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.

中文翻译：

---

麻风病中枢神经系统表现的范围：对已发表病例报告和病例系列的系统回顾

背景 本系统综述旨在通过分析多个队列研究、个体病例和病例系列来调查麻风病中枢神经系统（CNS）的参与情况。方法 遵循系统评价和荟萃分析指南的首选报告项目。使用预定义的检索策略对 PubMed、Scopus 和 Embase 数据库进行了截至 2023 年 7 月 8 日的检索。纳入标准包括诊断为麻风病并有中枢神经系统受累证据的患者。使用乔安娜·布里格斯研究所清单评估纳入病例的质量。结果 共识别出 34 条记录，其中包括 18 项队列研究和 16 份描述 27 个孤立病例的报告。尸检显示脊髓、神经原纤维缠结和老年斑的宏观变化。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、丙种球蛋白增加以及检测到麻风分枝杆菌抗原和抗体。21 名患者 (78%) 检测到脊髓/臂丛神经异常。大多数患者的 MRI 显示颈髓存在 T2/液体衰减反转恢复 (FLAIR) 高信号。在脑干受累的患者中，小脑脚、面神经核和/或其他脑神经核中出现 T2/FLAIR 高信号。三名患者发现脑实质受累。结论 根据尸检结果、神经影像学、脑脊液分析和神经生理学研究，本系统评价提供了麻风病中枢神经系统受累的证据。