抗DPPX(二肽基 - 肽酶样蛋白-6)在2013年发现的脑炎是一种罕见的影响中枢神经系统(CNS)的自身免疫性疾病,其特征是一系列神经系统和胃肠道症状。该疾病是由针对DPPX的抗体引起的,DPPX是KV4.2钾通道的调节亚基,这对于控制神经元兴奋性至关重要。dppx在包括海马和小脑以及胃肠道系统的骨髓丛中的各个大脑区域表达。受体的广泛分布解释了多灶性疾病的表现。通过抗DPPX抗体破坏了这些通道,导致一系列症状,它们本质上是神经系统和胃肠道的[1,2]。抗DPPX脑炎的早期迹象通常包括无法解释的体重减轻和胃肠道障碍,尤其是严重的腹泻。这些症状通常随后是一系列神经系统问题,包括认知功能障碍,躁动,幻觉和夸张的惊吓反应。除了这些神经精神症状外,患者还会出现运动障碍,例如静止震颤,僵硬,肌阵挛,甚至癫痫发作。睡眠迷惑(例如REM睡眠行为障碍)也很常见。大多数患者是中年患者,出现了C.52岁的中位年龄,男性似乎比女性更频繁。通过鉴定患者血液或CSF中的抗DPPX自身抗体来确认诊断[1,2]。
ABI Acquired brain injury ABLV Australian bat lyssavirus ADEM Acute disseminated encephalomyelitis AES Acute encephalitis syndrome ALS Amyotrophic lateral sclerosis AMPAR α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor AMR Region of the Americas AR African region CASPR2 Contactin-associated protein-2 CFR Case fatality rate CMV Cytomegalovirus CNS Central nervous system CP Cerebral palsy CSF Cerebrospinal fluid D2R Dopamine-2 receptor DALYs Disability-adjusted life years DNA Deoxyribonucleic acid DPPX Dipeptidyl-peptidase-like protein-6 EAN European Academy of Neurology EBV Epstein Barr virus EBVL European bat lyssavirus ECDC European Centre for Disease Prevention and Control EEEV Eastern equine encephalitis virus EFNS European Federation of Neurological Societies EFTA European Free Trade Association ELISA Enzyme-linked immunosorbent assay EMR Eastern Mediterranean Region EU European Union EUR European Region EV Enterovirus GABAaR γ-aminobutyric acid-A receptor GABAbR γ-aminobutyric acid-B receptor GAVI Global Alliance for Vaccines and Immunisation GBD Global Burden of Disease GlyR Glycine receptor GOS Glasgow Outcome Scale GP General practitioner HHV-6 Human herpesvirus-6 HIC High-income country HSE Herpes simplex encephalitis HSV Herpes simplex virus ICD International Classification of Disease ICU Intensive care unit ID皮内IHME健康指标和评估研究所IM肌内IQR IQR四分位数iv静脉内静脉内静脉炎日本脑炎日本脑炎日本脑炎病毒病毒LCMV淋巴细胞性绒毛膜炎病毒lgi-1