脑白质病变专题宣讲

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,上海交通大学从属第六人民医院,付剑亮,脑白质病变,Cerebral White Matter Disease,脑白质病变专题宣讲,第1页,脑白质病变定义,脑白质病变病因,血管源性白质病变,白质病变与认知损害,脑白质病变专题宣讲,第2页,脑白质病变临床常见，各种疾病累及神经系统白质,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,诊疗：临床+MRI+病理,脑白质病变,Ann N Y Acad Sci.Oct;1142:266309.,脑白质病变专题宣讲,第3页,Genetic,2,Demyelinative,3,Infectious,4,Inflammatory,5,Toxic,Etiology of CWM Disorders,6,1,7,8,9,10,1,1,Metabolic,Vascular,Traumatic,Neoplastic,Hydrocephalic,Degenerative,脑白质病变专题宣讲,第4页,Genetic Diseases,Leukodystrophies(e.g.,adrenoleukodystrophy,metachromatic leukodystrophy,globoid cell leukodystrophy)脑白质营养不良（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,Aminoacidurias(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,脑白质病变专题宣讲,第5页,MRI of X-ALD,MLD,GLD,VWMD,(A)X-连锁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-weighted 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;1142:266309.,脑白质病变专题宣讲,第6页,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,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,.,脑白质病变专题宣讲,第7页,MRI in patients with MELAS,Criteria for diagnosis are stroke like episodes before 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 edema,are seen on MRI,脑白质病变专题宣讲,第8页,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 memory,information processing speed,temporal sequencing,and visuospatial functioning,but language is spared.,脑白质病变专题宣讲,第9页,Demyelinative Diseases,Multiple sclerosis,Acute 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 马尔堡病,Balos concentric sclerosis 同心圆性硬化,脑白质病变专题宣讲,第10页,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 presentation,Cognitive impairments,in MS also include a wide range of focal neurobehavioral syndromes and neuropsychiatric disturbances,脑白质病变专题宣讲,第11页,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 demonstration of the multiple lesions.(C)FLAIR MRI 6 months after marked clinical recovery shows much improved areas of hyperintensity.,脑白质病变专题宣讲,第12页,Infectious disease,HIV 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,Progressive 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,脑白质病变专题宣讲,第13页,HIV,FLAIR MRI showing hyperintensities in prefrontal white matter in a patient with HIV and,cognitive impairment,Schmahmann JD,Pandya DN.Fiber Pathways of the Brain.Oxford University Press;New York:,脑白质病变专题宣讲,第14页,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 confluent 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 deep whit