MULTIPLE SCLEROSIS

Xu, PingNeurologic department of the 1st affiliated hospital, ZMU

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

DEFINITION

MS— Chronic inflammatory autoimmune disorderOf white matter Demyelination In the CNS

EPIDEMIOLOGY(1)

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(1)

Still unknown!

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—cerebellum

MRI—optic nerve

MRI—cerebral hemisphere

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)

Thank you!