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1、Demyelinating disorders in central nerve system中枢神经系统脱髓鞘疾病,Myelin in CNS is formed by the oligodendrocytes,Chemical composition: proteolipid, myelin basic protein, 2-3 cyclic nucleotide phosphohydrolase, myelin-associated glyco-protein, myelin-oligodendrocyte glyco-protein.,Intact myelin is required
2、 for action potential conduction,Myelined nerve fiber are rich in white matter of cerebral 、cerebella、brain stem、spinal cord，optic nerve,Demyelinating myelin is broken, axon remains intact,Inflammatory demyelinating disorders in central nerve system,Inflammatory demyelinating disorders In CNSMultipl
3、e Sclerosis (MS),What is Multiple Sclerosis?,Multiple Sclerosis (MS) “sclerosis” comes from the Greek word “skleros”, meaning hard. In multiple sclerosis, hard areas called “plaques” . “Multiple” refers to the many different areas of the nervous system that may have damaged myelin.,What is Multiple
4、Sclerosis ?,chronic inflammatory disease of CNS malfunction of the immune system which leads to attacks against myelin sheath insulating myelin is damaged. The loss of myelin insulation degrades the nerve transmission ability. Thus a multitude of various neurological disabilities can be observed in
5、patients affected by this disease depending on which nerves are damaged.,Epidemiology approximately 1.1 million people are affected in US in all parts of the world and in all races, but whites of northern European descent have the highest incidence. occur in any age. usually diagnosed in aged 15-45
6、years; average age at diagnosis is 29 years in women and 31 years in men. female to male ratio is 2:1.,symptoms,MS was first described by Cruveilhier in 1835. A generally valid description of MS symptoms was made by Charcot in the year 1868. In 1904 the description was supplemented by Mller.,Multifo
7、cal lesions,Multifocal lesions,symptoms,Common symptoms: Sensory disturbance Weakness Problems in walking/balance/ coordination Visual problems： optic nerve,Other possible symptoms: Bladder problem Spasticity Fatigue Facial weakness Trigeminal neuralgia slurred speech trouble swallowing Deafness tem
8、porary blindness Cognitive problems Epilepsy Depression,signs,Local weakness Local sensory disturbances poor coordination of upper and lower extremity movements, wide-based gait with inability to tandem walk. nystagmus, internuclear ophthalmoplegia, visual disturbances, pallor of the optic disc, Lhe
9、rmitte sign, traverse spinal myelopathy，Brown-sequard syndrome in different levels of spinal cord,Courses (multiple phases),Laboratory findings,Magnetic Resonance Imaging (MRI) will show patches of tissue CSF：WBC，protein，MBP，OB，specific Abs Evoked Potentials：visual evoked potentials（VEP） auditory ev
10、oked potentials（BAEP）somatosensory evoked potentials （SEP）,How is multiple sclerosis diagnosed?,Timemutiple phasesSpace mutifocal lesionsExclude others,The Diagnostic Criteria of MS （Poser, 1983 ),Number of Attack,Evidence of More Than One Lesion,Clinical,Lab.,CSF OCB or IgG,A. Clinically Definite,B
11、. Lab-Supported Definite,C. Clinically Probable,D. Lab-Supported Probable,A1 2 2,A2 2 1 and 1,B1 2 1 or 1 +,B2 1 2 +,B3 1 1 and 1 +,C1 2 1,C2 1 2,C3 1 1 and 1,D1 2 0 0 +,Diagnostic Criteria for Multiple Sclerosis (McDonald Criteria，2001）（1）,Diagnostic Criteria for Multiple Sclerosis (McDonald Criter
12、ia，2001）(2),Differential diagnosis,First attack: single lesion or multiple lesions: infection, tumor, infarction, Relapse type: infarction, embolism, vasculitis, Progressive: inherited, degenerating,Pathology of MS,Gross neuropathologygrey plaques (acute plaque may be pink) may occur anywhereMicrosc
13、opic neuropathologyperivascular lymphocytes infiltrate; loss of oligodendrocytes; myelin stripping; macrophage infiltrate; astrogliosis; relative sparing of axons,What Causes MS?,The exact cause of MS is not known Environmental Factors： Geography is clearly an important factor the rate is approximately twice as below the 37th parallel. Genetic Factors： MS is not strictly an inherited disorder.Susceptibility to MS probably has a genetic component.,Pathogenesis of MS: incompletely understood.,
