Acute pharyngitis with the abrupt manifestation of neurological disorders, leading to a diagnosis of Neuro-Behcet’s disease

Acute pharyngitis with the abrupt manifestation of neurological disorders, leading to a diagnosis of Neuro-Behcet’s disease
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急性咽炎伴有神经系统疾病的突然表现,导致神经白塞氏病的诊断

DOI:
10.1080/23772484.2021.1975497
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发表时间:
2021
影响因子:
0.1
通讯作者:
Ito Ken
Ito Ken
中科院分区:
--
文献类型:
--
作者:
Yamagishi Junya;Kagoya Ryoji;Saito Maki;Fujimoto Chisato;Kikuchi Hirotoshi;Ito Ken

文献摘要

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神经behet病(NBD)累及神经系统,预后较差。由于神经系统症状发作的典型延迟是数年,当神经系统疾病几乎与其他主要症状同时出现时,诊断NBD是困难的。我们在此报告一个罕见的NBD病例,其主要症状和神经系统疾病几乎同时出现。一名32岁的日本妇女表现为严重的咽炎,有白色涂层和溃疡。入院第三天,她突然因尿失禁而在走路时晕倒。t2加权MRI显示脑干多发高强度信号。生殖器溃疡和背部假性毛囊炎也被发现。稳定性测量显示主要受累于外周前庭系统。脑脊液检查显示以淋巴细胞为主的细胞数量增加。白细胞介素-6水平明显升高。脉冲皮质类固醇治疗导致症状的减轻。
Neuro-Behçet’s disease (NBD) involves the nervous system and has a poorer prognosis. Since the typical delay in the onset of neurological symptoms is several years, difficulties are associated with diagnosing NBD when neurological disorders occur almost simultaneously with other major symptoms. We herein report a rare case of NBD that presented with the almost simultaneous manifestation of principal symptoms and neurological disorders.A 32-year-old Japanese woman presented with severe pharyngitis with a white coating and ulcers. On the third day of admission, she suddenly fainted while walking with urinary incontinence. T2-weighted MRI showed multiple high-intensity signals in the brainstem. Genital ulcers and pseudofolliculitis on the back were also detected. Stabilometry showed principal involvement of the peripheral vestibular system. A cerebrospinal fluid examination revealed an increase in the number of cells with lymphocyte dominance. The interleukin-6 level was markedly elevated. Pulse corticosteroid therapy led to the attenuation of symptoms.