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MOHD4 Exam 4 Brain Stuff > Neurodegenerative Disease/Dementia > Flashcards

Neurodegenerative Disease/Dementia Flashcards

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
Q

Progressive Supranuclear Palsy shows atrophy of ___ (3)

A

Basal ganglia
Midbrain
Pons

Also, depigmentation of substantia nigra

26
Q

Huntington’s Disease
Genetic inheritance:
Type of trinucleotide repeat:

A

AD. HD gene in short arm of chrom. 4

CAG

27
Q

Spinocerebellar ataxia
Genetic inheritance:
Type of trinucleotide repeat:

A

AD or AR

CAG

28
Q

Freidreich’s ataxia
Genetic inheritance:
Type of trinucleotide repeat:

A

AR

GAA repeats

29
Q

Spinal-bulbar muscular atrophy
Genetic inheritance:
Type of trinucleotide repeat:

A

X-linked recessive

CAG

30
Q

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

A

C. Cortical atrophy greater in frontal, parietal, occipital regions in temporal lobe

31
Q

T/F: Amyotrophic Lateral Sclerosis affects lower motor neurons and mainly affects children.

A

False. Affects upper/lower motor neurons

Affects adults

32
Q

T/F: Spinal Muscular Atrophy affects lower motor neurons and mainly affects infants and children.

33
Q

Name the disease:

Degeneration of motor neurons in motor cortex, brainstem, spinal cord

A

Amyotrophic Lateral Sclerosis

34
Q

SOD1 gene contributes to what disease?

A

Amyotrophic Lateral Sclerosis

35
Q

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

A

C. Precentral gyrus

36
Q

Bunina bodies in anterior horn cells found in what disease?

A

Amyotrophic Lateral Sclerosis

37
Q

Greatest density of NFTs found where in Alzheimer’s Disease?

A

Entorhinal cortex

38
Q

Greatest density of amyloid plaques found where in Alzheimer’s Disease?

A

Behind central sulcus in parietal and temporal lobes

39
Q

Lowest density of amyloid plaques found where in Alzheimer’s Disease?

A

Entorhinal cortex

40
Q

BRAAK staging I-II in Alzheimer’s Disease

A

Trans-entorhinal stage

41
Q

BRAAK staging III-IV in Alzheimer’s Disease

A

Spread to hippocampus

42
Q

BRAAK staging V-VI in Alzheimer’s Disease

A

Neocortex stage: spread to all of cortex

43
Q 
Which of the following is NOT a risk factor for Alzhemier's Disease?
A. Diabetes
B. Obesity
C. Hypertension
D. Heart disease
E. Smoking
A

B

Type 2 diabetes increases risk the most
Smoking = #2

44
Q

Name the 4 genes associated in Alzheimer’s Disease

A

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
Q

T/F: Mild Cognitive Impairment characterized by subjective and objective memory problems BUT intact cognition and daily activities

46
Q 
Which of the following is LEAST affected by Alzheimer's Disease?
A. Limbic cortex
B. Association cortex
C. Amygdala
D. Motor cortex
47
Q

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

A

D. No disturbance of consciousness is a criteria

48
Q

PET scan of Alzheimer’s Disease shows hypometabolism in ____ (3)

A

Bitemporal
Parietal
Frontal (later)

49
Q 
Which of the following is NOT a drug treatment of Alzheimer's Disease?
A. Cholinesterase inhibitors
B. NMDA antagonist
C. Vit. E
D. Barbituates
50
Q

T/F: Alzheimer’s pts can not learn and retain motor skills

MOHD4 Exam 4 Brain Stuff (11 decks)

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