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Diseases of the Myelin

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Question
Answer
show A disruption of normal myelin causing neurologic disease  
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Definition of dysmyelinating diseases   show
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show Multiple sclerosis  
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What are the clinical forms of MS?   show
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Pathologic characteristics of multiple sclerosis plaques   show
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show Relapsing-remitting MS  
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show Primary progressive MS  
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show Secondary-progressive MS  
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Common clinical symptoms of relapsing-remitting form of MS   show
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What are the type of MS plaques?   show
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Histologic characteristics of active MS plaques   show
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show (1)Demyelination with "naked" axons (2)Minimal inflammatory reaction (3)Dense gliosis (4)Periphery of the plaque is hypocellular  
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Histologic characteristics of shadow MS plaques   show
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show (1)Periventricular areas (2)Cortical gray/white matter transition zone (3)Optic nerves, chiasm, tracts (4)Periaqueductal area (5)Cerebellum white matter (6)Cerebellar peduncles (7)Dorsal spinal cord (8)Subpial region of spinal cord (10)Pericentral canal  
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A very aggressive form of MS where the disease progresses rapidly to disability or death.   show
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A rare variant of MS characterized by lesions consisting of rings of demyelination alternating with rings of intact myelin.   show
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show High dose steroids  
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show Interferon Beta (1a and 1b) or Natalizumab.  
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An acute demyelinating disorder characterized by widely scattered small foci of periventricular inflammation and demyelination most frequently associated with antecedent immunization or infection.   show
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Most common preceding infections of postinfectious encephalomyelitis   show
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Most common preceding vaccinations of postvaccinal encephalomyelitis   show
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Biochemical abnormality associated wtih X-linked Adrenoleukodystrophy (ALD)   show
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A rare inherited progressive neurologic disease with adrenal insufficiency.   show
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The distribution of lesions of X-linked adrenaleukodystrophy   show
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show X-linked adrenaleukodystrophy  
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show Dysmyelination results from the myelin membrane insability induced by very long chain fatty chain acid (VLCFA) esters while the demyelination is initiated by cyotkines from an inflammatory response.  
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show (1) very long chain fatty acid esters levels in chorionic villus samples (CVS) (2) needle-like trilaminar bodies in cultured fibroblasts of culture of amniotic fluid cells.  
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show Bone marrow transplant  
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Treatment options for X-linked adrenoleukodystrophy   show
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