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1、MULTIPLE SCLEROSISDEFINITION OF MSlIt is defined clinically by the involvement of different parts of the CNS at different times-provided that other disorders causing multifocal central dysfunction have been excludedEPIDEMIOLOGYlInitial symptoms generally commence before the age of 55 years old with
2、a peak incidence between ages 20 and 40.Women are affected nearly twice as often as menEPIDEMIOLOGY AND GENETICShIncidence rate in United States:30-60/100,000 less than 5/100,000 in AsiahGeographic Gradients and incidence ratesEquator:low Latitude40oN and 40oSPathologylDemyelinative lesions are comm
3、only in cerebral hemisphere,optic nerve,spinal cord,brainstem,cerebellum.lPeriventricular scattered areas of demyelination followed by a reactive gliosis,there may be axonal damage as welllThese lesion occur in the white matter of the brain and cord and in optic(II)nervePathophysiologylThe cause of
4、MS is unknown,but tissue damage and neurological symptoms are throught to be triggered by an immune mechanism directed against myelin antigenslViral infection or other inciting factors may promote the entry of T cells and antibodies into the CNS by disrupting the blood brain barrierhInitial or Prese
5、nting Symptoms&Signs:hCommon initial complaints are focal weakness,numbness,tingling,or unsteadiness in a limb,Lhermitte signhSudden loss or blurring of vision in one eye(optic neuritis)hDiplopia,internuclear ophthalmoplegia,nystagmushDisequilibriumhBladder-function disturbance(urinary urgency or he
6、sitancy)such symptoms are often transient,disappearing after a few days or weeks with residual deficitCLINCAL FEATURES OF MS Other patients present with an acute or gradually progressive spastic parapresis and sensory deficithSubsequent Course:hInterval of months or years after the initial episode b
7、efore further neurologic symptoms appearhNew symptoms may then develop,or original ones may recur and progress.hRelapses may be triggered by infection and,in women,are more likely in the 3 months or so following childbirthCLINCAL FEATURES OF MShArise in body temperature can cause transient deteriora
8、tion in patient with a fixed and stable deficithWith time-and after a number of relapses and usually incomplete remissions-the patient may become increasing disabled by weakness,stiffness,sensory disturbances,unsteadiness of the limbs,impaired vision,and urinary incontinence Based on its course,the
9、disease is divided into 4 major types CLINCAL FEATURES OF MSCLINICAL SUBTYPES OF MS hRelapsing remitting(RR)hProgression does not occur between attackshPrimary progressive(PP)hThere is gradual progression of disability from clinical onsethSecondary progressive(SP)hA progressive course after an initi
10、al RR patternhProgressive relapsing(PR)MRI FINDINGS SUPPORTIVE OF MS DIAGNOSISh1GD-DTPA contrast enhance lesion OR 9 non contrast enhance high signal lesions on MR T2 sequencehAt least 1 periventricular lesionhAt least 1 infratentorial(Brainstem or cerebellar)lesionhAt least 1 juxtacortical lesion h
11、 Ovoid lesions perpendicular h Open ring on Gad T1-weighted hSpinal MRI to r/o congenital or acquired surgical treatable lesionCEREBROSPINAL FLUID IN MShMild lymphocytosishNormal glucosehNormal or modest increase in proteinhAbsent red blood cellshIncreased IgG Index/SynthesishOligoclonal bands 90%LA
12、B TESTSlMonocular visual stimulation with a checkerboard pattern(visual evoked potentials,VEPs)lMonaural stimulation with repetitive clicks(brainstem auditory evoked potentials,BAEPs)lElectrical stimulation of a periphral nerve(somatosensory evoked potentials,SEPs)one or more abnorm in MSSCHUMACHER
13、CRITERIA FOR MShOnset at appropriate agehCNS white matter diseaseh Disseminated in time and spacehObjective abnormalitiesh Attacks lasting over 24 hours one month aparthGradual or stepwise progressionhNo alternative diagnosisDiagnosis Criteria for MSPoser(1983)lClinical definite MS,CDMSlLaboratory-s
14、upported definite MS,LSDMSlClinical probable MS,CPMSlLaboratory-supported probable MS,LSPMS2005 Rev.McDonald CriteriaEDSS for MSthe Expanded Disability Status Scalethe Expanded Disability Status Scale neurological testing of functional systemneurological testing of functional systeml1.Pyramidal func
15、tion(ability to walk)l2.Cerebellar function(coordination)l3.BrainStem(speech&swallowing)l4.Sensory(touch&pain)EDSS for MSneurological testing of functional systemneurological testing of functional systeml5.Bowel&bladderl6.Visuall7.Mentall8.OtherEDSS for MSthe Expanded Disability Status Scalethe Expa
16、nded Disability Status ScalelFrom 0.0 normal neurological exam to10.0-deathle.g.6.0 intermittent or unilateral constant assistance(cane,crutch or brace)required to walk 100 meters with or without restingle.g.8.0 essentially restricted to bed,chair or wheelchair,but may be out of bed much of day;reta
17、ins self care functions,generally effective use of armsDIFFERENTIAL DIAGNOSIS OF MS-Neuromyelitis Optica(NMO)Neuromyelitis optica:is a relapsing disorder(formerly known as Devic disease and once considered a variant of MS)is associated wuth a specific antibody marker,NMO-IgG,that targets the water c
18、hannel aquaporin-4.The fully established disorder is chararcterized by optic neuritis and acute myelitis and associted with MRI changes that extend over at least 3 segments of the spinalDIFFERENTIAL DIAGNOSIS OF MS-Neuromyelitis Optica(NMO)Usually no brainstem,cerebellar,or cerebral demyelinative le
19、sion and normality on MRI of white matter,absence of OB band and IgG in CSF,more in Asia countries Acute attack treated with MP pusal,IVIG,PE with long term immnosuppressive theraphyDIFFERENTIAL OF MS-Acute Disseminated Encephalomyelitis(ADEM)Occurs as a single episode of neurological symptomss and
20、signs developed in few days associated with nonspecific viral infection or after immunization.CSF is nl or Protein increased,Glucose nlMRI is helpful in white matter lesion even with gray matter.IMMUNOPROPHYLACTICAGENTS FOR RR MShINTERFERONhINTERFERON beta-1b(Betaseron)Subcutaneous,every other day r
21、ecombinant protein E col,250 g hINTERFERON beta-1a(AVONEX)IM,weekly,recombinant protein,30 ghINTERFERON beta 1-a(REBIF)SQ 3X/WEEK 22 OR 44 g May use for more than 2 yearshGlatiramer acetate (Copaxone)Synthetic polypeptide,subcut 20mg dailyIMMUNOPROPHYLACTICAGENTS FOR RR MShNatalizumab,an alpha 4 int
22、egrin antibody,reduces the relapsing rate,but in rare unstances is associated with progressive multifocal leukoencephalopathyhMethyl-predinisolone (1 g daily)3-5 days,followed by an oral prednisone taper (80 mg/d for a week,with rapid reduction over the ensuing 1-2 weeks)hIntravenous immunoglobulin(
23、IVIG)0.4 g/kg for 5 days OR Plasmapheresis(PE)TREATMENT OFFERED FOR SP AND PP MShCyclophosphamidehAzathiopprine/methotrexatehCladribine/cyclosporinehMitoxantronehSteroid pulse with high-dose IV MP 1.0g once a month hIFN-beta-1b effectiveTREATMENT OF ACUTE EXACERBATIONS OF MShGlucocorticosteroids Hig
24、h-dose i.v.methylprednisolone 0.5 to 1.0 g/day variable combinationshPlasma Exchange PROGNOSISlAt least partial recovery from an acute episode can be anticipated,but it is impossible to predict when the next relapse will occure.lMore favorable prognosis:female,onset before 40,and presentation with visual or somatosensory,rather than pyramidal or cellellar dysfunction.lAbout 50%of all patients are only mildly or moderately disabled 10 years after the onset of symptoms THANKS!THANKS!