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18 Cards in this Set
- Front
- Back
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What is the pathology of demyelinating diseases?
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inflammatory, sporadic, immune-mediated destruction of biochemically
normal myelin - central myelin (multiple sclerosis, acute disseminated encephalomyelitis |
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What is the pathology of dysmyelinating diseases?
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non-inflammatory, familial, destruction of chemically abnormal myelin
- may involve both central and peripheral myelin (metachromatic leukodystrophy, globoid cell leukodystrophy, adrenoleukodystrophy) |
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What is the pathology of hypomyelinating disease?
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general paucity of biochemically normal myelin deposition during
development (Alexander’s disease, vanishing white matter disease) |
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What is the pathology of myelinolytic diseases?
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non-inflammatory, sporadic or familial
- intramyelinic edema of biochemically normal myelin (central pontine myelinolysis, vitamin B12 deficiency, toxins |
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What are labratory findings with MS? What kind of myelin disease is?
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Demyelinating disease (inflam of bio normal). - mild mononuclear pleocytosis
- increased CSF IgG levels - oligoclonal bands (on gel electrophoresis) - MRI lesions in white matter of brain and spinal cord |
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What are pathological findings with MS?
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enhancing lesions on MRI with gadolinium
contrast - may produce mild mass effect - large hyper-reactive astrocytes - perivascular macrophages, lymphocytes - demyelination |
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What findings are associated with chronic MS?
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demyelination
- depressed greyish plaques - small old reactive astrocytes - few perivascular lymphocytes - usually no macro |
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What are the viruses/infxns that can precede acute disseminated encephalomyelitis? vaccines?
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measles, mumps, chickenpox (varicella)
German measles (rubella), influenza, infectious mononucleosis (Epstein-Barr virus), Mycoplasma pneumonia Campylobacter jejuni, group A streptococci. Vaccines: influenza, rabies, smallpox |
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What are the clinical manifestations of acute dissemintated encephalomyelitis? When does it come on and when subsde?
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sudden headache, vomiting, fever
- rapid development of weakness, sensory loss, ataxia, visual impairment, incontinence, and stupor - seizures may occur - spinal cord involvement may predominate. Comes on after 3 days and takes weeks to overcome |
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What is seen on a stain in a brain with ADEM? What kind of myelin disease is ADEM?
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multiple perivenular areas of demyelination and infiltration with chronic inflammatory cells (LFB-PAS stain). ADEM is demyelinating (inflam of bio norm myelin)
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What are the clinical manifestations of progressive multifocal leukoencephalopatny? What is the disease progression?
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focal neurologic deficits
dysarthria, limb weakness, visual disturbance, ataxia, personality changes, seizure. disease is relentless over months with impairment, dementia, death |
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What are the pathological findings with PML?
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multiple granular lesions in deep white matter. sheets of foamy macrophages and enlarged oligodendrocyte nuclei, oligo numbers decreased, viral antigens present in inlusions.
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What kind of myelin disease is metachromatic leukodystrophy? What is it caused by?
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Dysmyelinating disease (non-inflam attack of abnormal myelin w/ axon loss). Caused by lack of arylsulfatase leading to elevated sulfatides.
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What is seen histologically with MLD?
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metachromatic stains exhibit blue-red dues with MLD. Metachromatic deposits showing abnormal membrane bound inclusions
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What causes Krabbes disease? What kind of myelin disease is it?
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def of lysosomal enzyme galactocerebroside-β-galactosidase
deficiency leading to psychosine (a related galactocerebroside metabolite) toxicity which is toxic to oligos and myelin. Krabbe's is a dysmyelinating disease |
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What accumulates in Krabbe's disease (ie. what kind of cells)? What are these cells derived from?
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PAS-+, uninucleated/multinucleated epitheloid cells (globoid) which are derived from monocytes
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What is the biochemical defect of adrenoleukodystrophy? how is it transmitted?
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peroxisomal enzyme defect of an ATP-binding
cassette (ABC) transporter - reduced capacity to form coenzyme A derivative of very long chain fatty acids. X-linked transmission |
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What organs besides brain is affected in ALD? What is seen in the white matter histologically?
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adrenal cortex and testis (get ADHD, hearing and visual disturb, etc). lymphocytic inflitrates and lipid-laden macros in white matter
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