Hansen Klaus, Crone Clarissa, Kristoferitsch Wolfgang
Department of Neurology, Rigshospitalet, University Clinic Copenhagen, Denmark.
Handb Clin Neurol. 2013;115:559-75. doi: 10.1016/B978-0-444-52902-2.00032-1.
Lyme neuroborreliosis (LNB) designates the nervous system disorders caused by the tick-borne spirochete Borrelia burgdorferi (Bb). The clinical syndromes are usually distinct and are classified as early and the rare late or chronic LNB. Early LNB occurs 3-6 weeks after infection most frequently as a lymphocytic meningoradiculoneuritis (LMR). Symptoms are mainly due to a painful sensory radiculitis and a multifocal motor radiculo-neuritis. Fifty percent have cranial nerve involvement predominantly uni- or bilateral facial nerve palsies. Meningitic symptoms occur primarily in children. Nerve biopsies, autopsies, animal models, and nerve conduction studies showed that the pathology is a lymphocytic perineuritis leading to multisegmental axonal injury of nerve roots, spinal ganglia, and distal nerve segments. Due to meningeal and root inflammation cerebrospinal fluid (CSF) shows lymphocytic inflammation. The only evidence that Bb causes peripheral neuropathy without CSF inflammation is seen in patients with acrodermatitis chronica atrophicans (ACA), a chronic dermatoborreliosis. In the rare chronic or late LNB the pathology and thus the clinical presentation is primarily due to chronic meningitis and meningovascular CNS involvement, whereas the peripheral nervous system is not primarily affected. In early and late LNB the diagnosis is based on a characteristic clinical appearance and CSF inflammation with Bb-specific intrathecal antibody production. Both conditions, but not the ACA-associated neuropathy, respond to antibiotic therapy.
莱姆病神经螺旋体病(LNB)指由蜱传播的螺旋体伯氏疏螺旋体(Bb)引起的神经系统疾病。临床综合征通常各不相同,分为早期以及罕见的晚期或慢性LNB。早期LNB在感染后3至6周出现,最常见的表现为淋巴细胞性脑膜神经根神经炎(LMR)。症状主要源于疼痛性感觉神经根炎和多灶性运动神经根神经炎。50%的患者有颅神经受累,主要是单侧或双侧面神经麻痹。脑膜症状主要出现在儿童身上。神经活检、尸检、动物模型和神经传导研究表明,病理改变是淋巴细胞性周围神经炎,导致神经根、脊神经节和远端神经节段的多节段轴索损伤。由于脑膜和神经根炎症,脑脊液(CSF)显示淋巴细胞炎症。在慢性萎缩性肢端皮炎(ACA)患者中可以看到Bb导致无脑脊液炎症的周围神经病变的唯一证据,ACA是一种慢性皮肤伯氏疏螺旋体病。在罕见的慢性或晚期LNB中,病理改变以及临床表现主要是由于慢性脑膜炎和脑膜血管中枢神经系统受累,而周围神经系统并非主要受影响。在早期和晚期LNB中,诊断基于特征性临床表现以及脑脊液炎症伴Bb特异性鞘内抗体产生。这两种情况对抗生素治疗均有反应,但ACA相关的神经病变除外。