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NMSP > Degenerative Diseases part 1 > Flashcards

Degenerative Diseases part 1 Flashcards

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1
Q

Degenerative diseases are diseases of _____ matter

A

Gray matter diseases

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2
Q

In general, Degenerative diseases involve the progressive loss of ____ and presence of ______

A

Progressive loss of neurons

Presence of protein aggregates (inclusions)

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3
Q

In general, Degenerative diseases involve the progressive loss of ______ and the presence of _____

A

Progressive loss of neurons

Presence of protein aggregates (inclusions)

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4
Q

What is Dementia?

A

Progressive loss of cognitive function = NOT a normal part of aging

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5
Q

The incidence of Alzheimer Disease increases with?

A

Increasing age – most cases sporadic

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6
Q

What is the most common type of Dementia?

A

Alzheimer Disease

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7
Q

A pathologic examination of the brain is needed for a definitive diagnosis of Alzheimer Disease. How does the brain look?

A

Frontal, Temporal and Parietal lobe have cortical atrophy!

= Widened sulci and hydrocephalus ex vacuo

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8
Q

A pathologic examination of the brain is needed for a definitive diagnosis of Alzheimer Disease. How does the brain look?

A

Frontal, Temporal and Parietal lobe have cortical atrophy

= widened sulci and hydrocephalus ex vacuo

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9
Q

What is the initiating event for Alzheimer Disease development?

A

Amyloid beta generation

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10
Q

What correlated to the degree of dementia with Alzheimer Disease?

A

The # of tangles correlates with the degree of dementia

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11
Q

What are the 2 histo items seen with Alzheimer Disease?

A

Amyloid Beta plaques in neuropil

Tau Neurofibrillary Tangles intracellularly in the neuron

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12
Q

What are the 2 histo items seen with Alzheimer Disease?

A

Amyloid Beta plaques in the neuropil

Tau Neurofibrillary Tangles intracellularly in the neuron

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13
Q

What gene has an increased risk of developing Alzheimer Disease?

A

ApoE

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14
Q

What are the 2 types of plaques seen with Alzheimer Disease?

A
  • Neuritic plaques

- Diffuse plaques

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15
Q

Neuritic plaques are seen with Alzheimer Disease. Describe them.

A

Neuritic processes around an amyloid core

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16
Q

Diffuse plaques are also seen with Alzheimer Disease. Describe them.

A

NO Amyloid core!

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17
Q

Neuritic plaques with Alzheimer Disease have an Amyloid Beta core. What stain will show positive and what Amyloid Beta is contained in the core?

A

Congo red stain +

– Amyloid Beta 40 and 42

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18
Q

This disease has abundant Granulovacuolar degeneration and eosinophilic Hirano bodies made of actin

A

Alzheimer Disease

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19
Q

What angiopathy usually accompanies Alzheimer Disease?

A

Cerebral Amyloid Angiopathy

– Amyloid beta in vessel walls

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20
Q

What syndrome has early onset Alzheimer Disease due to the APP (amyloid precursor protein) being on the affected chromosome?

A

Down Syndrome (trisomy 21)

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21
Q

What are Frontotemporal Lobar Degenerations (FTLDs)?

A

Diseases that involve dementia and have degeneration of the frontal and temporal lobes

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22
Q

What 2 inclusions are options to be seen with Frontotemporal Lobar Degenerations (FTLDs)?

A

Tau

TDP-43

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23
Q

What are 2 FTLDs - tau inclusions?

Study These Flashcards
A

Pick Disease

Progressive Supranuclear Palsy

24
Q

What are the symptoms of Pick Disease (FTLD-tau)?

Study These Flashcards
A

Behavioral changes and language disturbances

25
With Pick Disease, there is atrophy of the frontal and temporal lobes. What is spared?
Posterior 2/3 of superior temporal gyrus
26
How do the Gyri look with Pick Disease?
KNIFE-edge thin gyri
27
What is the symptom of Progressive Supranuclear Palsy (FTLD-tau) that classifies it as a type of parkinson syndrome?
Progressive truncal rigidity
28
This disease involves progressive truncal rigidity with globose (4R-tau) neurofibrillary tangles and neuronal loss
Progressive Supranuclear Palsy (FTLD-tau)
29
Degenerative Diseases of the Basal Ganglia and Brainstem are associated with what types of disorders?
Movement disorders
30
Degenerative Diseases of the Basal Ganglia and Brainstem involve what pathway specifically in the Basal Ganglia that is commonly affected?
Nigrostriatal pathway --> movement disorders
31
What are the 3 main types of Degenerative Diseases of the Basal Ganglia and Brainstem?
1. Parkinson Disease 2. Atypical Parkinson Syndromes (3) 3. Huntington Disease
32
List some of the symptoms of Parkinson Disease?
- Masked facies - Stooped posture - Pill-rolling tremor - Bradykinesia (slow movements) - Festinating gait (short and fast steps)
33
What types of neurons are lost and from where with Parkinson Disease?
Loss of dopaminergic neurons from the Substantia Nigra --> slow movement
34
If patients are responsive to ____ they likely have what disease?
L-Dopa | = Parkinson Disease
35
What gene is the most common cause of AD form of Parkinson Disease?
LRRK2
36
What 3 genes encode mitochondrial dysfunction in the AR form of Parkinson Disease?
DJ-1 PINK1 Parkin
37
Pallor of the substantia niga and locus ceruleus suggests?
Parkinson Disease
38
Lewy bodies are seen with Parkinson Disease. How do they look and what are they made of?
Eosinophilic cytoplasmic inclusion with dense core surrounded by a pale halo -- Alpha Synuclein clumps is what they are made of
39
Lewy bodies are seen with Parkinson Disease. How do they look and what are they made of?
Eosinophilic cytoplasmic inclusion with dense core surrounded by a pale halo -- Alpha Synuclein clumps is what they are made of
40
Dementia with lewy bodies can occur. What is the common symptom of that?
Hallucinations
41
What are 3 types of Atypical Parkinsonian Syndromes?
- Progressive Supranuclear Palsy - Corticobasal Degeneration - Multisystem Atrophy
42
What are 3 types of Atypical Parkinsonian Syndromes?
- Progressive Supranuclear Palsy - Corticobasal Degeneration - Multisystem Atrophy
43
What differentiates Atypical Parkinsonian Syndromes from Parkinson Disease?
The syndromes have extra symptoms and are NOT RESPONSIVE to L-dopa!
44
What inclusion is seen and where with Corticobasal Degeneration?
Tau in gray and white matter
45
What inclusion is seen with Multisystem Atrophy and what are 3 symptoms?
Alpha-synuclein 1. Parkinsonism 2. Ataxia 3. Autonomic Dysfunction
46
How is Huntington Disease inherited?
Autosomal Dominant -- symptoms do not start until 4th-5th decade of life
47
What are the symptoms of Huntington Disease?
Dementia and Chorea (jerky movements)
48
With Huntington disease, what repeats are present?
CAG -- polyglutamine trinucleotide repeats
49
With Huntington Disease, what repeats are present and what does more repeats correlate with?
CAG -- polyglutamine trinucleotide repeats | = More repeats = Earlier age of onset
50
What chromosome encodes the Huntington Protein?
Chromosome 4
51
What types of neurons are lost with Huntington Disease?
Loss of medium spiny striatal neurons that normally dampen motor activity
52
What type of mutation does the Huntington protein get and where does it accumulate?
Gain of function mutation | -- intranuclear aggregates
53
What part of the brain is usually atrophied with Huntington Disease?
Atrophy of the CAUDATE NUCLEUS | -- later the putamen and frontal lobes
54
What part of the brain is usually atrophied with Huntington Disease?
Atrophy of the CAUDATE NUCLEUS | -- later the putamen and frontal lobes
55
Anticipation: repeat expansions during spermatogenesis leads to an earlier onset of this disease
Huntington Disease

NMSP (28 decks)

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