小儿神经系统疾病课件

1、小儿神经系统疾病1neonatology: hypoxic-ischemic encephalopathy, hie小儿神经系统疾病2main contents lclinical definitionletiology/high risk factors lpathogenesis and pathophysiologylclinical manifestations and diagnostic neuroimaging lprognosislclinical management 小儿神经系统疾病3clinical definition brain damage in fetus and

2、 neonates caused by hypoxic and/or decreasing or abruption of blood flow to brain during perinatal period.小儿神经系统疾病4etiologyalmost all the factors causing asphyxiaresulting hie, and maternalplacenta and umbilicus abnormality substantial pulmonary, cardiac and cns disease of the fetus and neonatespron

3、ged partum medication during delivering 小儿神经系统疾病5high risk factors prolonged fetal bradycardia repeated late decelerations low apgar scores at 5 minutes or later low fetal scalp or cord ph requirement for prolonged resuscitation with positive-pressure ventilation 小儿神经系统疾病6pathogenesis and pathophysi

4、ology change of cerebral blood flow normal term stable cbf: 50-60ml/min/100g cbf 20ml/min /100g, brain damage小儿神经系统疾病7pathogenesis and pathophysiology change of cerebral metabolism increase in anaerobic glycolysis na +, ca2 + pump function intracellular atp exhausted na +, ca2 + endosmosis irritabil

5、ity amino acid blocking oxidative phosphorylation in mitochondrion blood stream reperfusion oxygen free radical 小儿神经系统疾病8pathogenesis and pathophysiology change of nuropathology term baby: cortex infarction gray matter in partes profunda necrosis preterm: intraventricular haemorrhage white matter in

6、jury cerebral inflammationil-1, tnf- , cks cellular apoptosis 小儿神经系统疾病9clinical manifestations mild excitation/ irritability apparent at 24 hr no convulsion normal eeg小儿神经系统疾病10clinical manifestations moderate convulsion, 50% with disorder of consciousness apparent at 24-48 hr deterioration: intensi

7、ty of anterior fontanelle coma小儿神经系统疾病11clinical manifestations severe light coma or coma at birth irregular respiration and apnea convulsion with 12 hr poor muscle tone intensity of anterior fontanelle most die in 1 week survivors with severe nerosequelees 小儿神经系统疾病12hie的诊断的诊断临床表现临床表现 1.1. 胎儿宫内窒息史，严

8、重的胎儿宫内窘迫表现胎儿宫内窒息史，严重的胎儿宫内窘迫表现 ( (胎心胎心100100次，持续次，持续5 5分钟以上；和分钟以上；和/ /或羊水或羊水iiiiii度污染度污染) )2.2. 出生时有重度窒息出生时有重度窒息: :（apgarapgar评分评分1 1分钟分钟 3 3分）分） 至至5 5分钟时仍分钟时仍 5 5分；或出生时脐动脉血气分；或出生时脐动脉血气ph 7.00ph 7.00 ；3 3、出生后、出生后24 24 小时内出现神经系统表现；小时内出现神经系统表现；4 4、排除低钙血症、低糖血症、感染、产伤和颅内出血等引、排除低钙血症、低糖血症、感染、产伤和颅内出血等引 起的抽搐，

9、以及遗传代谢性疾病和其他先天性疾病所引起的抽搐，以及遗传代谢性疾病和其他先天性疾病所引 起的神经系统疾患。起的神经系统疾患。同时具备以上同时具备以上4 4条者可确诊，第条者可确诊，第4 4条暂时不能确定者作为条暂时不能确定者作为 拟诊病例。拟诊病例。中华医学会儿科学会新生儿学组中华医学会儿科学会新生儿学组 2004年年11月修订月修订; 长沙长沙小儿神经系统疾病13hie的诊断的诊断脑电图脑电图 在生后在生后1周内检查周内检查 脑电图异常程度与临床分度基本一致脑电图异常程度与临床分度基本一致 脑电图异常表现：脑电图异常表现： 脑电活动延迟脑电活动延迟 (落后于实际胎龄落后于实际胎龄)， 背景活

10、动异常背景活动异常 (以低电压和爆发抑制为主以低电压和爆发抑制为主) 振幅整合脑电图振幅整合脑电图 (aeeg)中华医学会儿科学会新生儿学组中华医学会儿科学会新生儿学组 20042004年年1111月月 长沙修订长沙修订小儿神经系统疾病14hie的诊断的诊断影象学检查影象学检查 头颅头颅b超超 可在可在hie病程早期病程早期 (72小时内小时内) 开始检查开始检查 有利于了解脑水肿、基底神经节丘脑损伤有利于了解脑水肿、基底神经节丘脑损伤 和脑动脉梗死等病理改变和脑动脉梗死等病理改变 ct 生后生后4-7天为宜天为宜 mri 对对hie病变性质与程度评价方面优于病变性质与程度评价方面优于ct中华

11、医学会儿科学会新生儿学组中华医学会儿科学会新生儿学组 2004年年11月修订月修订; 长沙长沙小儿神经系统疾病15uscerebraledemaneuroimaging 小儿神经系统疾病16ctmricerebral edemaneuroimaging 小儿神经系统疾病17 injury in hypothalamus and basal gangliausneuroimaging 小儿神经系统疾病18ctinjury in hypothalamus and basal ganglianeuroimaging 小儿神经系统疾病19 iinjury in area adjacent to the

12、 sagittalneuroimaging 小儿神经系统疾病20cerebral arteryinfarction in terms早期回声增强usneuroimaging 小儿神经系统疾病21cerebral arteryinfarction in terms ineuroimaging 小儿神经系统疾病22pvl in prematureusneuroimaging 小儿神经系统疾病23pvl in prematuremrineuroimaging 小儿神经系统疾病24punctate encephalon haemorrhagemrineuroimaging 小儿神经系统疾病25seve

13、rity and diagnosis mild irritability, normal tone.moros: ; sucking: normalnormal respiration，no convulsion moderate oppressed，muscle tone ，moros and sucking convulsion。7-10d, may have sequelae severecoma，frequently convulsionirregular respiration or apnea. respiration failure. very high death rate s

14、urvivors usually have sequelae中华医学会儿科学会新生儿学组中华医学会儿科学会新生儿学组 2004年年11月修订月修订; 长沙长沙小儿神经系统疾病26prognosis mild and moderate recovered 7d,or severe worse outcome小儿神经系统疾病27clinical management for an asphyxiated newborn: immediate maintenance of ventilation and perfusion control of seizures maintenance of met

15、abolic homeostasis, especially blood glucose levels to avoid additional cerebral insult 小儿神经系统疾病28clinical management maintenance of adequate ventilation: avoidance of hypoxemia and hypercapnia to avoid systemic hypotension cerebral perfusion prevention of fluid overload: current data in human newbo

16、rns do not provide convincing evidence that supports the use of antiedema therapy maintenance of normoglycemia 小儿神经系统疾病29clinical management control seizures begin with a loading dose of phenobarbital (20mg/kg) ,iv followed by additional 5-mg/kg, total dose 40 mg/kg for refractory seizures: lorazepa

17、m by iv may be indicated recent recommendations emphasis: brief duration of treatment; possible deleterious effects of anticonvulsants on the developing nervous system. 小儿神经系统疾病30clinical management cool cap (selective head hypothermia therapy) multi-center trial： us, canada, uk and new zealand: 25

18、sample: trial/control=116/118 apgar=6/5min+cord arterial ph 7.1 clinical hie+eeg abnormal aeeg severe: (n=46)：not effective aeeg moderate : (n=172); showed protective gluckman pd, cool cap trial group. lancet 2005小儿神经系统疾病31clinical management cool cap (selective head hypothermia therapy) aeeg modera

19、te : (n=172); showed protective death rate: severe neromotion disabled 48% vs 66%p=0.02 bayley mdi: 85 vs 77 p=0.04 bayley pdi: 90 vs 85 p=0.047gluckman pd, cool cap trial group. lancet 2005小儿神经系统疾病32clinical management whole body hypothermia nih neonatal network,us multi-center：16, sample：208 results; death: 2