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24 Cards in this Set
- Front
- Back
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how does dementia differ from MR?
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dementia is progressive. MR usually is not
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how does familial alzheimer's differ from sporadic?
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familial presents around age 50, earlier than sporadic.
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describe the gross appearance of an Alz brain
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general cerebral atrophy
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describe the micro appearance of an Alz brain
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plaques - AmyloidBeta deposits in the cortex.
tangles - hyperphosphorylated Tau protein in the Temporal lobe, then all of cortex -microangiopathy due to the Abeta in endothelium |
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what is the normal fxn of Tau protein?
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assemble and disassemble microtubules
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describe the APP and the enzymes that do/don't lead to Alz
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APP is a membrane protein, and can be cleaved by 3 secretases. If Beta and gamma secretase cut it, Amyloid beta forms = Alz
If alpha cleaves it, no Alz. |
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what cytokine is made my microglia in Alz that isn't made by normal microglia?
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IL-1
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what is Pick disease? what are the microscopic findings?
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It is a tau-opathy, atrophy of frontal lobes. Tau deposits (Pick Bodies) are round and less dispersed than those in Alz
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describe Progressive Supranuclear Palsy
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has Parkinsonism symptoms. tau is found not only in neurons, but also in glial cells. Tau inclusions are found in BG and Brainstem. Dementia usually develops eventually.
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what is the difference b/w Progressive Supranuclear Palsy and Corticobasal Degeneration
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CBD is often asymmetrical. yet both have Tau deposits in neurons and glial cells. CBD also has "ballooned" neurons.
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what is the 2nd most frequent cause of dementia?
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multi infarction vascular dementia
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what is alpha-synuclein? what is Parkin gene?
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a presynaptic protein, involved w/ DA transmission
parkin is involved in degrading alpha-synuclein -both are mutated in familial cases |
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relate Parkinson disease and Parkinsonism and Lewy bodies
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Parkinson Disease has Lewy Bodies in the SN
Parkinsonism can be caused by many things, and is marked by slow mvmts, rigidity, tremor -Lewy Bodies are eosinophilic cellular inclusions made up alpha synuclein protein |
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describe dementia with Lewy Bodies
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a disease that overlaps with Parkinsons and Alzheimer's. Lewy Bodies are Present in the SN. Parkinsonism may not be present, however. Dementia is present, and it may be caused by typical Alzheimer's causes.
-Must have: Lewy Body, Dementia -probably have: Parkinsonism; plaques and tangles -in Dw/LB, dementia shows first. (in Parkinsons Disease w/ Dementia, it is opposite) |
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what is the genetic defect in Huntington's
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CAG repeats = Glutamine repeats. Has a dominant inheritance.
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what is grossly degenerated in Huntington's
-ultimate effect on thalamus? |
caudate and putamen (striatum) and frontal cortex. loss of GABA inhibitory neurons, also some loss of ACh neurons (CAG = Caudate Ach Gaba)
-ultimately the thalamus receives less inhibitory input and thus gives more stimulatory input to the Cortex |
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look at the tables on page 420-1
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look at the tables on page 420-1
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alpha-synuclein inclusions in oligos describes what collection of diseases
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Multiple System Atrophy
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Progressive Supranuclear Palsy; Corticobasal degeneration. compare/contrast
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same: both are tau-opathies. both have tau inclusions in neurons and glia. both also have dementia. both also have Parkinsonism.
difs: PSP is bilateral, occurs in BG and Brainstem Corticobasal degeneration is asymmetric, occurs in Cortex and Brainstem |
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Krabbe Leukodystrophy, aka Globoid Cell Leukodystrophy. accumulated product?
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galactocerebrosides accumulate, some are eaten by macrophages which form globoid cells around BVs, some are metabolized to things toxic to oligodendrocytes
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Metachromatic Leukodystrophy, accumulated product?
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sulfatide (lipid w/ sulfate)
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what can thiamine (B1) deficiency lead to? Wernicke Korsakoff. describe it
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Clinical: confusion, ataxia, ophthalmoplegia; eventually memory loss with confabulation
-Pathology: degeneration of tissue adjacent to ventricles and MBs |
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Folate or B12, deficiency of which one leads to neuro problems in adult? howso
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no B12 - degeneration of spinal tracts' myelin. get paresthesias
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what is the most common gross CNS finding at autopsy in alcoholics?
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cerebrellar vermal atrophy. NOT wernicke-korsakoff degeneration of MB or ependymal cells, that is much less common
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