脱髓鞘幻灯2009医大.pptx
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1、Overview Demyelinative disease is a group of diseases of brain and spinal cord in which destruction of myelin is a prominent feature.Myelin sheaths play importance role in protecting and supporting for nervous fiber and transporting of material to neurons and maintaining of neurons ionic environment
2、.The formation of myelin sheaths in both the central and peripheral nervous system follows a similar pattern.Central myelin is formed by an extension of membrane of the oligodendrocyte wrapping the axons,whereas peripheral myelin is formed by the extension of the Schwann cell membrane.The commonest
3、accepted the pathologic criteria of demyelinative disease in CNS:1.Destruction of the myelin sheaths of nerve 1.Destruction of the myelin sheaths of nerve fibers;fibers;2.Relative sparing of other elements of nervous 2.Relative sparing of other elements of nervous tissue,i.e.of axis cylinders,nerve
4、cell,and tissue,i.e.of axis cylinders,nerve cell,and supporting structure,and a relative lack of supporting structure,and a relative lack of Wallerian,or secondary,degeneration off fiber Wallerian,or secondary,degeneration off fiber tracts.tracts.3.A particular distribution of lesions often is 3.A p
5、articular distribution of lesions often is perivenous and primarily in white matter.perivenous and primarily in white matter.Classification of demyelination disease in CNS Dysmyelinative type(leukodystrophy)Dysmyelinative type(leukodystrophy)It It results results from from failure failure to to form
6、 form normally normally constituted constituted myelin,myelin,due due to to genetic genetic enzymatic enzymatic disorders,disorders,such such as as adrenoleukodystrophy adrenoleukodystrophy(ADL),(ADL),Globod Globod leucodystrophy leucodystrophy(Krabbe),(Krabbe),Metachromatic Metachromatic leucodystr
7、ophy(MLDleucodystrophy(MLD)Demyelinating type Demyelinating typeIt is breakdown of normally constituted myelin.It is breakdown of normally constituted myelin.Classifications of the demyelination diseaseMultiple sclerosisMultiple sclerosis1.1.relapsing-remitting,RR relapsing-remitting,RR 2.primary pr
8、ogressive,PP2.primary progressive,PP3.secondary progressive,SP3.secondary progressive,SP4.progressive relapsing,PR4.progressive relapsing,PRNeuromyelitis optica(Devic)Neuromyelitis optica(Devic)Diffuse Diffuse cerebral cerebral sclerosis sclerosis(Schilder)(Schilder)&concentric concentric sclerosis
9、sclerosis of of Balo Balo Acute disseminated encephalomyelitis Acute disseminated encephalomyelitis Central pontine myelinnolysis Central pontine myelinnolysis Multiple sclerosis Multiple sclerosis is the most common demyelinating disorder.It is a disease of the central white matter with“lesions sep
10、arated in time and space”.Generally,MS is characterized clinically by remitting-relapsing course.The disease usually occurs in young adults.The peak age of onset is 20-40 and 95%patients occurs between 10-60 years.More females than males are affected.Etiology and pathogenesisEtiology and pathogenesi
11、s1 1 1 1.Geographical influenceGeographical influenceGeographical influenceGeographical influence:Populations Populations Populations Populations residing residing residing residing between between between between tropical tropical tropical tropical and and and and subtropical subtropical subtropica
12、l subtropical zones zones zones zones have have have have a a a a low low low low risk risk risk risk of of of of MS.MS.MS.MS.All All All All populations populations populations populations residing residing residing residing this this this this latitudes latitudes latitudes latitudes in in in in No
13、rth North North North America and Europe are higher risk.America and Europe are higher risk.America and Europe are higher risk.America and Europe are higher risk.2.2.Familial factors:Familial factors:Familial factors:Familial factors:It It It It was was was was found found found found that that that
14、 that almost almost almost almost 20%20%20%20%of of of of index index index index cases cases cases cases had had had had an an an an affected affected affected affected relative,relative,relative,relative,again again again again with with with with the the the the highest highest highest highest ri
15、sk risk risk risk in in in in siblings siblings siblings siblings in in in in a a a a large large large large population population population population study study study study in in in in British.British.British.British.studies of twins studies of twins studies of twins studies of twins histocompa
16、tibility histocompatibility histocompatibility histocompatibility antigens antigens antigens antigens(HLAs)(HLAs)(HLAs)(HLAs)associations associations associations associations with HLA-A3,B7,B18,and Dw2.with HLA-A3,B7,B18,and Dw2.with HLA-A3,B7,B18,and Dw2.with HLA-A3,B7,B18,and Dw2.Etiology and pa
17、thogenesisEtiology and pathogenesis3.3.3.3.Infection and immunological mechanismInfection and immunological mechanismInfection and immunological mechanismInfection and immunological mechanism:immunoregulatory defects may play a critical role in immunoregulatory defects may play a critical role in im
18、munoregulatory defects may play a critical role in immunoregulatory defects may play a critical role in pathophysiology.pathophysiology.pathophysiology.pathophysiology.autoimmunization of T lymphocytes against myelin basic autoimmunization of T lymphocytes against myelin basic autoimmunization of T
19、lymphocytes against myelin basic autoimmunization of T lymphocytes against myelin basic protein protein protein protein caused several different viruses.caused several different viruses.caused several different viruses.caused several different viruses.(Johanson)(Johanson)(Johanson)(Johanson)cellular
20、 cellular cellular cellular factors:factors:factors:factors:T T T T lymphocytes lymphocytes lymphocytes lymphocytes regulate regulate regulate regulate humoral humoral humoral humoral immune immune immune immune responses responses responses responses either either either either as as as as potentia
21、tors potentiators potentiators potentiators(T-helper(T-helper(T-helper(T-helper cells)cells)cells)cells)or or or or as as as as inhibitors inhibitors inhibitors inhibitors(T-suppressor(T-suppressor(T-suppressor(T-suppressor cells)cells)cells)cells)A A A A reduction reduction reduction reduction in i
22、n in in the the the the blood blood blood blood of of of of suppressor suppressor suppressor suppressor T T T T lymphocytes lymphocytes lymphocytes lymphocytes was was was was thought thought thought thought to to to to characterize characterize characterize characterize clinical clinical clinical c
23、linical relapse,relapse,relapse,relapse,or or or or an an an an increase increase increase increase in in in in helper helper helper helper suppressor suppressor suppressor suppressor ratios,ratios,ratios,ratios,(CD4/CD8)(CD4/CD8)(CD4/CD8)(CD4/CD8)dose dose dose dose appear appear appear appear to t
24、o to to be be be be associated associated associated associated with with with with increasing disability in patients of MS.increasing disability in patients of MS.increasing disability in patients of MS.increasing disability in patients of MS.Raised Raised Raised Raised titres titres titres titres
25、to to to to many many many many common common common common virusesvirusesvirusesviruses have have have have been been been been found found found found in in in in the the the the serum serum serum serum and and and and CSF CSF CSF CSF of of of of MS MS MS MS patients,patients,patients,patients,but
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- 脱髓鞘 幻灯 2009
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