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NMSP > Degenerative Diseases part 1 > Flashcards

Degenerative Diseases part 1 Flashcards

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?

A

Pick Disease

Progressive Supranuclear Palsy

24
Q

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

A

Behavioral changes and language disturbances

25
Q

With Pick Disease, there is atrophy of the frontal and temporal lobes. What is spared?

A

Posterior 2/3 of superior temporal gyrus

26
Q

How do the Gyri look with Pick Disease?

A

KNIFE-edge thin gyri

27
Q

What is the symptom of Progressive Supranuclear Palsy (FTLD-tau) that classifies it as a type of parkinson syndrome?

A

Progressive truncal rigidity

28
Q

This disease involves progressive truncal rigidity with globose (4R-tau) neurofibrillary tangles and neuronal loss

A

Progressive Supranuclear Palsy (FTLD-tau)

29
Q

Degenerative Diseases of the Basal Ganglia and Brainstem are associated with what types of disorders?

A

Movement disorders

30
Q

Degenerative Diseases of the Basal Ganglia and Brainstem involve what pathway specifically in the Basal Ganglia that is commonly affected?

A

Nigrostriatal pathway –> movement disorders

31
Q

What are the 3 main types of Degenerative Diseases of the Basal Ganglia and Brainstem?

A
  1. Parkinson Disease
  2. Atypical Parkinson Syndromes (3)
  3. Huntington Disease
32
Q

List some of the symptoms of Parkinson Disease?

A
  • Masked facies
  • Stooped posture
  • Pill-rolling tremor
  • Bradykinesia (slow movements)
  • Festinating gait (short and fast steps)
33
Q

What types of neurons are lost and from where with Parkinson Disease?

A

Loss of dopaminergic neurons from the Substantia Nigra –> slow movement

34
Q

If patients are responsive to ____ they likely have what disease?

A

L-Dopa

= Parkinson Disease

35
Q

What gene is the most common cause of AD form of Parkinson Disease?

36
Q

What 3 genes encode mitochondrial dysfunction in the AR form of Parkinson Disease?

A

DJ-1
PINK1
Parkin

37
Q

Pallor of the substantia niga and locus ceruleus suggests?

A

Parkinson Disease

38
Q

Lewy bodies are seen with Parkinson Disease. How do they look and what are they made of?

A

Eosinophilic cytoplasmic inclusion with dense core surrounded by a pale halo
– Alpha Synuclein clumps is what they are made of

39
Q

Lewy bodies are seen with Parkinson Disease. How do they look and what are they made of?

A

Eosinophilic cytoplasmic inclusion with dense core surrounded by a pale halo
– Alpha Synuclein clumps is what they are made of

40
Q

Dementia with lewy bodies can occur. What is the common symptom of that?

A

Hallucinations

41
Q

What are 3 types of Atypical Parkinsonian Syndromes?

A
  • Progressive Supranuclear Palsy
  • Corticobasal Degeneration
  • Multisystem Atrophy
42
Q

What are 3 types of Atypical Parkinsonian Syndromes?

A
  • Progressive Supranuclear Palsy
  • Corticobasal Degeneration
  • Multisystem Atrophy
43
Q

What differentiates Atypical Parkinsonian Syndromes from Parkinson Disease?

A

The syndromes have extra symptoms and are NOT RESPONSIVE to L-dopa!

44
Q

What inclusion is seen and where with Corticobasal Degeneration?

A

Tau in gray and white matter

45
Q

What inclusion is seen with Multisystem Atrophy and what are 3 symptoms?

A

Alpha-synuclein

  1. Parkinsonism
  2. Ataxia
  3. Autonomic Dysfunction
46
Q

How is Huntington Disease inherited?

A

Autosomal Dominant – symptoms do not start until 4th-5th decade of life

47
Q

What are the symptoms of Huntington Disease?

A

Dementia and Chorea (jerky movements)

48
Q

With Huntington disease, what repeats are present?

A

CAG – polyglutamine trinucleotide repeats

49
Q

With Huntington Disease, what repeats are present and what does more repeats correlate with?

A

CAG – polyglutamine trinucleotide repeats

= More repeats = Earlier age of onset

50
Q

What chromosome encodes the Huntington Protein?

A

Chromosome 4

51
Q

What types of neurons are lost with Huntington Disease?

A

Loss of medium spiny striatal neurons that normally dampen motor activity

52
Q

What type of mutation does the Huntington protein get and where does it accumulate?

A

Gain of function mutation

– intranuclear aggregates

53
Q

What part of the brain is usually atrophied with Huntington Disease?

A

Atrophy of the CAUDATE NUCLEUS

– later the putamen and frontal lobes

54
Q

What part of the brain is usually atrophied with Huntington Disease?

A

Atrophy of the CAUDATE NUCLEUS

– later the putamen and frontal lobes

55
Q

Anticipation: repeat expansions during spermatogenesis leads to an earlier onset of this disease

A

Huntington Disease

NMSP (28 decks)

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