MULTIPLE SCLEROSIS Xu, Ping Neurologic department of the 1 st affiliated hospital, ZMU

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

DEFINITION MS— Chronic inflammatory autoimmune disorder Of 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 demyelination Plaques 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 myelin  Infiltration with macrophages or microglia  Preservation of axons  Degree of oligodendrocyte preservation determines remyelination potential  Slower conduction time along affected nerve PATHOLOGY(4)

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

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) MRI Blood and urine (non-specific)

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

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 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)

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 years after diagnosis Common causes of death: secondary complications of immobility; depression (suicide)

Thank you!