周围神经或自主神经系统副肿瘤性或自身免疫性疾病
Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system
- 层级
- 4 级
定义
外周神经和自主神经系统的副肿瘤性和自身免疫性疾病是由于外周神经系统（外周神经或自主神经病变）中的神经元或胶质细胞受到靶向免疫攻击所致。在副肿瘤方面，这种攻击是由来自系统性癌症的肿瘤神经抗原引发的潜在有效肿瘤免疫反应的结果。在称为自身免疫的非副肿瘤性疾病中，尽管越来越多的证据表明至少在某些病例中存在感染性触发因素，但其病因仍不清楚。发病可能是亚急性或隐匿性的，这些疾病可能是局限性的、多灶性的或全身性的。自身免疫性躯体周围神经疾病可能在多个层面影响神经，包括根、神经和神经丛，可能是轴突性、脱髓鞘性或两者兼而有之。自身免疫性自主神经疾病导致自主神经功能衰竭，可部分或全身。 自主神经病的原型是自身免疫性神经节病。 靶向神经节尼古丁乙酰胆碱受体（α3 gAChR）的抗体。癌症筛查适合于具有特定神经抗体谱和其他风险因素的人群。 周围神经病的相关神经抗体包括抗神经元核抗体1型或抗Hu、CASPR2、gAChR、神经节苷脂（GM1 IgG和IGM）抗体。
Paraneoplastic and autoimmune disorders of the peripheral and autonomic nervous system result from a targeted immune attack on neurons or glial cells in the peripheral nervous systems (peripheral or autonomic neuropathies). In the paraneoplastic context, this attack is a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. In the non-paraneoplastic context termed autoimmune the etiology remains elusive though increasing evidence indicates a preceding infectious trigger in at least some cases. Onset may be subacute or insidious and these disorders may be limited, multifocal or generalised. Autoimmune somatic peripheral nerve disorders may affect the nerve at multiple levels including root, nerve and plexus and may be axonal, demyelinating or both. Autoimmune autonomic disorders result in autonomic failure that can be partial or generalised. The prototypic autonomic neuropathy is the autoimmune ganglionopathy associated with antibodies targeting the ganglionic nicotinic acetylcholine receptor (α3 gAChR). Screening for cancer is appropriate for those with specific neural antibody profiles and other risk factors. Associated neural antibodies in peripheral neuropathies include anti neuronal nuclear antibody type 1 or anti-Hu, CASPR2, gAChR, ganglioside (GM1 IgG and IGM) antibodies.
索引词 可用于检索该条目的同义词与别名
- 周围神经或自主神经系统副肿瘤性或自身免疫性疾病
- 副肿瘤性感觉神经节病
- 副肿瘤性感觉神经病
- 副肿瘤性感觉元神经病
- 副肿瘤性感觉神经节病，神经自身抗体阴性
- 自身免疫性感觉神经节病
- 自身免疫性感觉神经节病，神经自身抗体阴性
- 自身免疫性感觉神经节病，神经自身抗体阳性
- 自身免疫性神经炎
- 自身免疫性臂丛神经炎
- 自身免疫性神经根神经丛神经病
- 神经根神经丛神经病
- 非糖尿病性神经根神经丛神经病
- 慢性免疫脱髓鞘性多发性神经病
- 慢性免疫脱髓鞘性多发性神经病变臂丛变异
- 手术后神经丛炎
- 术后神经丛炎
- 手术后臂丛神经炎
- 手术后腰骶丛神经炎
- 副肿瘤性感觉运动神经病
- 副肿瘤性感觉运动神经病，神经自身抗体阴性
- 副肿瘤性感觉运动神经病，神经自身抗体阳性
- 血管炎或血管炎性神经病
- 系统性多发性单神经病血管炎
- 多发性单神经病血管炎伴混合性冷球蛋白血症
- 多发性单神经病血管炎伴核周抗中性粒细胞胞质抗体相关性血管炎
- 多发性单神经病血管炎伴Churg-Strauss综合征
- 多发性单神经病血管炎伴韦格纳肉芽肿病
- 非系统性多发性单神经病血管炎
- 神经局限性多发性单神经病血管炎
- 自身免疫感觉运动神经病
- 自身免疫性感觉运动神经病，神经自身抗体阴性
- 自身免疫性感觉运动神经病，神经自身抗体阳性
- 自身免疫性自主神经病
- 自身免疫性自主神经节病
- 自身免疫纯自主神经衰竭
- 自身免疫性自主神经功能障碍
- 全植物神经功能不全
- 全植物神经功能不全，神经自身抗体阳性
- 全植物神经功能不全，神经自身抗体阴性
- 自身免疫性自主神经病，神经节乙酰胆碱受体抗体阳性
- 自身免疫性自主神经病，其他神经自身抗体阳性
- 自身免疫性自主神经病，神经自身抗体阴性
- 局限性自身免疫性自主神经病
- 局限性自身免疫性自主神经病，神经节乙酰胆碱受体抗体阳性
- 局限性自身免疫性自主神经病，神经自身抗体阳性
- 局限性自身免疫性自主神经病，神经自身抗体阴性
- 副肿瘤性自主神经性肠套叠
- 自身免疫性获得性自主神经性脑脊髓炎
- 副肿瘤性感觉神经病
- Denny Brown
- 副肿瘤性感觉神经病，神经自身抗体阴性
- 副肿瘤性感觉神经病，神经自身抗体阳性
- 自身免疫性感觉神经病
- 自身免疫性感觉神经病，神经自身抗体阴性
- 自身免疫性感觉神经病，神经自身抗体阳性
- 副肿瘤性运动神经病
- 副肿瘤性运动神经病，神经自身抗体阴性
- 自身免疫性运动神经病
- 自身免疫性运动神经病，神经自身抗体阴性
- 自身免疫性运动神经病，神经自身抗体阳性
- 意义不明的单克隆丙种球蛋白病相关性神经病
- MGUS[意义不明的单克隆丙种球蛋白病]相关性神经病
- Castleman病伴IgM相关性神经病
- 髓磷脂相关糖蛋白
- MAG[髓磷脂相关糖蛋白]
- 远端获得性脱髓鞘神经病
- DADS[远端获得性脱髓鞘神经病]
- IgG、IgA或IgM相关性骨髓瘤伴多神经病、器官肥大症、内分泌病、M-spike和皮肤改变
- 副肿瘤性神经肌病和神经病
- 癌性神经病
- 癌性神经肌病 [possible translation]
- 感觉性副肿瘤性神经病[Denny Brown] [possible translation]
- 癌性神经肌病
- 感觉性副肿瘤性神经病[Denny Brown]
- 脊髓副肿瘤性疾病
- 副肿瘤性自主神经病变
- 未特指部位的副肿瘤性神经病
英文词条（75）
- Paraneoplastic or autoimmune disorders of the peripheral or autonomic nervous system
- Paraneoplastic sensory ganglionopathy
- Paraneoplastic sensory neuronopathy
- Paraneoplastic sensory ganglionopathy, neural autoantibody negative
- Autoimmune sensory ganglionopathy
- Autoimmune sensory ganglionopathy, neural autoantibody negative
- Autoimmune sensory ganglionopathy, neural autoantibody positive
- Autoimmune neuritis
- Autoimmune brachial neuritis
- Autoimmune radiculoplexoneuropathy
- Radiculoplexoneuropathy
- Nondiabetic radiculoplexoneuropathy
- Chronic immune demyelinating polyradiculoneuropathy
- Brachial variant of chronic immune demyelinating polyradiculoneuropathy
- Post surgical plexitis
- Postsurgical plexitis
- Post surgical brachial plexitis
- Post surgical lumbosacral plexitis
- Paraneoplastic sensorimotor neuropathy
- Paraneoplastic sensorimotor neuropathy, neural autoantibody negative
- Paraneoplastic sensorimotor neuropathy, neural autoantibody positive
- Vasculitis or vasculitic neuropathy
- Systemic multiple mononeuropathy vasculitis
- Multiple mononeuropathy vasculitis with mixed cryoglobulinemia
- Multiple mononeuropathy vasculitis with perinuclear antineutrophil cytoplasmic antibody-associated vasculitis
- Multiple mononeuropathy vasculitis with Churg-Strauss syndrome
- Multiple mononeuropathy vasculitis with Wegener granulomatosis
- Non-systemic multiple mononeuropathy vasculitis
- Nerve restricted multiple mononeuropathy vasculitis
- Autoimmune sensorimotor neuropathy
- Autoimmune sensorimotor neuropathy, neural autoantibody negative
- Autoimmune sensorimotor neuropathy, neural autoantibody positive
- Autoimmune autonomic neuropathy
- autoimmune autonomic ganglionopathy
- autoimmune dysautonomia
- autoimmune pure autonomic failure
- Pandysautonomia
- Pandysautonomia neural autoantibody positive
- Pandysautonomia neural autoantibody negative
- Autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positive
- Autoimmune autonomic neuropathy, other neural autoantibody positive
- Autoimmune autonomic neuropathy, neural autoantibody negative
- Limited autoimmune autonomic neuropathy
- Limited autoimmune autonomic neuropathy, ganglionic acetylcholine receptor antibody positive
- Limited autoimmune autonomic neuropathy, neural autoantibody positive
- Limited autoimmune autonomic neuropathy, neural autoantibody negative
- Paraneoplastic autonomic intussusception
- Autoimmune acquired autonomic encephalomyelitis
- Paraneoplastic sensory neuropathy
- Denny Brown
- Paraneoplastic sensory neuropathy, neural autoantibody negative
- Paraneoplastic sensory neuropathy, neural autoantibody positive
- Autoimmune sensory neuropathy
- Autoimmune sensory neuropathy, neural autoantibody negative
- Autoimmune sensory neuropathy, neural autoantibody positive
- Paraneoplastic motor neuropathy
- Paraneoplastic motor neuropathy, neural autoantibody negative
- Autoimmune motor neuropathy
- Autoimmune motor neuropathy, neural autoantibody negative
- Autoimmune motor neuropathy, neural autoantibody positive
- Monoclonal gammopathy of undetermined significance-associated neuropathies
- MGUS - [monoclonal gammopathy of undetermined significance] associated neuropathies
- Castleman disease with IgM-associated neuropathy
- Myelin-associated glycoprotein
- MAG - [Myelin-associated glycoprotein]
- Distal acquired demyelinating neuropathy
- DADS - [Distal acquired demyelinating neuropathy]
- IgG, IgA or IgM-associated osteosclerotic myeloma with polyneuropathy organomegaly endocrinopathy M-spike and skin changes
- Paraneoplastic neuromyopathy and neuropathy
- Carcinomatous neuromyopathy
- Sensorial paraneoplastic neuropathy [Denny Brown]
- carcinomatous neuropathy
- Paraneoplastic disorders of the spinal cord
- Paraneoplastic autonomic neuropathy
- Paraneoplastic neuropathy of unspecified site
后组配轴 Postcoordination
后组配是在主干编码之后追加的可选扩展轴（如侧别、具体解剖部位、严重程度），用于把「部位 + 侧别 + 病程」等细节一次表达完整。只有标注「必填」的轴在组合编码时必须给出，其余可留空。
- 临床表现hasManifestation可选
- 相关疾患associatedWith可选
- 病因hasCausingCondition必填