Department of Neurology, Huizhou Central People's Hospital, No. 41, Eling North Road, Huizhou, Guangdong Province, 516001, China.
BMC Neurol. 2024 Jan 19;24(1):34. doi: 10.1186/s12883-024-03538-x.
Neuroleptospirosis and anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis are both very rare and have only been reported in the form of respective case reports. There are no reports of anti-DPPX encephalitis combined with neuroleptospirosis in the literature. We reported the first case of neuroleptospirosis combined with elevated DPPX antibodies in serum and cerebrospinal fluid (CSF).
A previously healthy 53-year-old Chinese male farmer with a history of drinking raw stream water and flood sewage exposure was brought to the hospital due to an acute onset of neuropsychiatric symptoms. No fever or meningeal irritation signs were detected on physical examination. Routine laboratory investigations, including infection indicators, leukocyte and protein in CSF, electroencephalogram and gadolinium-enhanced magnetic resonance imaging of the brain, all revealed normal. While metagenomic next-generation sequencing (mNGS) identified the DNA genome of Leptospira interrogans in the CSF. Anti-DPPX antibody was detected both in blood and in CSF. A diagnosis of neuroleptospirosis combined with autoimmune encephalitis associated with DPPX-Ab was eventually made. He resolved completely after adequate amount of penicillin combined with immunotherapy.
We highlight that in patients with acute or subacute behavioral changes, even in the absence of fever, if the most recent freshwater exposure is clear, physicians should pay attention to leptospirosis. Due to the low sensitivity of routine microscopy, culture, polymerase chain reaction and antibody testing, mNGS may have more advantages in diagnosing neuroleptospirosis. As autoimmune encephalitis can be triggered by various infections, neuroleptospirosis may be one of the causes of autoimmune encephalitis. Since neuronal antibody measurements themselves are not that common in neuroleptospirosis, future studies are needed to determine whether the detection of anti-DPPX antibodies is a rare event in leptospirosis. Early identification of autoimmune encephalitis and timely administration of immunotherapy may lead to a better outcome.
神经莱姆病和抗二肽基肽酶样蛋白 6（DPPX）脑炎均非常罕见，且仅以各自的病例报告形式报道。文献中尚无抗 DPPX 脑炎合并神经莱姆病的报道。我们报告首例血清和脑脊液（CSF）中存在升高的 DPPX 抗体的神经莱姆病合并抗 DPPX 脑炎。
一名既往健康的 53 岁中国男性农民，有饮用生水和接触洪水污水史，因急性起病的神经精神症状就诊。体格检查未发现发热或脑膜刺激征。常规实验室检查，包括感染指标、CSF 白细胞和蛋白、脑电图和脑钆增强磁共振成像，均正常。而宏基因组下一代测序（mNGS）在 CSF 中识别出钩端螺旋体 interrogans 的 DNA 基因组。血液和 CSF 中均检测到抗 DPPX 抗体。最终诊断为神经莱姆病合并与 DPPX-Ab 相关的自身免疫性脑炎。足量青霉素联合免疫治疗后完全缓解。
我们强调，对于急性或亚急性行为改变的患者，即使没有发热，如果最近有淡水暴露史，医生应注意莱姆病。由于常规显微镜检查、培养、聚合酶链反应和抗体检测的敏感性较低，mNGS 可能在诊断神经莱姆病方面具有更多优势。由于各种感染均可引发自身免疫性脑炎，神经莱姆病可能是自身免疫性脑炎的病因之一。由于神经元抗体检测本身在神经莱姆病中并不常见，因此需要进一步研究来确定在莱姆病中检测到抗 DPPX 抗体是否为罕见事件。早期识别自身免疫性脑炎并及时给予免疫治疗可能会改善预后。
