脱髓鞘幻灯说2009医大课件.ppt

Demyelinating disease of the CNS 中枢神经系统脱髓鞘疾病 Tianjin medical University General hospital Zhou Guang xi 天津医科大学总医院神经内科 周广喜 7702 Overview Demyelinative disease is a group of diseases of brain and spinal cord in which destruction of myelin is a prominent feature. Myelin sheaths play importance role in protecting and supporting for nervous fiber and transporting of material to neurons and maintaining of neurons’ ionic environment. The formation of myelin sheaths in both the central and peripheral nervous system follows a similar pattern. Central myelin is formed by an extension of membrane of the oligodendrocyte wrapping the axons, whereas peripheral myelin is formed by the extension of the Schwann cell membrane. The commonest accepted the pathologic criteria of demyelinative disease in CNS: 1. Destruction of the myelin sheaths of nerve fibers; 2. Relative sparing of other elements of nervous tissue, i.e. of axis cylinders, nerve cell, and supporting structure, and a relative lack of Wallerian, or secondary, degeneration off fiber tracts. 3. A particular distribution of lesions often is perivenous and primarily in white matter. Classification of demyelination disease in CNS Dysmyelinative type (leukodystrophy) It results from failure to form normally constituted myelin, due to genetic enzymatic disorders, such as adrenoleukodystrophy (ADL), Globod leucodystrophy (Krabbe), Metachromatic leucodystrophy (MLD) Demyelinating type It is breakdown of normally constituted myelin. Classifications of the demyelination disease Multiple sclerosis 1. relapsing-remitting , RR 2. primary progressive, PP 3. secondary progressive, SP 4. progressive relapsing , PR Neuromyelitis optica (Devic) Diffuse cerebral sclerosis (Schilder) concentric sclerosis of Balo Acute disseminated encephalomyelitis Central pontine myelinnolysis Multiple sclerosis Multiple sclerosis is the most common demyelinating disorder. It is a disease of the central white matter with “ lesio