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Degenerative disorders Flashcards

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1
Q

What is fibrillogenesis

A

abnormal intracellular accumulation of linear fibrils

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2
Q

What happens when arrays of fibrils begin to condense in a cell

A

the cellular response is activated, the fibrils aggregate, and their constituents are ubiquinated

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3
Q

What does ubiquinate mean

A

to be tagged for degradation

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4
Q

What is neurodegenerative disease

A

loss of functionally related neurons

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5
Q

Cortical NGD leads to

A

dementia

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6
Q

Basal ganlia NGD leads to

A

movement disorder

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7
Q

Cerebellar NGD leads to

A

ataxia

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8
Q

Motor NGD leads to

A

weakness

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9
Q

What are the three reasons neurons die

A

excitoxicity
free radical injury
fibrillogenesis

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10
Q

Protein fibrillogenesis consists of protein deposition where

A

extracellularly-plaque

intracellularly-inclusions

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11
Q

Cyoplasmic inclusions include

A

structural protein (tau and/or synuclein)

stress protein (ubiquitin, crystalline, HSP’s)

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12
Q

How are inclusions detected

A

light/electron microscopy

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13
Q

NGD may start focally, where in ALZ, Parkinsons, and ALS

A

Alzheimer’s- starts in hippocampus»memory problems

Parkinson’s- starts in substantia nigra»hemiparkisonism

ALS- hand weakness»other muscle groups

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14
Q

NGD may overlap, where in Parkinsons and ALS

A

Parkinson»movement disorder develops into dementia later

ALS»>hand weakness»>frontotemporal dementia

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15
Q

Parkinsons is common is what group

A

older persons

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16
Q

What brain structure atophies in parkinsons

A

substantia nigra

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17
Q

What neuron in loss in parkinsons

A

dopamine projecting to striatum

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18
Q

What are microscopic characteristics of parkinsons disease

A

lewy bodies composed of alpha-synuclein

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19
Q

What are Lewy bodies and what is its characteristics

A

Lewy bodies are abnormal aggregates of protein that develop inside nerve cells in Parkinson’s disease

Dark center with hollow outer segment (Hallow)Lewy bodies appear as spherical masses within the pigmented neuron that displace other cell components

Also, they contain Stress proteins (ie ubiquitin)

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20
Q

What are the three abnormal motor signs of parkinsons

A

rigidity
tremor
bradykinesis

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21
Q

Name two other causes of parkinsonism

A

Post-encephalitic parkinsonism-Tauopathy

Progressive supranuclear palsy-Tauopathy

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22
Q

What are characteristics of abnormal tau aggregation

A

Tangles of darkened fibrill in the neuron

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23
Q

Name two NGD associated with ataxia

A

Multiple system atrophy

Fredreich’s ataxia

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24
Q

NG ataxias is categorized by what two possiblities

A

degeneration of inflow or outflow pathways

degeneration of cerebellary cellular constitutents

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25
What protein is abnormal in Multiple system atrophy
Alpha synuclein
26
What are the three classic structures degenerated in multiple system atrophy
striatonigral degeneration>>parkinsonism olivo-ponto-cerebellar degeneration>>ataxia shy drager dysautonomia>>autonomic dysfx
27
What three structures show GROSS atrophy in multiple system atrophy
PONS Medullary olives Cerebellum
28
Is freidreich's ataxia common
Yes *most common HEREDITARY ataxia AUTOSOMAL RECESSIVE
29
What is the genotype pathology associated with freidreich's ataxia
GAA triple repeat expansion of the FRATAXIN gene
30
What is the two anatomically gross characteristics of freidreich's ataxia
atophy of cerebellum atrophy of multiple long tracts (DC,STT,Spinocerebellar, Dorsal root, Clarks column)
31
Name two motor neuron NG disorders
Amyotrophic lateral sclerosis Infantile progressive spinal muscular atophy
32
Amyotrophic lateral sclerosis involves atrophy of
both upper and lower motor neurons
33
Familial ALS results from a mutation of
superoxide dismutase **not a loss of free radical handling, its a gain of function mutation
34
What is the gross appearance of ALS of the spinal cord
motor pathway degeneration (CST, anterior horn, anterior roots)
35
Cinical localization of higher cortical functions involve deficits in what three functional aspects of the brain
right and left hemispheres higher cortical functions higher executive functions
36
agnosias are defects of
knowing
37
aparaxias are defects in
doing
38
aphasias are defects in
language (spoken, writte, signing)
39
Higher executive function defects involves
frontal disinhibition planning
40
Cortical degeneration results in
dementia
41
Name three diseases of dementia
Alzheimer Picks Lewy body dementia
42
Microscopic examination of alzheimers reveals abnormal protein folding of what two proteins
Beta-amyloid>>plaques (extracellular) Tau>>tangles (intracellular)
43
What three gross characteristics are evident in alzheimer disease
cortical atrophy>>thinning of cortex and widening of sulci ventricle enlargement>>compensatory hydrocephalus EX VACUO decreased brain weight
44
What is the relationship b/t the severity of dementia and tangles at death with alzheimers
positive correlation
45
What is the relationship b/t the number of plaques in the brain and cognitive impairment at death with alzheimers
No correlation
46
Atrophy in alzheimer's usually starts in what three brain structures
amygdala hippocampus temoral lobe
47
What basal forebrain nucleus is atrophied in alzheimers
basal nuclea of Meynert
48
Alzheimer's involves disturbance of
memory d/t loss of cortical cholinergic innervation
49
What two drug classes are used to slow alzheimers
central cholinesterase inhibitors glutamate antagonists
50
Name a two drugs used to slow alzheimers
donepezil (central cholinesterase inhibitor) memantine (NMDA antagonists)
51
Pick's disease involves degeneration of what two cortical structures
frontal lobe temporal lobe (sparing of 2/3 of sup. temporal gyrus, partietal lobes, and precentral gyrus)
52
What two brain structures are depleted in picks diseases
neurons glia
53
With marked temporal and frontal atrophy, what characteristic appearnce does the frontaltemporal lobe gyrus have
'knife-edge" gyri
54
What behavioral manifestations is common with picks disease
Frontal disinhibition>>impaired judment, impulseness, and criminal behavior
55
Pick involves what abnormal protein aggregation
Tau *purely a Tauopathy
56
Picks in characterized by intraneuronal cytoplasmic inclusions known as
pick bodies *neurons are look pink and swollen and no plaques or tangels VS. PSP - tau with tangles
57
Diffuse Lewy Body Disease is characterized by what abnormal protein folding
alpha syncuclein
58
Diffuse Lewy Body disease combines what two ND disorders
Alzheimer's and Parkinsons *cognitive, visual hallucinations, and extrapyramidal features Hallow appearance on microscopy with possivle beta amyloid plaques
59
What three disorders are synucleinopathies
Parkinsons Multiple system Atrophy Lewy body dementia
60
What disease is associated with superoxide dismutase
Familial ALS
61
What two diseases are associated with Tau
Progressive supranuclear palsy Pick's disease
62
What disease is associated with intraneronal tangles (tau) and extracellual plaque (b-amyloid)
alzheimer's disease
63
Huntington's disease is genetically passed via
autosomal dominance
64
What gene loci is huntington's disease located on
chromosome 4
65
What two main characteristics distinguishes huntingtons from parkinsons
combines dementia with choreiform movements onset in 20-40's
66
What does the brain look like in huntington's disease
atrophy of caudate nucleus and putamen compensatory hydrocephalus
67
What NT is diminished with Huntington's disease
GABA
68
What is the genetic defect in Huntington's disease
Trinucleotide repeat amplification

Nervous system (7 decks)

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