1、重视脑损伤后发作性交感过度兴奋重视脑损伤后发作性交感过度兴奋中中重度脑损伤患者的临床表现中重度脑损伤患者的临床表现中,常可见到一组发作性的常可见到一组发作性的高热高热、多汗多汗、呼吸急促呼吸急促、心动过速心动过速、血压升高血压升高、瞳孔改变瞳孔改变、烦躁并全身强直烦躁并全身强直、阵挛阵挛等肌张力障碍等症候群。等肌张力障碍等症候群。Perkes I,Baguley IJ,Nott MT,Menon DK.A review 1.of paroxysmalsympathetic hyperactivity after acquired brain injury.Ann Neurol2010;68:1
2、26135.tachycardia(120beats/min),tachypnea(30/min),systolic hypertension(160mmHg),hyper/hypothermia,excessive sweating,decerebration/decortication,increased muscle tone,horripilation鸡皮疙瘩 and/or flushing皮肤发红 is collectively referred to as“dysautonomia”or“paroxysmal sympathetic hyperactivity”syndrome典型
3、的体温血压图典型的体温血压图过山车过山车发病率高低不一发病率高低不一 9.333%.Kishner S,Augustin J,Strum S.Post head injury autonomic complications.Last updated 4 October 2006 October 4.Accessed 18 June 2007.Fearnside MR,Cook RJ,McDougall P,McNeil RJ.The westmead head injury project outcome in severe head injury.A comparative analysis
4、 of pre-hospital,clinical and CT variables.British Journal of Neurosurgery 1993;7:267279.In the first post-injury week in ICU.Of the whole sample,33%developed heart rates 120/min and respiratory rates 30/min and 25%had blood pressure 160mmHg and temperature 39 C at some time in the first week.Lemke
5、DM.Sympathetic storming after severe traumatic braininjury.Crit Care Nurse 2007;27(1):307.onset of dysautonomic paroxysms and various afferent stimuli,both noxious and non-noxious.Such stimuli have included pain,endotracheal suctioning,passive movements such as turning,bathing and muscle stretching,
6、constipation便秘 or a kinked catheter导尿,emotional stimuli,as well as environmental stimuli such as loud noises Laxe S,Terr R,Len D,Bernabeu M.How does dysautonomia influence the outcome of traumatic brain injured patientsadmitted in a neurorehabilitation unit?Brain Inj.2013;27(12):1383-7.All patients
7、had been referred to the S.Anna Institute RAN in the years 19982005 for being in a VS/UWS condition.PSH occurred in 26.1%of them,with greater incidence after traumatic than non-traumatic brain injury(31.9%vs 15.8%).Outcome was worse following non-traumatic brain damage irrespective of PSH and worst
8、among non-traumatic subjects with PSH.untreated Dysautonomia increases mortality through prolonged hyperthermia,excessive catabolism分解代谢,high catecholamine儿茶酚胺 levels and spasticity/dystonia临床值得关注和重视！临床值得关注和重视！Baguley IJ,Heriseanu RE,Gurka JA,Nordenbo A,Cameron ID.Gabapentin in the management of Dys
9、autonomia followingsevere traumatic brain injury:a case series.J Neurol NeurosurgPsychiatr 2007;78(5):53941 it is not possible to completely exclude an epileptogenic aetiology for all cases of Dysautonomia multiple attempts to either identify or treat epilepsy in Dysautonomic patients have produced
10、negative results 常见原因常见原因 脑外伤、肿瘤、脑积水、颅内出血、脑外伤、肿瘤、脑积水、颅内出血、蛛网膜下腔出血、缺氧性脑病蛛网膜下腔出血、缺氧性脑病,其中其中脑外伤脑外伤是最常见的原因是最常见的原因 也有各种原因导致的也有各种原因导致的缺氧性脑病缺氧性脑病 Dysautonomia临床涵盖多个综合征 These syndromes include NMS,SS,Parkinsonian-Hyperpyrexia Syndrome(PHS)intrathecal baclofen withdrawal Autonomic Dysreflexia Malignant Catat
11、onia紧张症 Malignant Hyperthermia Stiff Man Syndrome and Irukandji Syndrome.针对脑损伤后的症候群针对脑损伤后的症候群-命名命名 创伤性脑损伤后自主神经功能障碍、创伤性脑损伤后自主神经功能障碍、自主神经功能障碍综合征、自主神经功能障碍综合征、急性下丘脑功能不稳、急性下丘脑功能不稳、下丘脑中脑功能失调综合征、下丘脑中脑功能失调综合征、间脑综合征、间脑综合征、间脑发作、间脑发作、发作性自主神经或交感神经爆发、发作性自主神经或交感神经爆发、中枢热、中枢热、高热伴持续性肌肉收缩高热伴持续性肌肉收缩 病因区别病因区别 脑损伤后发作性自主
12、神经功能障碍 家族性遗传性自主神经功能障碍、病毒感染后自主神经功能障碍、Guillain-Barre综合征伴发的自主神经功能障碍、脊髓损伤后的自主神经功能障碍Blackman JA,Patrick PD,Buck ML,Rust Jr.RS.Paroxysmal autonomic instability with dystonia after brain injury.Archives of Neurology 2004;61:321328.Paroxysmal Autonomic Instability with Dystonia(PAID)non-specific term“Dysaut
13、onomia”diagnosis of PAID requires at least one(otherwise undefined)daily paroxysm occurring for at least 3 days to fulfil criteria目前较为接受的名称目前较为接受的名称 Paroxysmal sympathetic hyperactivity after traumatic brain injury PSHFernandez-Ortega JF,Prieto-Palomino MA,Garcia-Caballero M,Galeas-Lopez JL,Quesada-
14、Garcia G,Baguley IJ.Paroxysmal sympathetic hyperactivity after traumatic brain injury:clinical and prognostic implications.J Neurotrauma.2012;29(7):1364-70.诊断标准诊断标准争议争议 Baguley等以具有上述等以具有上述7项中的项中的5项作为诊断依据。项作为诊断依据。Blackman等拟定了更为严格的诊断标准等拟定了更为严格的诊断标准,要求有严重脑损伤要求有严重脑损伤(Rancho Los Amigos量表认知功能量表认知功能)、体温、体温
15、 38.5&、脉搏脉搏130次次/m in、呼吸、呼吸 20次次/m in、躁动、多汗、肌张力、躁动、多汗、肌张力障碍障碍,上述症状每天最少发作上述症状每天最少发作1次、持续最少次、持续最少3 d,并排除其他并排除其他疾病。疾病。Rabinstein认为该标准过于严格认为该标准过于严格,漏诊的患者会因得不到相应漏诊的患者会因得不到相应处理而对预后不利处理而对预后不利。鉴别诊断鉴别诊断 需要与感染(尤其是颅内感染)、间脑癫痫、颅内压升高(减压窗膨出、脑脊液压力升高)、抗精神病药物引起的恶性综合征(使用多巴胺受体阻滞剂或激动剂)、抗抑郁药引起的5-羟色胺综合征、脊髓损伤(T6 8以上)后自主神经反
16、射异常(尤其合并脑外伤时)、脑外伤后精神障碍、恶性高 热、麻醉药物戒断、药物撤离综合征(如巴氯芬的减量过快或突然撤药)等鉴别。而当与上述疾病交织存在时诊断更加复杂,但上述疾病应首先给予排除以免延误病情处理。Baguley IJ,Heriseanu RE,Cameron ID,Nott MT,Slewa-Younan S.A Critical Review of the Pathophysiology of Dysautonomia Following Traumatic Brain Injury.Neurocrit Care.2008;8(2):293-300.下丘脑自主神经功能损伤或与皮质、
17、皮质下、脑干下丘脑自主神经功能损伤或与皮质、皮质下、脑干 神经核团联系中断神经核团联系中断;交感、副交感平衡失调交感、副交感平衡失调;Disconnection theories suggest that Dysautonomia follows the release of one or more excitatory centres from higher centre control 脑干和间脑在失去皮质、皮质下结构控制后的释放现象脑干和间脑在失去皮质、皮质下结构控制后的释放现象disconnection theory,the Excitatory:Inhibitory Ratio(EI
18、R)Model,suggests the causative brainstem/diencephalic centres are inhibitory in nature,with damage releasing excitatory spinal cord processes.可能的机制可能的机制 anatomical and physiological evidence suggests that Dysautonomic paroxysms are more consistently associated with mesencephalic rather than dienceph
19、alic lesions paroxysmal episodes can be triggered by environmental events and minimised by various but predictable neurotransmitter effects.excitatory:inhibitory ratio(EIR)SEI,spinal excitatory:inhibitory centre;BEI,brainstem excitatory:inhibitory centre;MC,motor centres;+/,excitatory/inhibitory pat
20、hways.Opiate and dopaminergic pathways：Morphine settled both hyperdynamic cardiac function and posturing；bromocriptine decreased temperature and sweatingclonidine controlled blood pressure but did not obviously affect either the number of Dysautonomic episodes or the subjects temperaturepropanolol d
21、ecreases circulating catecholamines,and reduces both cardiac work and catabolic driveGABA agonist baclofen；ITB acts on inhibitory interneurons in the spinal cord,gabapentin(GABA 2)appeared to reduce the number and severity of paroxysms and allowed an overall reduction in other medications,including
22、ITB,without a recurrence of symptoms典型病例典型病例 病例简介：男，27岁，外伤致左额硬膜下血肿清除术后16天，睁眼昏迷(VS)，GCS 4分。PSH表现：呼吸 心率 大汗 体温40 肌紧张 血压 1次/46h。处置：地西泮，氯硝安定，氯丙嗪/异丙嗪，巴氯芬，加巴喷丁，普萘洛尔，溴隐亭，冰盐水滴注加降温毯 *2.5monthPSH逐渐好转,随着原发病情改善自发缓解的过程治疗治疗-对症对症躁动躁动、激惹激惹明显时给予氯丙嗪25 50mg或氟哌啶醇5 10mg肌肉注射、地西泮5 10 mg 静脉注射以迅速控制症状,然后氟哌啶醇2 4 mg 或奋乃静2 8mg口服
23、,每日3次。多汗多汗症状往往在其他症状控制后可明显改善,必要时给予中成药或方剂煎服。高热高热通常采用物理降温,亦可予布洛芬混悬液(美林)15 30m l或吲哚美辛(消炎痛)6.25 25 mg 口服,每日3次;持续的中度热可予溴隐停口服,效果好;心动过速心动过速给予受体阻滞剂普萘洛尔;血压升高血压升高按照原发性高血压病常规降压治疗,病情改善后血压可逐渐恢复正常;肌张力障碍肌张力障碍是最难解决的问题,巴氯芬或者加巴喷丁效果一般。氯硝西泮1 2mg口服,每日2 3次,效果良好,但部分患者迅速出现耐受,需加大剂量,但可降低患者的觉醒水平,加重意识障碍;发作性呼吸加快发作性呼吸加快往往随其他症状控制而缓解。减少激发因素减少激发因素治疗治疗-治本治本 原发脑损伤的恢复 继发脑积水的处理 全身并发症的防治自限性？自限性？重视重视PSH是根本是根本 正确、早期认识PSH 尽早干预改善预后
