北京协和-中枢经系统脱髓鞘疾病.pptVIP

Other demyelinating disorders Marchiafava-Bignami disease leukodystrophy inherited dysmyelinating disease adrenoleukodystrophy metachromatic leukodystrophy spongy degeneration (Canavan disease) globoid cell (Krabbe) leukodystrophy Alexander disease Pelizaeus-Merzbacher disease Cockayne syndrome adrenoleukodystrophy X-linked recessive demyelination of cerebral white matter adrenal insufficiency (unresponsive to ACTH) CT: white-matter dz: occipital regions -- frontal progression -- generalized atrophy MRI: hypointense T1/hyperintense T2, atrophic splenium of corpus callosum metachromatic leukodystrophy dymyelinating disease autosomal recessive aryl sulfatase A -- absent from urine and serum most present by 2 yrs, die at 3-4 yrs may arise at any age MR: hypointense T1 / hyperintense T2, of white matter, primarily in centrum semiovale metachromatic leukodystrophy metachromatic leukodystrophy 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. Immune-mediated disorder with an initial trigger Molecular mimicry Oligodendrocyte susceptible CNS infections may also lead to the transmigration of activated T lymphocytes into the CNS, which initiate the process How is MS Treated? Immunotherapy Corticosteroids corticosteroids can reduce the duration of symptoms, they do not cure MS. Used in Acute attacksintravenous methylprednisolone 1000 mg daily for three days. Prednisone: Clinicians differ on whether to taper off treatment with oral prednisone for two weeks, but this probably does not improve results and increases side effects. Interferons Beta interferon drugs have shown to be effective in treating the relapsing-remitting type of MS