8年制病理学英文课件：16神经系统(95页PPT)

1、Chapter 16：Disease of Central Nervous System第1页，共95页。Introduction 1.Delicate structure, more than 50% human genes are neuronal related：complex or arcane2.Lesions may have location indication( selective dysfunction) silent area3.Dual influences of some structures, such as skull and dura, protection o

2、f brain and may facilitate increased intra-cranial pressure.4. Special disease :degenerative disease, demyelination disease, psychiatric diseases less understanding Congenital anomalies: high incidence第2页，共95页。Basic Changes of Cells Neuron（神经元）第3页，共95页。Basic Changes of Cells Neuron（神经元）1.Central Chr

3、omatolysis (中央性尼氏小体溶解) Cause： axonal injury, Viral infection, deficiency of Vit.B, anoxia. Morphology: dispersion of the central Nissal substance and swelling of the neuronal body Sequalae: In early stage, increased dissociated ribosome from RER may facilitate protein synthesis. The change would be

4、reversible, if cause abolished. Insistent change may lead to neuronal death.第4页，共95页。5Central Chromatolysis第5页，共95页。2.Ischemic Changes （Acute Necrosis） Cause：ischemia, anoxia, hypoglucemia, lower blood pressure, epilepsy Morphology：vacuolation, red neuron Basic Changes of Cells Neuron 第6页，共95页。7red

5、neuron第7页，共95页。Simple Degeneration (单纯性萎缩） Cause: uncertain, seen in degenerative Dis. Morphology: neuronal progressive, chronic death, without glial reactionBasic Changes of Cells Neuron 第8页，共95页。Neurophagia(嗜神经元现象） dead neuron engulfed and phagocytosed by M.Satellite Phenomenon(卫星现象) more than 5 O

6、ligodendrocytes surrounding one neuron uncertain significanceBasic Changes of Cells Neuron 第9页，共95页。Inclusion Bodies (包涵体) Rabies: Negri body diagnostic hallmark of rabies HSV; Encephalitis B Jap.virus; Poliovirus Parkinson Dis.： Lewy body Basic Changes of Cells Neuron 第10页，共95页。11Rabies第11页，共95页。12

7、Lewy body第12页，共95页。Wallerian Degeneration (华勒变性） Basic Changes of Cells Neuron 第13页，共95页。Senile Plaque (老年斑)： the core composed with -amyloid protein, surrounded by a halo and swollen degenerative axons Neurofibrillary Tangle (神经原纤维缠结):the tangle composed by double spiral strands of neurofibils with

8、 abnormal phosphorylated tau protein marker of dying neuron seen in Alzheimers Dis., boxer brain, post-encephalitis, ParkinsonismBasic Changes of Cells Neuron 第14页，共95页。15Senile Plaque第15页，共95页。16Neurofibrillary Tangle第16页，共95页。Basic Changes of Cells Astrocyte（星形胶质细胞）Hypertrophy：The cytoplasm is sho

9、wn with HE stain.The cell and its nuclei are enlarged with binuclei, multinuclei or bizarre nucleiSeen in local anoxia, edema, infarct and at the periphery of abscess or tumor. Proliferation ： reactive: repair process after insults, forming glial scar.Corpora amylacea(淀粉样小体）: with age glycoprotein-r

10、ich material 第17页，共95页。Basic Changes of Cells Oligodendrocyte (少突胶质细胞 )Leucodystrophy (白质营养不良) myelin sheath formation disturbance different congenital enzyme deficiency different type of leukodystrophyDemyelination (脱髓鞘病变) formed myelin sheath destroyed due to allergy, anoxia or toxification第18页，共9

11、5页。19Demyelination第19页，共95页。20Demyelination第20页，共95页。 Ependymal cells (室管膜细胞) Silence, Oncogenesis, Deficiency after injury may repaired by As, forming so called granular ependymitis (颗粒性室管膜炎)Basic Changes of Cells 第21页，共95页。Microglia (小胶质细胞） Resting microglia may activated and turn into M Focal pro

12、liferation forming microglial nodule Rodlike microglia seen in advanced syphilis第22页，共95页。23Demyelination第23页，共95页。Common Complications脑水肿 （Brain Edema)Morphology: brain volume, weight, narrow sulci, widened gyri,cutting surface showed small ventricle, increased reflection. Herniation may ensure.第24

13、页，共95页。Common Complications脑水肿 （Brain Edema)Increased water contents within brain parenchymaCause: anoxia,infarction,inflammation, injury, toxification and tumor.Mechanism :Vasogenic: disrupted normal BBB, allowing increasing permeability and fluid escaping from vessels into the interstitial space o

14、f brain (interstitial edema) white mattergray matter2. Cytotoxic: cytomembranous pump (ATPase) leading to irons and water accumulation in cells ( intracellular edema) white matter=gray matter 3. Usually mixed type第25页，共95页。Common complications Hydrocephalus （脑积水) Accumulation of excessive CSF with v

15、entricular dilatation 第26页，共95页。27第27页，共95页。Over-secretion of CSF(tumor of choroid plexus) Absorption disturbances of CSFNoncomunicating type (obstructive)：obstruction occurs in ventricular system, e.g. tumor, inflammatory, adhesion, hemorrhage, or deformity in III ventricle.Communicating type: obst

16、ruction exists in CSF circulation , but out of ventricles . It may caused by meningitis, subarachnoid hemorrhage, with subsequent organization, scar fomattion of arachnoid granulation or Villi.Cause & Pathogenesis第28页，共95页。Morphology: Dilation of ventricles with atrophy of parenchyma of brain, due t

17、o compression of CSF.CPC: headache, vomiting, edema of papilloedema of optic N.Common complications Hydrocephalus （脑积水) 第29页，共95页。Common complicationHypertension of intracranial pressure(ICP) and Herniation (颅内压升高和脑疝形成）The CSF pressure more than 2kPa( normally 0.6-1.8kPa) with lateral recumbent posi

18、tion 第30页，共95页。Cause：（1）cerebral edema, hydrocephalus（2）occupying lesion: tumor, hemorrhage, hematoma（3）inflammation: meningitis,cerebral abscess, encephalitis（4）brain infarctionThe factors influence the results:（1）the size of the lesion and its development rapidity.（2）existed cranial cavity situati

19、onsenile atrophy or unclosure of fontanelle allowing more space for expending of brainCommon complications Hypertension of ICP & Herniation第31页，共95页。Sequalae： （1）headache, vomiting, papilloedema, coma, death （2）herniationSubfalcine (cingulate gyrus) herniation：local tissue hemorrhagic and necrotic,

20、weakness and sensory dysfunction of leg2) Transtentorial (uncal gyrus) herniation：ipsilateral III N compressed leading to pupils constricted dilatedKernohan incisionparalysis of ipsilateral extremities(false localization sign)periaquaductal hemorrhageTonsillar herniation, life-threatening press resp

21、iration centers in medulla oblongata sudden deathCommon complications Hypertension of ICP & Herniation第32页，共95页。33第33页，共95页。34Transtentorial (uncal gyrus) herniation第34页，共95页。35Tonsillar herniation第35页，共95页。Hemodynamic Derangement & Cerebral Vascular Disorders第36页，共95页。Hemodynamic Derangement & Cere

22、bral Vascular DisordersCirculation disturbances: ischemic encephalopathy infarction( thrombotic or embolitic) hemorrhageVascular disorders: arterosclerosis, atherosclerosis, arteritis, aneurysm, ateriovenous malformation(AVM)第37页，共95页。Cerebral injury caused by hypertension, cardiac arrest,hemorrhage

23、 and shock.Predisposing factors：higher metabolic rate：more susceptible NeuronAsOligoEndo Gray MatterWhite Matter 3rd、5th、6th layers of cortex are most vulnerableHemodynamic Derangement & Cerebral Vascular DisordersIschemic Encephalopathy第38页，共95页。Peresistence and severity of ischemia mild ischemia:

24、no remarkable change severe ischemia, survive few hours before death: not remarkable moderate ischemia, survive more than 12 hours：typical changesArchitecture of cerebral arteries the location at the border zone of cerebral arteries is much more vulnerable.Hemodynamic Derangement & Cerebral Vascular

25、 DisordersIschemic Encephalopathy第39页，共95页。40第40页，共95页。Changes:laminar cortical necrosis : neurons in 3rd, 5th, 6th layers of cortex involvedhippocampal sclerosis ：pyramidal neuron deathborder zone infarction ： early stage： “C” shaped infarct later stage: astrogliosis (granular atrophy) cardiopetal

26、developmentglobal necrosis(respirator brain) CPC：weakness sensation abnormalities coma, vegetable status deathHemodynamic Derangement & Cerebral Vascular DisordersIschemic Encephalopathy第41页，共95页。Laminar Cortical NecrosisHemodynamic Derangement & Cerebral Vascular DisordersIschemic Encephalopathy第42

27、页，共95页。Fresh border zone infarctGranular atrophyCutting surface of granular atrophyRespirator brainHemodynamic Derangement & Cerebral Vascular DisordersIschemic Encephalopathy第43页，共95页。44Respirator brain第44页，共95页。Cause： thrombosis, embolism, space occupying lesion, local vessels compressed by hernia

28、Types: thrombotic: on the sites of atherosclerosis inner carotid A,basilar A, cerebral arteries, post-communicating A, superior cerebellar A. insidious and gradual development the symptoms: from weakness of muscles to semiplegia or comaembolic: the emboli often are cardiogenic , or from atherosclero

29、tic plaque, with sudden onset and poor prognosis.Hemodynamic Derangement & Cerebral Vascular DisordersCerebral Infarction第45页，共95页。The most common form of cerebrovascular disease, accounting for 70%80% of all cerebralvascular accidents “stroke”Changes: extent of ischemia: Occlusion in inner carotid

30、artery: circle willis may compensate completely, no infarctionOcclusion in mid-size artery: as middle cerebral A, the infarct smaller than its supply area due to partial anastomosis.Occlusion in terminal arteries: leading to sudden area infracted.Hemodynamic Derangement & Cerebral Vascular Disorders

31、Cerebral Infarction第46页，共95页。Types: white infarctred infarct: incomplete occlusion or frangible emboli going further to small vessels, resulting in blood escape from injured vascular wall.Morphology changes: first 412h: normal then: ischemic neuronal changes 36-48h: swollen and soft; demarcation bet

32、ween gray and white matter becomes blurred due to edema the third day: macrophage, progressive marked demarcation of the lesion 1 month: liquefaction, irregular cavities 6 month: completely liquefied 第47页，共95页。Brain infarctionlacunae: necrosis less than 1.5cm in diameter.TIAs( transient ischemic att

33、acks) transient episode of neurologic dysfunction lasting several minutes24 hours an important predictor of subsequent infarcts 1/3 patients with TIA developing clinically significant infarcts within 5 years/第48页，共95页。Hemodynamic Derangement & Cerebral Vascular DisordersBrain HemorrhageIntracerebral

34、 HemorrhageCause: hypertension * congenital saccular aneurysms, tumors, hemorrhagic diathesis, vasculitis, AVM, traumaPathogenesis:anoxia of vascular wallanoxia of perivascular tissue Charcot Bounchard microaneurysmsmicro-softening focivessels rupturedspasm of vessels B.P hemorrhage第49页，共95页。Changes

35、: In the center of foci, normal structure is destroyed and filled with RBC，at periphery multifoci of hemorrhage The old hemorrhage foci becomes cavitated & with hemosiderin.Hemodynamic Derangement & Cerebral Vascular DisordersBrain Hemorrhage第50页，共95页。CPC：B.G hemorrhage:directed to insular contralat

36、eral semiplegia directed to ventricle , thalamus deathPons hemorrhage: pin-like pupils, persistent high fever or sudden deathCerebellar hemorrhage: severe occipital headache, frequent vomitingHemodynamic Derangement & Cerebral Vascular DisordersBrain Hemorrhage第51页，共95页。Subarachnoid hemorrhageThe mo

37、st common cause of spontaneous(nontraumatic) Rupture of a saccular(berry) aneurysm approximately 1% of the general population different from the fusiform dialtion in atheroslcerosis or infectious (mycotic) aneurysm 80% arise at the arterial bifurcations in the territory of the internal carotid arter

38、y: MCA, ACMDeveloped the infarct of brain parenchyma第52页，共95页。CPC: Abrupt, severe headache, vomitting, loss of consciousness Meningeal signs Bloody CSF 50% died in several days acute hydrocephalus herniation brain infarction chronic: hydrocephalus第53页，共95页。Vascular malformationAbnormalities in angio

39、genesis in the developing brain AVM: the most common caused vessels of variable caliber including A,V Hemorrhagic, calcification, reactive gliosis第54页，共95页。Tumors of CNSAstrocytoma（星形胶质细胞瘤)The most common categories of brain tumors in CNS Gliomas shares 40% of brain tumors，astrocytoma shares 70% of

40、gliomasMost astrocytomas are of diffuse infiltrativeAstrocytomas in Children and Adults Location differentiation demarcation often in brain stemcerebellum beneathtentoriumwell, gelatinousin gross appearanceChildrenpoormost often above tentorium in hemispheresrelatively poor granular in gross appeara

41、ncewellAdults第55页，共95页。Morphology: pilocytic astrocytoma: common in children , elongated processes extend from two poles( grade I)fibrillary astrocytoma and cytoplasmic astrocytoma : minic their original astrocytes(grade II)gemistocytic astrocytoma: (grade IIIII)anaplastic astrocytoma: (grade III)gl

42、ioblastoma multiform: ( grade IV) GFAP(+)Tumors of CNSAstrocytoma（星形胶质细胞瘤)第56页，共95页。The tumor originates from menigothelium of arachoid granules villa or fibroblasts. Its grows outside the brain, pressing the brain parenchyma and may be resected complete.Grossly: tumor shows spherical or lobulated,

43、expanding in growthHistology: Menigothelial or syncytial type Fibroblastic variants Transitional type OthersPrognosis: most benign, a few (15%) recurs after resection, few undergoes malignant transformation EMA(+) Vimentin(+)Tumors of CNS Meningioma （脑膜瘤 )第57页，共95页。Embryogenic tumor, malignant, most

44、ly seen in children under ten with poor prognosisThe tumor originates from primitive neuroecdermal cells of vermis or out layers of granular cells of cerebellum.The tumor shows whitish pink or gray in color, located at IV ventricle or cerebellar hemisphere.The cells are small, primitive with scanty

45、cytoplasm. The nuclei are round or carrot-shaped with frequent mitoses. Sometimes, may surround a fibrillary core having rosette formationMutual differentiation : GFAP （+） NF （+）Tumors of CNS Meduloblastoma （髓母细胞瘤)第58页，共95页。The benign tumor originates from schwann cell, often located at 8th nerve (a

46、coustic neurilermmoma 听神经瘤）or trunk of peripheral N. Slow growth, very rare malignant transformation Spherical, or lobulated mass, white-gray in color on cut surface, or shows light yellow color when mucinous degeneration occur. quite often cavitation Spindle shaped cells , in whirl or tight arrange

47、ment (Antoni A type) or in reticular arrangement （Atoni B型）Tumors of CNS Schwannoma （神经鞘瘤,施万氏瘤)第59页，共95页。Etiology the disease cause by living pathogens, which are infective , endemic in certain geographic areas and in certain seasons (传染性，流行性，地方性，季节性）Unique route of invasion, a given pathogen has un

48、ique entrance of invasionunique mode of spreading in hostunique affinity for special tissue or organs , causing special pathological changesPathogenesis bacteria: endotoxin and /or exotoxin viruses: cellular and /or humoral immunityInfectious DiseaseCommon Features(共同特性）第60页，共95页。Basic pathologic ch

49、anges: inflammation( acute/chronic) depending on host pathogen host: immunity pathogen: invasion ability, toxins, metabolic substance evocation of allergic reaction of hostClinical course Incubation period: Predromal period: non specific symptoms and signs Dominant period: diagnostic symptoms and si

50、gns peak Recovery period: the disease subsides typical/atypical/subclinical courseInfectious DiseaseCommon Features(共同特性）免疫性第61页，共95页。ConsequencesComplete recovery the host gains temporary or permanent immunityChronic courseRecurrence of diseaseDeathInfectious DiseaseCommon Features(共同特性）第62页，共95页。I

51、nfectious DiseaseCommon Features in CNS(共同特性）Meninges dura 硬脑膜 arachnoid 蛛网膜 pia 软脑膜leptomenige （软脑膜）第63页，共95页。Route of infectionHematogenic: septicema, viremiaLocal disseminated: opened skull fracture, sinusitis, mastoiditisDirect infected:trauma, iatrogenic interference(lumbar puncture)Through per

52、ipheral nervous system: rabies, HSVInfectious DiseaseCommon Features in CNS(共同特性）第64页，共95页。Inflammation featureStereotyped Reaction neurons: degeneration, necrosis secondary demyelination limited exudation with perivascular cuffing formation Presence of BBB (blood brain barrier) and V-R space limits

53、 the spread of inflammation Absence of intrinsic lymphatics and lymphoid tissue T/B cells are blood born(exogenic)glia nodule formation microglial nodule in early stage. astrocytic nodule in later stage, repair第65页，共95页。Pathogens: Pyogenic meningitis: Meningococci, H influenza, Pneumococci, Streptoc

54、occi, Staphylococci, E Coli Granulomatous meningitis: T.B.mycobacterium, Fungi Lymphocytic meningitis: viruses, spirochetesMeningitis第66页，共95页。Epidemic Meningococcic Meningitis(流行性脑膜炎双球菌性脑膜炎)ConceptPathogen: meningococci, epidemic seasons : Winter & SpringBacteria spread by air (sneeze and spray), l

55、ocated at nasopharyx, most are bacteria carrier (15% population, in epidemic season:7080%) Victims are children, most younger than 10yrs oldBasic changes：acute purulent inflammation (Leptomeninge & CSF )Clinical symptoms & signs: fever,headache, vomiting, petechia & ecchymosis on the skin & mucosa,

56、meningeal irrigative signs, shock in severe casesTemporary immunity after recovery第67页，共95页。MeningitisPathogen: meningococci, endotoxin and capsule are pathogenicRoute of infection:Spray from carrierMucosa of nasopharyxBlood (septicemia or bacteremia)leptomenigesmeningitisUsually 5%30%,7080% in epid

57、emic periodURI, catarrh(sore throat red and edematous mucosa, mucous discharge)Petechia ecchymosisBacterial emboliVascular paralysis, dilation, thrombosisshockbilateral severe hemorrhage of adrenal cortex ( Waterhouse- Friderichsen syndrome)(+)第68页，共95页。MeningitisPathological Changes: Vassels: bacte

58、rial thrombi, thrombosis, focal hemorrhage Meninge: acute purulent inflammation Gross: dura: tense, hyperemic Subarachnoid space filled with grayish yellow pus especially at convex of hemisphere at the base in cisternae along circle Willis, roots of cranial & spinal NervesLM： Strikingly enlarged sub

59、arachnoid space with large amount of purulent exudation , mainly polys. Vascular congestion Adjacent brain parenchyma be edematous第69页，共95页。70第70页，共95页。71第71页，共95页。MeningitisClinical Pathological Correlation(CPC)Meningeal irritation signs inflammationSwelling of roots of cranial & spinal NCompressio

60、n at ostiole or intervertebral holesPreventive muscular spasm at back in order to fix the position of nerve root at holesKernigs Sign(+) Brudzinski Sign(+)neck stiffness or opisthotonus第72页，共95页。Meningitis CPC Hypertention of ICPInflammatory exude adhesion of arachnoid granulesdecreasing absorption