Neurodegenerative Disease/Dementia Flashcards

(50 cards)

1
Q
Which of the following is NOT a degenerative disease affecting cerebral cortex?
A. Alzheimer's Disease
B. Parkinson Disease
C. Pick's Disease
D. Dementia with Lewy Bodies
A

B. Parkinson Disease affects basal ganglia & brainstem

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2
Q
Which of the following is NOT a disease affecting basal ganglia & brainstem?
A. Multiple System Atrophy
B. Huntington's Disease
C. Progressive Supranuclear Palsy
D. Pick's Disease
A

D. Pick’s Disease affects cerebral cortex

Parkinson Disease also affects basal ganglia & brainstem

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3
Q
Which of the following affect motor neurons?
A. Alzheimer's Disease
B. Parkinson Disease
C. Huntington's Disease
D. Amyotrophic Lateral Sclerosis
A

D

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4
Q

Two categories of clinical manifestation of neurodegenerative disease

A

Dementia

Movement disorders

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5
Q

T/F: Alzheimer’s Disease is relatively sparring of the occipital lobe.

A

True

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6
Q
Neuritic plaques found in all the following areas of the brain in Alzheimer's EXCEPT:
A. Hippocampus
B. Amygdala
C. Neocortex
D. Basal Ganglia
A

D

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7
Q

Secretory cleavage by _____ makes A-beta42 in neuritic plaques

A

Beta and gamma-secretase

Mutation that gain of function w/ increased production of A-beta42

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8
Q

All the following about neurofibrillary tangles are true EXCEPT:
A. Argyrophilic
B. Paired helical filaments in Pick’s Disease
C. Hyperphosphorylated forms of tau
D. Tau is encoded by gene 17q21

A

B. UNpaired helical filaments in Pick’s Disease

Paired helical filaments in Alzheimer’s Disease

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9
Q

T/F: Pick’s Disease mainly affects the parietal lobe.

A

False. Frontal lobe

Spares posterior superior temporaly gyrus

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10
Q

T/F: Neuritic plaques and amyloid angiopathy is found in Pick’s Disease.

A

False. Found in Alzheimer’s, not Pick’s

Neurofibrillary tangles found in both

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11
Q

Pick bodies =

A

Tau containing intraneuronal inclusion bodies
Spherical in neuronal cell bodies
Strongly argyrophilic
Found in pyramidal neurons, dentate granule cells in hippocampus, neocortex

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12
Q

Lewy bodies found in ____

Pathology appearance:

A

Parkinsonism
Eosinophilic core w/ outer halo

Needed for definitive diagnosis of Parkinson’s

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13
Q

Name the disease:

Loss of pigmented neurons in substantia nigra due to damages of dopaminergic neurons.

A

Parkinsonism

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14
Q

2nd most common cause of dementia

A

Dementia w/ Lewy Bodies (DLB)

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15
Q

Lewy-related neurites contain ____

A

Alpha-synuclein + material within neuropils

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16
Q

Dementia w/ Lewy Bodies present pathologically will all the following EXCEPT:
A. Cortical Lewy bodies
B. Lewy-related neurites
C. Neuritic plaques
D. Lipofuscin-like pigment in glial cells
E. Neurofibrillary tangles

A

D. Feature of Striatonigral degeneration (Multiple System Atrophy)

Grossly: pallor of substantia nigra and locus ceruleus

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17
Q

Multiple System Atrophy (MSA): Striatonigral degeneration predominated by

A

Parkinsonism

Striatonigral degeneration shows grossly w/ atrophy of putamen

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18
Q

Multiple System Atrophy (MSA): Olivopontocerebellar atrophy (OPCA) predominated by

A

Cerebellar signs

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19
Q

Multiple System Atrophy (MSA): Shy-Drager syndrome predominated by

A

autonomic failure

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20
Q

Glial inclusions are linked to ____

A

Multiple System Atrophy (MSA)

Caveat: not all cases of Olivopontocerebellar atrophy (OPCA) have glial inclusions

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21
Q

T/F: Progressive Supranuclear Palsy multisystem atrophy of middle and late age characterized by parkinsonism

A

False: degeneration, NOT atrophy

22
Q
Tau-associated lesions in Progressive Supranuclear Palsy are found in all EXCEPT:
A. Basal ganglia
B. Cortex
C. Brainstem
D. Cerebellum
23
Q
Progressive Supranuclear Palsy associated w/ signs and symptoms EXCEPT:
A. Supranuclear ophthalmoplegia 
B. Pseudobulbar palsy
C.Speech abnormalities
D. Cholera 
E. Mild dementia
24
Q

What is necessary for diagnosis of Progressive Supranuclear Palsy?

A

Globoid NFTs in basal ganglia and brainstem

25
Progressive Supranuclear Palsy shows atrophy of ___ (3)
Basal ganglia Midbrain Pons Also, depigmentation of substantia nigra
26
Huntington's Disease Genetic inheritance: Type of trinucleotide repeat:
AD. HD gene in short arm of chrom. 4 | CAG
27
Spinocerebellar ataxia Genetic inheritance: Type of trinucleotide repeat:
AD or AR | CAG
28
Freidreich’s ataxia Genetic inheritance: Type of trinucleotide repeat:
AR | GAA repeats
29
Spinal-bulbar muscular atrophy Genetic inheritance: Type of trinucleotide repeat:
X-linked recessive | CAG
30
Gross pathology of Huntington's Disease shows all of the following EXCEPT: A. Severe atrophy of entire brain B. Neostriatum shows gliosis and neuronal loss C. Cortical atrophy greatest in occipital lobe D. Other brain regions show loss of neurons w/o glosis
C. Cortical atrophy greater in frontal, parietal, occipital regions in temporal lobe
31
T/F: Amyotrophic Lateral Sclerosis affects lower motor neurons and mainly affects children.
False. Affects upper/lower motor neurons | Affects adults
32
T/F: Spinal Muscular Atrophy affects lower motor neurons and mainly affects infants and children.
True
33
Name the disease: | Degeneration of motor neurons in motor cortex, brainstem, spinal cord
Amyotrophic Lateral Sclerosis
34
SOD1 gene contributes to what disease?
Amyotrophic Lateral Sclerosis
35
Amyotrophic Lateral Sclerosis gross pathology include all of the findings EXCEPT: A. Atrophy of spinal cord (anterior motor roots) B. Atrophy of cranial motor nerves C. Atrophy of postcentral gyrus D. Muscle atrophy and wasting
C. Precentral gyrus
36
Bunina bodies in anterior horn cells found in what disease?
Amyotrophic Lateral Sclerosis
37
Greatest density of NFTs found where in Alzheimer's Disease?
Entorhinal cortex
38
Greatest density of amyloid plaques found where in Alzheimer's Disease?
Behind central sulcus in parietal and temporal lobes
39
Lowest density of amyloid plaques found where in Alzheimer's Disease?
Entorhinal cortex
40
BRAAK staging I-II in Alzheimer's Disease
Trans-entorhinal stage
41
BRAAK staging III-IV in Alzheimer's Disease
Spread to hippocampus
42
BRAAK staging V-VI in Alzheimer's Disease
Neocortex stage: spread to all of cortex
43
``` Which of the following is NOT a risk factor for Alzhemier's Disease? A. Diabetes B. Obesity C. Hypertension D. Heart disease E. Smoking ```
B Type 2 diabetes increases risk the most Smoking = #2
44
Name the 4 genes associated in Alzheimer's Disease
APP: amyloid protein precursor gene on chrom. 21 PS1: presenilin-1 gene in chrom. 14 PS2: presenilin-2 gene on chrom. 1 APOE4 allele on chrom. 19: E4 most concerning
45
T/F: Mild Cognitive Impairment characterized by subjective and objective memory problems BUT intact cognition and daily activities
True
46
``` Which of the following is LEAST affected by Alzheimer's Disease? A. Limbic cortex B. Association cortex C. Amygdala D. Motor cortex ```
D
47
Which of the following is NOT a criteria for Alzheimer's Disease? A. Abnormal results on cognitive screening and neuropsychological tests B. Deficits in 2 or more spheres of cognition C. Progression of amnesia and other cognitive deficits D. Disturbance of consciousness
D. No disturbance of consciousness is a criteria
48
PET scan of Alzheimer's Disease shows hypometabolism in ____ (3)
Bitemporal Parietal Frontal (later)
49
``` Which of the following is NOT a drug treatment of Alzheimer's Disease? A. Cholinesterase inhibitors B. NMDA antagonist C. Vit. E D. Barbituates ```
D
50
T/F: Alzheimer's pts can not learn and retain motor skills
False