P-NBD是一种临床表现多样且多系统受累的自身免疫性疾病,头晕症状并不少见,且易被忽视。早期充分的免疫治疗是改善预后的关键。

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.