Degenerative Disorders Flashcards

0
Q

Where does dengeneration occur in Parkinson’s?

A

SN compacta

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1
Q

Where does dengeneration occur in Alzheimer’s and pick disease?

A

The cortex

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2
Q

Where does dengeneration occur in Amyotropic lateral sclerosis?

A

Peripheral muscles

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3
Q

What is the main things of Alzheimer’s disease?

A
  • cortex
  • dementia
  • gross: atrophy
  • microscopic plaques and tangles
  • hippocampus, language centers
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4
Q

What is the clinical picture of Alzheimer’s?

A

Most common cause of dementia in elderly (60)
- stages
Early: forgetfulness, mood/behavioral changes
Next: disorientation, memory loss, aphasia, loss of motor skills
5-10 years: Px profoundly disabled, mute, immobile, death from infection usually

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5
Q

What changes happen when a Px comes in for Alzheimer’s?

A

Hippocampus and frontal cortex

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6
Q

What is the morphology of AD?

A

Amyloid plaques - tortuous neurite, amyloid beta peptide

Tangles - tau, MAP2, ubiquitin

Other: gliosis, neuronal loss, cerebral amyloid in vessels

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7
Q

What do you view plaques and amyloid with?

A

Plaques - silver stain for cell processes too

Amyloid - Congo red - polarized light looks green

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8
Q

What are neurofibrillary tangles?

A

Tau tangles

Kills neurons

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9
Q

How do you diagnosis Alzheimer’s?

A

Clinically

  • APP, aggregates are neurotoxicity and pro-inflammatory
    Number of plaques and tangles link with symptoms
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10
Q

What is tau accumulation caused by?

A

A beta peptide (amyloid) abnormal

Comes from APP

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11
Q

How does B amyloid abnormality lead to tau accumulation?

A

B amyloid breaks microtubules

Allowing tau to be hyperphosphorylated and accumulate in the cell body

Not fully supported

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12
Q

What is Pick disease?

A

Frontotemporal lobar degeneration/dementia

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13
Q

What must you know in pick disease (FTLD)?

A
  • Personality and language changes PRESENTATION
  • severe atrophy of frontal and temporal lobe
  • pick bodies containing tau protein
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14
Q

What is the progress of Pick disease?

A

60 or younger

  • frontal lobe signs (personality) temporal signs (language)
  • later dementia
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15
Q

What is a pick body?

A

Tau protein aggregation within neurons

Use silver stain

16
Q

What is the main sign you see in Parkinson’s?

A

Trouble initiating voluntary movements

Mask like facees

Pill rolling tremor

17
Q

What are the main things you need to know about Parkinson’s?

A
  • degen of SN
  • tremor, rigidity, bradykinesia
  • Lewy bodies
  • slightly shortened life span
18
Q

What is Parkinsonism?

A
  • masked facees
  • stooped posture
  • slowness of voluntary movement
  • festinanting gait
  • rigidity
  • pill-rolling tremor
19
Q

How do you test for Parkinson’s?

A

See if they respond to L-DOPA

20
Q

Why is the SN dark?

A

There is melanin in it

21
Q

What are Lewy bodies?

A

Melanin containing body

22
Q

What is the Parkinson’s pathogenesis?

A

Alpha synuclein

23
Q

What is ALS?

A

Lugarigs disease

24
Q

What must you know about ALS?

A
  • degeneration of motor neurons
  • rapidly progressive weakness, spasticity, and dysphagia
  • sensory and cognitive function are unaffected
  • death within 2-3 years due to respiratory compromise
  • peripheral muscles atrophy
25
Q

What are clinical features of ALS?

A

Early: asymmetric hand weakness, arm/leg spasticity, twitching, slurred speech
Later: atrophy, fasciculations, creeping paralysis
Eventually: respiratory muscles involved (infection)

26
Q

What is the pathogenesis of ALS?

A
  • cause of motor neuron degeneration is not well understood
  • SOD is mutated in inherited cases but lack of degeneration doesn’t seem to be the cause
  • bottom line, degeneration often with toxic protein accumulation
    SOD (superoxide dismutase)
27
Q

What is the morphology of ALS?

A
  • thin anterior roots of spinal cord
  • degeneration of corticospinal tracts
  • decrease in anterior horn neurons
  • skeletal muscles atrophy