中枢MS教材讲义.ppt

Central Nervous System Categories of White Matter Disease Demyelinating Normal myelin is injured or destroyed Further divided into primary and secondary Dysmyelinating Intrinsic abnormality of myelin formation or maintenance Rare, usually seen in pediatric population Demyelinating Disorders Primary demyelinating disease Multiple sclerosis Secondary demyelingating disease Acute disseminated encephalomyelitis Progressive multifocal leukoencephalopathy Osmotic demyelination Disseminated Necrotizing Leukoencephalopathy Chemotherapy and radiation Multiple Sclerosis: Epidemiology Most common demyelinating disease encountered in practice First described in 1868 by Charcot Female predominance Second to fifth decades of life Northern European extraction Temperate climates Multiple Sclerosis: Clinical Presentation Evidence of two or more white matter lesions separated by time and in different locations Range of symptoms Cranial nerve palsy Optic neuritis Vague sensory complaints Paresis Variable pattern of presentation Exacerbations and remissions Chronic progressive Attack followed by absence of clinical disease Rapidly progressive Multiple Sclerosis: Diagnosis Schumacher’s criteria: two or more white matter lesions with either… Two or more episodes of worsening, each lasting at least 24 hrs and each at least a month apart Slow stepwise progression of signs or symptoms for at least six months MR plays role in confirming clinical diagnosis Possitive study with appropriate clinical data strongly supports diagnosis of MS Multiple Sclerosis: Pathology Myelin breakdown with associated lymphocyte and macrophage infiltration of affected area Astrocyte proliferation and infiltration of demyelinated area, with continued signs of inflammation Gliotic regions with decreased cellularity and no myelin Multiple Sclerosis: Dawson’s Finger Inflammatory lesions along periventricular medullary veins Radiate from ventricular surface Lesions preceed demyelination Multiple Sclerosis: Imaging