背景:本系统综述旨在通过分析多个队列研究来研究麻风病的中枢神经系统(CNS)受累,个案和案例系列。
方法:遵循系统评价和Meta分析指南的首选报告项目。PubMed,截至2023年7月8日,使用预定义的搜索策略搜索了Scopus和Embase数据库。纳入标准包括诊断为麻风病并有中枢神经系统受累的患者。使用JoannaBriggs研究所检查表评估了纳入病例的质量。
结果:总共确定了34条记录,包括18项队列研究和16例报告,描述了27例孤立病例。尸体解剖显示脊髓的宏观变化,神经原纤维缠结和老年斑。使用PCR和酚糖脂1染色在延髓和脊髓的神经元中检测到麻风分枝杆菌。脑脊液(CSF)分析显示炎症变化,增加γ球蛋白和检测麻风分枝杆菌抗原和抗体。在21名患者(78%)中,发现脊髓/臂丛神经异常。在大多数情况下,MRI显示颈髓的T2/液体衰减倒置恢复(FLAIR)高强度。在脑干受累的患者中,T2/FLAIR高强度见于小脑柄,面部神经核和/或其他颅神经核。在三名患者中发现了脑实质受累。
结论:本系统综述提供了麻风病患者中枢神经系统受累的证据,根据尸检结果,神经影像学,脑脊液分析和神经生理学研究。
BACKGROUND: This systematic review aimed to investigate central nervous system (CNS) involvement in leprosy by analysing multiple cohort studies, individual cases and
case series.
METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. PubMed, Scopus and Embase databases were searched up to 8 July 2023, using a predefined search strategy. Inclusion criteria included patients diagnosed with leprosy with evidence of CNS involvement. The quality of the included cases was evaluated using the Joanna Briggs Institute checklist.
RESULTS: A total of 34 records were identified, including 18 cohort studies and 16
reports describing 27 isolated cases. Autopsies revealed macroscopic changes in the spinal cord, neurofibrillary tangles and senile plaques. Mycobacterium leprae was detected in neurons of the medulla oblongata and spinal cord using PCR and phenolic glycolipid 1 staining. Cerebrospinal fluid (CSF) analysis showed inflammatory changes, increased gamma globulins and detection of Mycobacterium leprae antigens and antibodies. In 21 patients (78%), spinal cord/brachial plexus abnormities were detected. In the majority, MRI revealed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensity in the cervical cord. In patients with brainstem involvement, T2/FLAIR hyperintensity was noted in the cerebellar peduncles, facial nerve nuclei and/or other cranial nerve nuclei. Brain parenchymal involvement was noted in three patients.
CONCLUSIONS: This systematic review provides evidence of CNS involvement in leprosy, based on autopsy findings, neuroimaging, CSF analysis and neurophysiological studies.