脱髓鞘幻灯2009医大课件.ppt

1、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 p

2、rotecting 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 ol

3、igodendrocyte 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

4、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,Dysmyelinativ

5、e 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.,Classific

6、ations 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) it may then represent the initial manifestation of multiple scle

7、rosis. Furthermore, fever, stupor, and coma, which are characteristic, rarely occur in MS.,Differential diagnosis,2.systemic lupus erythematosus and other autoimmune disease. there may be multiple lesions of CNS white matter. Close attention to the characteristic history (rash and arthritis etc.) an

8、d serologic findings should permit the distinction of the two diseases. 3.Cervical spondylosis The typical protein abnormalities of MS is absent. The disturbance of bladder function occur late or not at all in it. 4. Behcet disease are recurrent iridocyclitis and meningitis , mucous membrane ulcer o

9、f mouth and genitalia , and symptoms of articular. renal, lung and multifocal cerebral disease.,Treatment,Corticosteroids: Methylprednisolone is 500 to 1000 mg daily for 3-5 days, iv., followed by high oral doses of prednison 60 mg daily and tapering this dosage over a 12 day is generally effective and 5-10/week. Immune globulin (IG) iv 0.4/kg /d 3-5 days. Beta-Interferon 1-a and 1-b: (30ug or 6.6 million unit im/weekly) and Copolymer 1 (Glatiramer acetate) (20mg/daily, subcutaneous) Suppression of the immune system: Azathioprine or cyclophosphamide . Plasmapheresis