脑白质病变精选PPT.ppt
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1、关于脑白质病变第1页,讲稿共48张,创作于星期三脑白质病变定义脑白质病变定义脑白质病变病因脑白质病变病因血管源性白质病变血管源性白质病变白质病变与认知损害白质病变与认知损害第2页,讲稿共48张,创作于星期三脑白质病变临床常见,多种疾病累及神经系统白质脑白质病变临床常见,多种疾病累及神经系统白质分原发、继发性两大类分原发、继发性两大类.病理过程:髓鞘形成延迟、髓鞘形成障碍、脱髓鞘病理过程:髓鞘形成延迟、髓鞘形成障碍、脱髓鞘诊断:临床诊断:临床+MRI+病理病理脑白质病变Ann N Y Acad Sci.Oct 2008;1142:266309.第3页,讲稿共48张,创作于星期三Genetic2
2、2Demyelinative3 3Infectious4 4Inflammatory5 5Toxic Etiology of CWM Disorders6 61 17 78 89 910101 11 1MetabolicVascularTraumaticNeoplasticHydrocephalicDegenerative第4页,讲稿共48张,创作于星期三Genetic DiseasesLeukodystrophies(e.g.,adrenoleukodystrophy,metachromatic leukodystrophy,globoid cell leukodystrophy)脑白质营养
3、不良(肾上腺、异染性、球样细胞脑营养不良)Vanishing white matter disease白质消融性脑白质病Alexanders disease亚历山大病Adult-onset leukodystrophy with neuroaxonal spheroids神经轴索球样细胞脑白质营养不良Mitochondrial encephalopathy with lactic acid and stroke(MELAS)线粒体脑肌病伴乳酸酸中毒和卒中样发作综合征Fragile X tremor-ataxia syndrome(FXTAS)脆性X相关震颤和(或)共济失调综合征Aminoaci
4、durias(e.g.,phenylketonuria)氨基酸尿(如苯丙酮尿症)Phakomatoses(e.g.,neurofibromatosis)斑痣性错构瘤病Mucopolysaccharidoses粘多糖贮积病Myotonic dystrophy强直性肌营养不良Callosal agenesis新生儿胼胝体发育不良第5页,讲稿共48张,创作于星期三MRI of X-ALD,MLD,GLD,VWMD(A)X-连锁的肾上腺脑白质营养不良(X-ALD),T1 image;(B)异染性脑白质营养不良(MLD),FLAIR image;(C)球形细胞脑白质营养不良(GLD),T2-weight
5、ed image;(D)白质消融性脑白质病(VWMD),T1-weighted image.X-ALD:impaired psychomotor speed,spatial cognition,memory,and executive functions;MLD:psychosis,behavioral disturbances,and dementia;GLD:hemiparesis,intellectual impairment,cerebellar ataxia,and visual failure;Ann N Y Acad Sci.Oct 2008;1142:266309.第6页,讲稿
6、共48张,创作于星期三神经轴索球样细胞脑白质营养不良 (A)FLAIR MRI in the axial plane showing confluent high signal in the periventricular,deep,and subcortical white matter of the frontal and parietal lobes extending through the splenium of the corpus callosum.(B)Gross pathology of a coronal section of the cerebral hemisphere
7、,showing gliosis in the centrum semiovale(arrow)and internal capsule(arrowhead).The disorder usually presents with executive system dysfunction and other neurobehavioral deficits,progressing to dementia.第7页,讲稿共48张,创作于星期三MRI in patients with MELAS Criteria for diagnosis are stroke like episodes befor
8、e age 40(not confined to vascular territories);encephalopathy characterized by seizures,dementia,or both;with lactic acidosis and/or ragged-red fibers.Recurrent headache or vomiting may be present.Periventricular and diffuse WM hyperintensities,as well as areas of cortical infarction and cerebral ed
9、ema,are seen on MRI第8页,讲稿共48张,创作于星期三脆性X相关震颤和(或)共济失调综合征 White matter pallor is seen in the cerebellar parenchyma(A),as well as in the middle cerebellar peduncles(B).Progressive cognitive decline is characterized by impaired executive function,working memory,intelligence,declarative learning and memor
10、y,information processing speed,temporal sequencing,and visuospatial functioning,but language is spared.第9页,讲稿共48张,创作于星期三Demyelinative DiseasesMultiple sclerosisAcute disseminated encephalomyelitis 急性播散性脑脊髓炎Acute hemorrhagic encephalomyelitis 急性出血性脑脊髓炎Schilders disease 谢耳德氏病,弥漫性轴周性脑炎Marburgs disease
11、马尔堡病Balos concentric sclerosis 同心圆性硬化第10页,讲稿共48张,创作于星期三FLAIR MRI in multiple sclerosis(A)White matter hyperintensity perpendicular to the lateral ventricle(Dawsons finger),shown by the arrow.(B)In a second case,the focal area of hyperintensity(arrow)corresponded to the initial clinical presentationC
12、ognitive impairments in MS also include a wide range of focal neurobehavioral syndromes and neuropsychiatric disturbances第11页,讲稿共48张,创作于星期三MRI features of ADEM(A)Coronal T1-weighted postgadolinium image showing enhancing lesions in the right more than left hemispheres.(B)Axial zero-B MRI demonstrati
13、on of the multiple lesions.(C)FLAIR MRI 6 months after marked clinical recovery shows much improved areas of hyperintensity.第12页,讲稿共48张,创作于星期三Infectious diseaseHIV and AIDS dementia complex 艾滋病痴呆综合征Progressive multifocal leukoencephalopathy 进行性多灶性白质脑病Subacute sclerosing panencephalitis 亚急性硬化性全脑炎Prog
14、ressive rubella panencephalitis 进行性风疹全脑炎Varicella zoster encephalitis水痘-带状疱疹病毒性脑炎Cytomegalovirus encephalitis 巨细胞病毒脑炎Lyme encephalopathy 莱姆脑病第13页,讲稿共48张,创作于星期三 HIV FLAIR MRI showing hyperintensities in prefrontal white matter in a patient with HIV and cognitive impairmentSchmahmann JD,Pandya DN.Fibe
15、r Pathways of the Brain.Oxford University Press;New York:2006第14页,讲稿共48张,创作于星期三PML(A)T2-WI shows involvement of white matter of the right occipital region(arrow),accounting for the hemianopsia in this HIV-positive patient.(B)FLAIR MRI in a patient with systemic lymphoma and PML,demonstrating conflue
16、nt prefrontal white matter lesion spreading across the genu of the corpus callosum(arrow),and additional lesions affecting local association fibers of the right prefrontal and parieto-occipital cortices(arrowheads).(C,D)Axial FLAIR images in an HIV-positive patient showing confluent subcortical and
17、deep white matter involvement by PML.Cognitive presentations include frontal lobe syndromes and aphasia,progressing to quadriparesis,mutism,and unresponsiveness第15页,讲稿共48张,创作于星期三Autoimmune Inflammatory DiseaseSystemic lupus erythematosus 系统性红斑狼疮Behcets disease 白塞病,贝西氏病,贝赫切特病Sjgrens syndromeWegeners
18、granulomatosiTemporal arteritis 颞动脉炎Polyarteritis nodosa 结节性多动脉炎Scleroderma 硬皮病Isolated angiitis of the central nervous system 孤立的中枢神经系统血管炎Sarcoidosis 结节病第16页,讲稿共48张,创作于星期三Toxic DiseasesCranial irradiation 颅脑照射Therapeutic drugs(e.g.,methotrexate甲氨蝶呤,BCNU卡莫司汀,cyclophosphamide环磷酰胺)Drugs of abuse(e.g.,
19、toluene甲苯,heroin海洛因)Toluene:a dramatic syndrome of dementia,ataxia,and other neurologic signsAlcohol(MarchiafavaBignami disease又称胼胝体变性,一种慢性酒精中毒性疾病)Environmental toxins(e.g.,carbon monoxide)第17页,讲稿共48张,创作于星期三Toxic LeukoencephalopathyFLAIR MRI in the axial plane of a patient with cognitive decline aft
20、er receiving methotrexateT2-W MRI appearance in the axial plane of toluene encephalopathy in two patients a dramatic syndrome of dementia,ataxia,and other neurologic signs第18页,讲稿共48张,创作于星期三Heroin Clinical manifestations include cerebellar motor findings of ataxia,dysmetria and dysarthria,bradykinesi
21、a,rigidity,and hypophonia,and the syndrome may progress over weeks to pseudobulbar palsy,akinetic mutism,decorticate posturing,and spastic quadriparesis.MRI scans after heroin inhalation,known colloquially as“chasing the dragon.”FLAIR images in the axial plane(AD).Inhalation of heated heroin vapor(c
22、olloquially termed“chasing the dragon”)produces a devastating,progressive spongiform leukoencephalopathy.第19页,讲稿共48张,创作于星期三Metabolic DiseasesCobalamin deficiency VitB12缺乏Folate deficiency 叶酸缺乏Central pontine myelinolysis 桥脑中央髓鞘溶解症Hypoxicischemic injury 缺氧缺血性脑损伤Posterior reversible encephalopathy syn
23、drome 可逆性后部白质脑病综合征Hypertensive encephalopathy/eclampsia 高血压脑病/惊厥High-altitude cerebral edema 高原脑水肿第20页,讲稿共48张,创作于星期三Hypoxic ischemic encephalopathyAxial MRI in delayed leukoencephalopathy after hypoxicischemic insult.(A)FLAIR image shows extensive,symmetric white matter hyperintensities with relativ
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