北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件

1、北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病Myelin 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.Intact myelin is required for action potential conductionMyelined nerve

2、 fiber are rich in white matter of cerebral 、cerebella、brain stem、spinal cord，optic nerveDemyelinating myelin is broken, axon remains intactDemelinated disorders in CNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMOADEMBalosInflammatory demyelinating disorders in central nerv

3、e systemMultiple Sclerosis(MS)1Neuromyelitis optica (NMO)2Acute Disseminated Encephalomyelitis (ADEM)3Concentric sclerosis(Balos disease)4Inflammatory demyelinating disorders In CNS Multiple Sclerosis (MS)What is Multiple Sclerosis?Multiple Sclerosis (MS)“sclerosis” comes from the Greek word “sklero

4、s”, 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 Sclerosis ?chronic inflammatory disease of CNSmalfunction of the immune system which leads to attacks against myeli

5、n sheathinsulating 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.Epidemiologyapproximately 1.1 million people a

6、re 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 2:1. symptomsMS was fir

7、st 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.Multifocal lesionsMultifocal lesionssymptomsCommon symptoms:Sensory disturbanceWeakness Problems in walking/balance/ coordination

8、Visual problems： optic nerve Other possible symptoms:Bladder problem Spasticity Fatigue Facial weakness Trigeminal neuralgiaslurred speechtrouble swallowingDeafnesstemporary blindnessCognitive problems EpilepsyDepression signsLocal weakness Local sensory disturbancespoor coordination of upper and lo

9、wer extremity movements, wide-based gait with inability to tandem walk. nystagmus, internuclear ophthalmoplegia, visual disturbances, pallor of the optic disc, Lhermitte sign, traverse spinal myelopathy，Brown-sequard syndrome in different levels of spinal cordCourses (multiple phases)Laboratory find

10、ingsMagnetic Resonance Imaging (MRI) will show patches of tissue CSF：WBC，protein，MBP，OB， specific Abs Evoked Potentials： visual evoked potentials（VEP） auditory evoked potentials（BAEP） somatosensory evoked potentials （SEP）How is multiple sclerosis diagnosed?Timemutiple phasesSpace mutifocal lesionsEx

11、clude othersThe Diagnostic Criteria of MS （Poser, 1983 )Number of 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

12、1D1 2 0 0 +Diagnostic Criteria for 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 mor

13、e attacks 1 objective clinical lesion 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 cl

14、inical attack 1 attack 1 objective 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 Multiple Scle

15、rosis (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 MRI lesions or positive VEP with frontal progression - generalized atrophy MRI: hypointense T1/hyperintense T2, atrophic splenium of corpus callosum metachromatic leukodystrophy dymyelinating d