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Term 5: Neuro > 5/27- Degenerative Disorders > Flashcards

5/27- Degenerative Disorders Flashcards

1
Q

Symptoms seen with loss of cortical neurons?

A

Dementia

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2
Q

Symptoms seen with loss of basal ganglia neurons?

A

Movement disorder

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3
Q

Symptoms seen with loss of cerebellar neurons?

A

Ataxia

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4
Q

Symptoms seen with loss of motor neurons?

A

Weakness

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5
Q

What causes neuronal death in degeneration?

A

Diverse insults

Common final pathways (work in concert):

  • Excitotoxicity
  • Free radical injury
  • Protein fibrillogenesis (extra/intracellular)

(Death via either necrosis or apoptosis)

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6
Q

Consequences of neuronal death (broad, 2)

A
  • System dysfunction
  • NT loss
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7
Q

What does it mean if cellular reaction to injury is “reactive”?

A

It can recover (Necrosis-Apoptosis-Reactive-Normal)

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8
Q

What is protein fibrillogenesis?

A

Protein misfolds and starts to polymerize/accumulate, aggregating into fibrils or filaments which may lead to cellular dysfunction or death

(early phases e.g. fibrils rather than inclusions are more destructive)

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9
Q

Types of protein fibrillogenesis?

A

May be:

  • Extracellular protein deposit, “plaque”
  • Intracellular protein deposit, “inclusion” (most intra are cytoplasmic, but some nuclear) (Innocent bystander proteins may be incorporated into fibrillization/inclusions)
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10
Q

How can fibrillogenesis be identified?

A
  • Light or electron microscopy
  • Immunohistochemistry for inclusions in nucleus/cytoplasm
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11
Q

What is the “stress protein”? What does it do?

A

Ubiquitin

Marks the misfolded protein for refolding or degradation by proteasome

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12
Q

What is a non-proteasome pathway for degradation of misfolded protein?

A

Autophagy by lysosomes

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13
Q

Composition of cytoplasmic inclusions?

A

Structural protein:

  • Tau
  • Synuclein

Stress protein:

  • Ubiquitin
  • Crystalline
  • HSPs
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14
Q

Difference between structure and toxicity of fibrils vs. inclusions?

A

Fibrils: ultrastructural, nm, VERY toxic

Inclusions: light microscopy, um, less toxic

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15
Q

What is Tau protein? Causes what diseases?

A

Intracellular neuronal protein associated with microtubules

Causes “Tauopathies”

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16
Q

What is alpha-synuclein? Causes what diseases?

A

Intracellular neuronal and glial protein

Causes Lewy body disorders, e.g. Parkinson’s

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17
Q

What is superoxide dismutase (SOD)? Causes what diseases?

A

Intracellular neuronal protein

Causes Familial ALS (can oxidize just fine, but protein misfolds and causes problems)

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18
Q

What is beta-amyloid? Causes what diseases?

A

Extracellular polymeration

Causes Alzheimer’s Disease

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19
Q

T/F: Neuodegeneration is focal/localized? 3 examples?

A

False

Neurodegeneration may start focally and then spread

  • Alzheimer’s typ starts in hippocampus with memory problems (but may start in Broca’s area with progressive aphasia)
  • Parkinson’s may start in 1 substantia nigra; hemikparkinsonism
  • ALS may start focally (e.g. hand weakness on 1 side); as dz progresses, it spreads to other muscle groups
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20
Q

Three main steps in prion-like templating of neurodegeneration?

A
  • Misfolding (stochastic, failure to degrade, or genetic propensity to misfold)
  • Templating (misfolded pr modifies 2’ structure of native protein in self-replicating cycle)
  • Aggregation (misfolded prot tends to aggregate; IC = tangles)
21
Q

Examples of overlapping neurodegenerative diseases (2)

A
  • Parkinson’s: dementia following movement disorder
  • ALS: later frontotemporal dementia
22
Q

What is a prion?

A

Misfolded protein that is so capable of modifying 2’ structure of native protein in self-replicating cycle that it is INFECTIOUS

23
Q

What is this?

A

Cortical atrophy (caudate atrophy)

Can see hydrocephalus ex vacuo

24
Q

What is this?

A

Cortical atrophy: atrophic frontal/temporal lobes moreso than parietal/occipital

25
Q

Which side is abnormal?

In what way?

Disease?

A

Abnormal: right side

Problem: substantia nigra atrophy

Disease: Parkinson’s disease

26
Q

What are these histo pics of?

Which is abnormal?

Disease?

A

Pictures of substantia nigra

Abnormal: right side

Problem: Parkinson’s disease

27
Q

What is shown in this picture?

A

Lewy body in pigmented neuron in Parkinson’s disease

28
Q

What is the composition of Lewy bodies?

Extra or intracellular?

A
  • Intracellular (cytoplasmic)

Strucutral protein: synuclein

Stress protein:

  • Ubiquitin
  • Crystalline
  • HSPs
29
Q

What is shown in this picutre?

A

Cortical Lew bodies (a-Synuclein)

30
Q

Most common causes of dementia (due to cortical degeneration)?

A
  • Alzheimer’s
  • Pick’s disease (frontotemporal dementia)
  • Lewy body dementia (person with Parkinson’s can develop this or vice versa, this can lead into Parkinson’s)
31
Q

Important pic

A

KNOW THIS

32
Q

Symptoms of Parkinson’s disease (7)

A
  • Cogwheel rigidity
  • Resting tremor
  • Bradykinesis
  • Postural instability
  • Clasp knife tonicity ?

(-masked facies)

(-en bloc turning)

33
Q

Non a-synuclein misfolding (Lewy body formation) causes of Parkinsonism?

A
  • Post-encephalitic Parkinsonism
  • Progressive supranuclear palsy (tauopathy)
34
Q

What is this? (pic 8)

A

Progressive supranuclear palsy (PSP)

35
Q

What is this?

A

Tau immunoreactive Globose Nueronal tangle in PSP (progressive supranuclear palsy)

36
Q

What is multiple system atrophy (MSA)?

A

Degenerative dz with varying components of parkinsonism, cerebellar ataxia, and autonomic dysfunction once thought to be 3 separate diseases It is a synucleinopathy (a-synuclein); glial cytoplasmic inclusions, GCI

37
Q

What is underlying mechanism of the different symptoms seen in MSA (multiple system atrophy)?

A
  • Parkinsonism: striatonigral degeneration
  • Ataxia: olivopontocerebellar degeneration
  • Autonomic dysfct: Shy Drager dysautonomia
38
Q

What degenerates in MSA?

A
  • Substantia nigra
  • Putamen
  • Olivary nuclei
  • Pontine nuclei
  • Cerebellum
39
Q

What does this show?

A

Atrophy of pons in multiple system atrophy

40
Q

What does this show?

A

Alpha-synuclein positive inclusions in multiple system atrophy

41
Q

What is Friedreich’s ataxia?

What protein does it involve?

A

Most common hereditary ataxia

  • Autosomal recessive
  • GAA triplet repeat expansion (both alleles or one with expansion and other with mutation)
  • Involves frataxin but NOT appeared to be a fibrillogenesis-related dz
  • Results in loss of function
42
Q

What organs does Friedreich’s ataxia affect?

A
  • CNS
  • Heart
  • Pancreas
43
Q

What does this show?

A

Cerebellar atrophy seen in Friedreich’s ataxia and other spinocerebellar ataxias

44
Q

What does this show?

A

Friedreich’s ataxia Multiple long tracts affected, including psinocerebellar

45
Q

What does this show?

A

Amyotrophic lateral sclerosis (ALS)

46
Q

What parts of the SC are affected in ALS (tracts)?

A
  • Corticospinal tract (lateral and anterior/ventral)
  • Ventral horns
47
Q

What does this show?

A

Amyotrophic Lateral Sclerosis (ALS)

48
Q

What causes ALS?

A

Familial forms from SOD1 (superoxide dismutase) mutations

Protein fibrils formal and damage cells (not a loss of free radical handling! Still functional)

Forms amyloid-like filaments and water-filled nanotubes

Term 5: Neuro (24 decks)

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