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白塞氏综合征患者神经系统受累的各种类似表现。

Mimickers of nervous system involvement among patients with Behçet's syndrome.

机构信息

School of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Goztepe Prof Dr Suleyman Yalcin City Hospital, Istanbul, Turkey.

出版信息

J Neurol. 2024 Oct;271(10):6903-6911. doi: 10.1007/s00415-024-12613-9. Epub 2024 Sep 4.

Abstract

OBJECTIVES

We aimed to identify conditions mimicking nervous system involvement among patients with Behçet's syndrome (BS) and to determine clinical, laboratory and imaging findings that may help in the differential diagnosis.

METHODS

We screened the charts of 500 consecutive BS patients to identify those who were referred to neurology at any time during their follow-up. The final diagnoses, presenting signs and symptoms, laboratory and imaging results were retrieved from patient charts. Patients who did not have a follow-up visit during the last 3 months were invited to the clinic.

RESULTS

Among the 500 BS patients, 116 (23%) had been referred to neurology. Among these, 29 (5.8%) were diagnosed with typical central nervous system involvement of BS (NeuroBS). The type of NeuroBS was parenchymal involvement in 21 patients, cerebral venous sinus thrombosis in 7 patients, and both in 1 patient. 30 patients (6%) had other conditions related to the nervous system, 46 (9.2%) did not have a nervous system disorder, and their symptoms recovered spontaneously, and 11 (2.2%) were lost to follow-up without a definite diagnosis. Of the 30 BS patients who were diagnosed with another nervous system condition, 14 (46%) had primary headache syndromes, 6 (20%) had psychiatric disorders, 2 had entrapment neuropathy, and 1 each had epilepsy, glial tumor, multiple sclerosis, Meniere's disease, optic neuritis, neuroretinitis, steroid myopathy and polyneuropathy.

CONCLUSION

Nervous system conditions other than NeuroBS are frequent among BS patients referred to neurology. Caution is required to avoid misdiagnosis of these patients as NeuroBS.

摘要

目的

我们旨在确定白塞病(BS)患者中类似神经系统受累的情况,并确定有助于鉴别诊断的临床、实验室和影像学发现。

方法

我们筛选了 500 例连续 BS 患者的病历,以确定在随访期间任何时候转至神经内科的患者。从患者病历中检索最终诊断、表现出的体征和症状、实验室和影像学结果。邀请在过去 3 个月内没有就诊随访的患者来诊所。

结果

在 500 例 BS 患者中,有 116 例(23%)曾转诊至神经内科。其中,29 例(5.8%)被诊断为典型的 BS 中枢神经系统受累(NeuroBS)。21 例为实质受累型,7 例为脑静脉窦血栓形成,1 例为两者兼有。30 例(6%)存在其他与神经系统相关的疾病,46 例(9.2%)无神经系统疾病,其症状自行缓解,11 例(2.2%)失访而无明确诊断。在被诊断为另一种神经系统疾病的 30 例 BS 患者中,14 例(46%)为原发性头痛综合征,6 例(20%)为精神障碍,2 例为神经卡压性神经病,各有 1 例患有癫痫、神经胶质细胞瘤、多发性硬化、梅尼埃病、视神经炎、神经视网膜炎、类固醇肌病和多发性神经病。

结论

在转至神经内科的 BS 患者中,除 NeuroBS 外,神经系统疾病较为常见。应谨慎避免将这些患者误诊为 NeuroBS。

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