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MULTIPLE SCLEROSIS Xu, Ping Neurologic department of the 1 st affiliated hospital, ZMU
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Most common disabling condition in young adults Most common demyelinating disorder Chronic disease of the CNS Progresses to disability in majority of cases Unpredictable course variety of signs and symptoms; sometimes mistaken for psychiatric diagnosis FACTS
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DEFINITION MS— Chronic inflammatory autoimmune disorder Of white matter Demyelination In the CNS
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EPIDEMIOLOGY(1)
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EPIDEMIOLOGY(2) Risk is increasing with the latitude Female dominance (F:M=2:1) Mainly young adults involved Peak age 20-40, mostly before 55
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ETIOLOGY(1) Still unknown!
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ETIOLOGY(2) Multifactory: Autoimmune(myelin antibody) Genetics---no clear-cut pattern of inheritance 20 fold increased familial incidence Infection---raised titres of many common viruses(HSV) in CSF and serum no virus-induced animal MS model
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PATHOLOGY(1)
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PATHOLOGY(2) Perivascular inflammation and demyelination Plaques occur anywhere in the CNS Most frequent: Periventricular region of the brain Optic nerve Brainstem Cerebellum Spinal cord
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PATHOLOGY(3) M ultiple---- Multifocal Multi-temporal Relapse-remitting progress S clerosis----plaques
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Degenerative changes in myelin Infiltration with macrophages or microglia Preservation of axons Degree of oligodendrocyte preservation determines remyelination potential Slower conduction time along affected nerve PATHOLOGY(4)
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CLINICAL FEATURES Inducement:infection, fatigue, delivery a baby… Initial symptoms: Impaired vision Disequilibrium 平衡失调 Heat intolerance 热耐受不良 Problems with bladder control Sensory disturbance Motor weakness Initial symptoms indicate the site of onset
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SENSORY DISTURBANCES Ascending numbness starting in feet Bilateral hand numbness Hemiparesthesia/dysesthesia 感觉迟钝 Dorsal column signs 脊髓 Loss of vibration/proprioception 本体感受 Lhermitte’s sign
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VISION DISTURBANCE Unilateral or bilateral partial/complete internuclear ophthalmoplegia 眼肌麻痹 Optic neuritis
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Weakness (mono-, hemi- or quadriparesis) Increased spasticity Pathologic signs (Babinski, Chaddock, Hoffman) Dysarthria( 构音不良 ) MOTOR DISTURBANCE
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Urinary incontinence, 尿失禁 incomplete emptying Cognitive and emotional abnormalities (depression, anxiety, emotional lability) Fatigue Sexual dysfunction OTHER SYMPTOMS
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INVESTIGATIONS CSF (Oligoclonal bands) Evoked potentials (VEP,BREP,SSEP) MRI Blood and urine (non-specific)
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MRI Most useful tool in diagnosis MRI is abnormal in 90% of definite MS Gadolinium enhancement identifies active lesions Lesions abutting central ventricles, with diameter of >0.6 cm,in the posterior fossa, help to diagnose MS
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MRI—cerebellum
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MRI—optic nerve
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MRI— cerebral hemisphere
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MRI—spinal cord
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Diagnostic criteria Clinical definite MS (CDMS): ① two times of attack and two lesions; ② two attacks, one lesion and one subclinical evidence; Laboratory supported definite MS (LSDMS): ① Two attacks, one subclinical evidence and CSF /OB/IgG; ② One attack, two lesions and CSF OB/IgG ; ③ One attack, one lesion, one subclinical evidence and CSF OB/IgG;
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Diagnostic criteria Clinical probable MS (CPMS): ① two attacks, one lesion ; ② one attack, two lesions ; ③ one attack, one lesion and other subclinical evidence; Laboratory supported probable MS (LSPMS) ① Two attacks ; CSF OB/IgG; ② Two attacks involving different part of CNS, intermission at lest one month ; each attack must continue for 24hs.
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Treatment--acute Immunotherapy with steroids or ACTH Suppress inflammatory response Decrease severity/duration of exacerbations Inhibit demyelinating process IV (3-5 days), then oral taper Other immunomodulators (imuran, 硫唑嘌呤 cytoxan, methotrexate)
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Treatment— relapse prevention Interferon 1-beta (Betaseron) Interferon 1-alpha (Avonex) Useful for relapsing-remitting, not for progressive type Significant side effects (nephrotoxicity, leukopenia)
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Prognosis EXTREMELY VARIABLE 50% chance of walking unaided 15 years after onset of disease Estimated longevity 25-35 years after diagnosis Common causes of death: secondary complications of immobility; depression (suicide)
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Thank you!
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