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Most common disabling condition in young adults
Most common demyelinating disorderChronic disease of the CNS Progresses to disability in majority of cas
esUnpredictable course variety of signs and symptoms; sometim
es mistaken for psychiatric diagnosis
FACTS
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
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
PATHOLOGY(1)
PATHOLOGY(2)Perivascular inflammation and demyeli
nationPlaques occur anywhere in the CNS
Most frequent: Periventricular region of the brain Optic nerve Brainstem Cerebellum Spinal cord
PATHOLOGY(3)
M ultiple---- Multifocal Multi-temporal Relapse-remitting progress S clerosis----plaques
Degenerative changes in myelinInfiltration with macrophages or microgliaPreservation of axonsDegree of oligodendrocyte preservation determines remyelination potential Slower conduction time along affected nerve
PATHOLOGY(4)
CLINICAL FEATURESInducement:infection, fatigue, delivery a baby…Initial symptoms:
Impaired vision Disequilibrium 平衡失调Heat intolerance 热耐受不良Problems with bladder controlSensory disturbance Motor weakness
Initial symptoms indicate the site of onset
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
VISION DISTURBANCE
Unilateral or bilateral partial/complete internuclear ophthalmoplegia 眼肌麻痹
Optic neuritis
Weakness
(mono-, hemi- or quadriparesis)
Increased spasticity
Pathologic signs
(Babinski, Chaddock, Hoffman)
Dysarthria( 构音不良 )
MOTOR DISTURBANCE
Urinary incontinence, 尿失禁
incomplete emptying
Cognitive and emotional abnormalities (depression, anxiety, emotional lability)
Fatigue
Sexual dysfunction
OTHER SYMPTOMS
INVESTIGATIONS
CSF (Oligoclonal bands)
Evoked potentials(VEP,BREP,SSEP)
MRIBlood and urine(non-specific)
MRI
Most useful tool in diagnosis MRI is abnormal in 90% of definite MSGadolinium enhancement identifies acti
ve lesions
Lesions abutting central ventricles, with diameter of >0.6 cm,in the posterior fossa, help to diagnose MS
MRI—spinal cord
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;
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.
Treatment--acute
Immunotherapy with steroids or ACTHSuppress inflammatory responseDecrease severity/duration of exacerbationsInhibit demyelinating processIV (3-5 days), then oral taper
Other immunomodulators (imuran, 硫唑嘌呤 cytoxan, methotrexate)
Treatment— relapse prevention
Interferon 1-beta (Betaseron) Interferon 1-alpha (Avonex)Useful for relapsing-remitting , not for
progressive type Significant side effects (nephrotoxicity,
leukopenia)
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)