neuro disease resulting in dementia and neurodegeneration 1/23

Question 1

degenerative disease of cerebral cortex

Answer 1

alzheimer’s

pick

vascular dementia

Question 2

to be able to see senile plaques and neurofibrillary tangles

Answer 2

need silver stain

Question 3

neuritic (senile) plaques

Answer 3

extracellular

amyloid proteins

Question 4

neurofibrillary tangles

Answer 4

intracellular

microtubular proteins (tau)

Question 5

paired helical filaments

Answer 5

predominant in neurofibrillary tangles

hyperphosphorylated tau proteins

Question 6

granulovacuolar degeneration

Answer 6

small clear intraneuronal cytoplasmic vacuoles

each contains agyrophilic (silver granule)

seen in hippocampus and olfactory bulb in AD

Question 7

hirano bodies

Answer 7

elongated glassy eosinophilic bodies

made of paracrystalline arrays of beaded filaments (actin)

mostly seen in hippocampal pyramidal cells

Question 8

progression of plaques and tangles in AD

Answer 8

entorhinal cortex (temporal)–> direction

hippocampus (memory)

neocortex

Question 9

degree of dementia correlates better with

Answer 9

neurofibrillary tangles and loss of synapses than with neuritic plaques

Question 10

pick bodies in pick disease

Answer 10

stain positive for silver and made of neurofilaments

Question 11

werdnig-hoffman disease

Answer 11

motor neuron disease of infants

symptomatic at birth:poor suck,little movements of arms and legs

Question 12

areas affected by Wernicke and Korsakof

Answer 12

mamillary bodies and walls of third ventricle=problems with short term memories

walls of 3rd and 4th ventricles=ataxia and ophthalmoplegia

Question 13

dementia pugulistica

Answer 13

poeple who boxed or suffered from repeated trauma to the head

Question 14

opalski cells

Answer 14

voluminous cells with eccentric nuclei in basal ganglia

specific for wilson’s

Question 15

metachromatic leukodystrophy

Answer 15

AR

deficiency in aryl sulfatase-A (lysosomal)–>

accumulation of galactosyl sulfatides (lipid)–>

demyelination

abnormal lipid breakdown products stain diffrent colors

products accumulate in viscera and secreted in urine

Question 16

Krabbe globoid cell leukodystrophy

Answer 16

AR

deficiency in galactocerebrocide beta-galactocidase

accumulation of galactocerebrosides–> metabolized to galactosylsphingosine–> oligodendrocytes injury

see globoid cells=large MQ filled with breakdown products

Question 17

adrenoleukodystrophy

Answer 17

x linked

inability to metabolize very long chain fatty acid VLCFA

demyelination

see addison’s