七年制医学ppt课件 神经病学 5MULTIPLE SCLEROSIS.ppt

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1、MULTIPLE SCLEROSIS(MS,P167-170),Prof.YAN YONGDept.of Neurology,the 1st Hospital,Chongqing University of Medical Science,PREFACE,MS is one of demyelinative diseases,with multifocal damage of the white matter of central nervous system(CNS,ie.brain and spinal cord)at different times that occurs in youn

2、g adults,with a peak incidence between age 20 and 40,women are affected nearly twice as oftenas men(2:1).,A.ETIOLOGY OF MS,The real cause of MS is unknown.1.A role for immune-mediated or infectionsfactors has been proposed,but data to supportthese postulates are fragmentary and indirect.2.Incidence

3、varies widely in differentgeographical areas.It is very low in the tropicsand high in the temperate zones of both northern and southern hemispheres(betweenlatitudes 400N and 400S).,3.A genetic predisposition is suggestedby twin studies,the occasional familial incidence,and the strong association bet

4、ween the disease and specific HL-antigens(HLA DR2).4.Present evidence supports the belief that the disease has an autoimmune basis.,B.PATHOLOGY,The disorder is characterized pathologically bythe development of focal-often periventricular scattered areas of demyelination followedby a reactive gliosis

5、;there may be axonal damage as well.These lesions occur in theCNS(eg.white matter of the brain,brainstem,spinal cord,cerebellum and in the optic()nerve).,C.CLINICAL FINDINGS,MS is a disease of young adults with a meanage of onset of 32(ages 1055,most 2040).This disease usually presents in the form o

6、frecurrent attacks of focal or multifocal neurologicdysfunction,reflecting lesions within the CNS.Typical episode:Symptoms worsen gradually over a period of afew days to 2 3 weeks,and then remit.Recoveryis usually lasting weeks or months.Remissionmay be complete,particularly after early attacks,howe

7、ver,remission of many patients is incompleteand as one attack follows another,a stepwise downward progression ensues with Increasing permanent deficit.The attacks occur-remit-recur-remit,seemingly randomly over many years.A half of MS take the form of an intermittentlyprogressive illness and sometim

8、es of a steadilyprogressive one,especially in patients more than 40 years of age.The first attack of MS may declare itself as asingle symptom or sign(45%)or as more thanone symptoms or signs(55%).,SYMPTOMS The symptoms and signs are variously bylesions position and damaged structures.Paresthesia 37

9、Gait disorder 35 Visual loss 15 Diplopia 10 Lower extremity weakness or incoordination 17 Upper extremity weakness or incoordination 10 Swanson JW,MS:Update in diagnosis.Mayo Clin 1989;64:577,SIGNS,Absent abdominal ref.81 Impaired joint positionHyperreflexia 76 sense 33Lower extremity ataxia 57 Inte

10、ntion tremor(ataxia)32Extensor plantar responses 54 Spasticity 31Impaired rapid alternating Impaired pain or temperaturemovements 49 sense 22Impaired vibratory sense 47 Dysarthria 19Optic neuropathy 38 Paraparesis 17Nystagmus 35 Internuclear ophthalmoplegia 11Swanson JW,MS:Update in diagnosis.Mayo C

11、lin 1989;64:577,Common initial complaints focal weakness numbness tingling diplopia unsteadiness in a limb disequilibrium sudden loss or blurring of vision in one eye,bladder-function disturbance(urinary urgency orhesitancy).Other patients present with an acute or graduallyprogressive spastic parapa

12、resis(paraplegia)or quadriplegia,and ataxia,and sensory deficit,andpsychic sym.and so on.,D.SUBSEQUENT COURSE Based on its course,the disease can be divided into 1.A relapsing-remitting form 缓解复发型 85%of cases in which progression does not occur between attacks;2.A secondary progressive form 继发进展型 80

13、%of cases after 25 years,characterized by a gradually progressive course after an initial relapsing-remitting pattern;,3.A primary progressive form 原发进展型 10%of cases in which there is gradual progression of disability from clinical onset.4.A progressive-relapsing form 进展-复发型 occurs rarely,with acute

14、 relapses being superimposed on a primary progressive course.,E.Special exams 1.CSF The oligoclonal bands(IgG)increased in CSF 2.Electrophysiological examinations Evoked potentials abnormalities VEP-visual evoked potential 视觉诱发电位 BAEP-brainstem auditory evoked potential 脑干诱发电位 SEPsomatosensory evoke

15、d potential 体感诱发电位 We can find subclinical lesions by evoked potentials.3.Myelography may be necessary to exclude the possibility of a single congenital or acquired surgically treatable lesions.,4.MRI:MRI is most sensitive means of detectinglesions of MS.MRI of brain can clearlyreveal lesions within

16、 white matter,brainstem and cerebellum,MRI of spinal cord may show the lesions also.They are better seen with T2-weightedthan T1-weighted images,but the FLAIR image压水成像 MRS and DWI are optimal.CT can be use sometimes.,Multiple,primarily punctate An abnormal region of high density,white matter high s

17、ignal intensity of plaques(periventricular)the cervical spinal cord,F.DIAGNOSIS,Diagnosis may be uncertain at the onset and in the early years of the disease,when symptoms andsigns point to a lesion in only one locus of the NS.Later,as the disease recurs and disseminatesthrougout the cerebrospinal a

18、xis,diagnosticaccuracy approaches 100%.In most cases the initial manifestations improvepartially or completely,to be followed after avariable interval by the recurrence of the sameabnormalities or the appearance of new ones inthe other parts of the CNS.,Examination in advanced cases commonly reveals

19、 optic atrophy,nystagmus,dytharthria,and upper motor neuron,sensory,or cerebellar deficits in some or all of the limbs.Note that the diagnosis cannot be based on any single symptom or sign,but only on a total clinical picture that indicates involvement of differentparts of the CNS at different times

20、.,The diagnosis of MS requires evidence that 1.At least two or more discrete lesionsseparated different regions of the central white matter have been affected at differenttimes,and a history of remission andrelapse.在CNS的白质中，在不同的时间(时间多发)，证实至少有两个或以上的孤立病灶(病灶多发)。So called“relapse”imply that the symptoms

21、 and signs lasted at least over24h and interval over a month once episode.,2.The onset must be in age range most characteristic of MS(ages1055yr,most2040).3.MRI demonstrated multifocal lesionsin CNS(brain,brainstem,cerebellum and spinal cord).4.Evoked potentials abnormalities.-(VEP,BAEP,SEP)5.Oligoc

22、lonal bands increased in CSF.,There are two diagnostic criterion by Poser(1983)and McDonald(2001)P235,p236 1.Clinically definite MS:A relapsing-remitting course and signs of atleast two lesions involving different regions ofcentral white matter,and conformed by MRI.2.Clinically probable MS:be confor

23、med multifocal white matter diseaseby MRI,but have had only one clinical attack,or have a history of at least two clinical episodesbut signs of only a single lesion.,G.TREATMENT,1.Relapsing-remitting type:Aim:To reduce the relapse rate Interferon-1a 30ug im,once weekly Interferon-1b 50ug subcutaneou

24、sly,qod.Intravenous immunoglobulin(IVIg)0.4g/kg/d5ds Corticosteroids:Methylprednisolone 1000 mg,iv./d,35ds,then followed by Prednisone tabella 60mg,oral,1mg/kg/d,for wks.,2.Progressive MS type:Interferon-1b(and probably Interferon-1a)are effective in reducing the progression rate asdetermined clinic

25、ally and MRI in secondaryprogressive disease.Cyclophosphamide azathioprine methotrexate cyceosporin A mitoxandrone cladribine Those may help to arrest the course of secondaryprogressive disease,but studies are inconclusive.with high-dose intravenous methylprednisolone(1g/d once a month)is also somet

26、imes effective.,3.Plasma exchange(PE)It is indicated especially in patients with a severe or rapidly progressive deficit or respiratory compromise.4.Intravenous immunoglobulin(IVIG)IVIG(400mg/kg/d for 5 days)appears to be equally effective and should be used in preference to plasmapheresis in adults

27、 with cardiovascular instability and in children.The two therapies are additive.,5.Symptomatic treatment should not be neglected,but the fatigue,spasticity are a serious problem.Treatment for other aspects of advanced MS such as cognitive deficits,pain,tremor and ataxia isgenerally less successful.,

28、G.Prognosis Symptoms and signs cease to progress by about 4 weeks into the illness.The disorder is self-limiting,and improvement occurs over the weeks or monthsfollowing onset.In the first episode of MS 70-75%of patients recover completely,25%are left with mild neurologic deficits,5%die of respirato

29、ry failure.The prognosis is poorer when there is evidence of preceding campylobacter jejuni infection 空肠弯曲菌感染,protracted course and less completer recovery.Advanced age,the need for ventilatory support,or more rapid onset of symptoms may also predict a poorer prognosis.,CASE REPORT,A woman,32 years

30、old.A worker,Marred.Chef complaint:weakness and numbness oflegs for 3 days with progressive.Present:She had weakness and numbness of legs 3 days ago.Then these symptoms had beengradually severs.she had numbness below lowabdomen and legs,urinary and fecal incontinence2 days ago.She had suffered from

31、optic neuritis on left eye 2 years ago and remitted with prednisone for 3 months.She had catch a cold 20 days ago.,The neurologic examination:Consciousness and cognitive function normal.Visual acuity decreased and color of the optic nerve head are pale.Visual fields normal.There are nystagmus when t

32、he eyes move.Other cranialnerves normal.The muscle tone,muscle strength,sense,deep tendon reflexes and coordination arenormal in the arms.There are loss of pain and temperature sensationbelow T10,and loss of reflex on the middle and low abdomen.The knee jerk and ankle reflex areincreased.The muscle

33、tone of legs spasticity,increased.The muscle strength is 2 grad in theleft leg and 3 grad in the right leg,the bilateral pathologic reflexes(Babinski and chaddock)are positive.Questions:1.Can you give the diagnosis and show reason?2.What investigative studies you need to make the correct diagnosis?,

34、Answers1.MS(multiple sclerosis)She has been suffering from weakness andnumbness of legs gradually developing for3 days;2 days ago she had urinary and fecal incontinence;Optic neuritis on left eye 2 years ago.Visual acuitydecreased and color of the optic nerve head are pale,nystagmus,pain and tempera

35、ture sensation below T10 and paraplegia.2.To confirm the diagnosis of MS we have to take MRI of spinal cord and brain,VEP,BAEP,SEP and CSF exams.,KEY POINT,1.What is the MS?2.Where are the common sits of the lesions of MS?3.What are the classic features of MS?4.What are the most effective investigat

36、ions for MS?5.How do treat to MS?Reference:Adams and Victors,P955-75,2001;Seventh Edition.McGraw-hill,Classification of the demyelinative diseases.Multiple sclerosis A.Chronic relapsing encephalomyelopathic form B.Acute MS C.Diffuse cerebral sclerosis.Neuromyelitis optical(Devic syndrom).Acute disse

37、minated encephalomylitis A.Following measles,chickenpox,smallpox,mumps,rubella,influenza,and other viral and soma bacterial infection B.Following rabies or smallpox and rarely other type of vaccination.Acute and subacute necrotizing hemorrhage encephalitis A.Acute encephalopathic from hemorrhagic leukoencephalitis of Hurst)B.Subacute necrotic myelopathy,