中枢神经系统脱髓鞘疾病Demyelinatingdisordersincentralnervesystem课件

1、中枢神经系统脱髓鞘疾病Demyelinatingdisordersincentralnervesystem中枢神经系统脱髓鞘疾病DemyelinatingdisordMyelin in CNS is formed by the oligodendrocytesChemical composition: proteolipid, myelin basic protein, 2-3 cyclic nucleotide phosphohydrolase, myelin-associated glyco-protein, myelin-oligodendrocyte glyco-protein.Mye

2、lin in CNS is formed by theIntact myelin is required for action potential conductionIntact myelin is required for Myelined nerve fiber are rich in white matter of cerebral 、cerebella、brain stem、spinal cord，optic nerveMyelined nerve fiber are rich Demyelinating myelin is broken, axon remains intactDe

3、myelinating Demelinated disorders in CNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMOADEMBalosDemelinated disorders in CNSCoInflammatory demyelinating disorders in central nerve systemMultiple Sclerosis(MS)1Neuromyelitis optica (NMO)2Acute Disseminated Encephalomyelitis (AD

4、EM)3Concentric sclerosis(Balos disease)4Inflammatory demyelinating disInflammatory demyelinating disorders In CNS Multiple Sclerosis (MS)Inflammatory demyelinating disWhat is Multiple Sclerosis?Multiple Sclerosis (MS)“sclerosis” comes from the Greek word “skleros”, meaning hard. In multiple sclerosi

5、s, hard areas called “plaques” .“Multiple” refers to the many different areas of the nervous system that may have damaged myelin. What is Multiple Sclerosis?MulWhat is Multiple Sclerosis ?chronic inflammatory disease of CNSmalfunction of the immune system which leads to attacks against myelin sheath

6、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 patients affected by this disease depending on which nerves are damaged.What is Multiple Sclerosis ?chEpidemiologyapproximatel

7、y 1.1 million people are affected in USin 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 years; average age at diagnosis is 29 years in women and 31 years in men. female to male ratio is 2

8、:1. EpidemiologysymptomsMS 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.symptomsMS was first describedMultifocal lesionsMultifocal lesionsMultifocal lesionsMultifocal l

9、esionssymptomsCommon symptoms:Sensory disturbanceWeakness Problems in walking/balance/ coordination Visual problems： optic nerve Other possible symptoms:Bladder problem Spasticity Fatigue Facial weakness Trigeminal neuralgiaslurred speechtrouble swallowingDeafnesstemporary blindnessCognitive problem

10、s EpilepsyDepression symptomsCommon symptoms:Other signsLocal weakness Local sensory disturbancespoor 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, Lhermitte

11、sign, traverse spinal myelopathy，Brown-sequard syndrome in different levels of spinal cordsignsLocal weakness Courses (multiple phases)Courses (multiple phases)Laboratory findingsMagnetic Resonance Imaging (MRI) will show patches of tissue CSF：WBC，protein，MBP，OB， specific Abs Evoked Potentials： visu

12、al evoked potentials（VEP） auditory evoked potentials（BAEP） somatosensory evoked potentials （SEP）Laboratory findingsMagnetic ReHow is multiple sclerosis diagnosed?Timemutiple phasesSpace mutifocal lesionsExclude othersHow is multiple sclerosis diagThe Diagnostic Criteria of MS （Poser, 1983 )Number of

13、 AttackEvidence of More Than One LesionClinicalLab.CSF OCB or IgGA. Clinically DefiniteB. Lab-Supported DefiniteC. Clinically ProbableD. Lab-Supported ProbableA1 2 2A2 2 1 and 1B1 2 1 or 1 +B2 1 2 +B3 1 1 and 1 +C1 2 1C2 1 2C3 1 1 and 1D1 2 0 0 +The Diagnostic Criteria of MS Diagnostic Criteria for

14、Multiple Sclerosis (McDonald Criteria，2001）（1）Clinical Presentation Additional Data Needed 2 or more attacks (relapses) 2 or more objective clinical lesions None; clinical evidence will suffice (additional evidence desirable but must be consistent with MS) 2 or more attacks 1 objective clinical lesi

15、on Dissemination in space, demonstrated by: MRI or a positive CSF and 2 or more MRI lesions consistent with MS or further clinical attack involving different site 1 attack 2 or more objective clinical lesions Dissemination in time, demonstrated by: MRI or second clinical attack 1 attack 1 objective

16、clinical lesion (monosymptomatic presentation) Dissemination in space by demonstrated by: MRI or positive CSF and 2 or more MRI lesions consistent with MS and Dissemination in time demonstrated by: MRI or second clinical attack Diagnostic Criteria for MultipDiagnostic Criteria for Multiple Sclerosis

17、 (McDonald Criteria，2001）(2)Insidious neurological progression suggestive of MS (primary progressive MS) Positive CSF and Dissemination in space demonstrated by: MRI evidence of 9 or more T2 brain lesions or 2 or more spinal cord lesions or 4-8 brain and 1 spinal cord lesion or positive VEP with 4-8

18、 MRI lesions or positive VEP with frontal progression - generalized atrophy MRI: hypointense T1/hyperintense T2, atrophic splenium of corpus callosum adrenoleukodystrophyX-linked rmetachromatic leukodystrophy dymyelinating disease autosomal recessive aryl sulfatase A - absent from urine and serum most presen