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Systems Path 1 > Neurodegenerative disorders > Flashcards

Neurodegenerative disorders Flashcards

1
Q

What are some general features of neurodegenerative disorders?

A

CNS degeneration, abnormal prtein accumulation. Patterns of neuronal loss, functional impact.

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2
Q

How are the cerebellum, motor neurons, and cortex affected in neurodegenerative conditions?

A

Cerebellum: ataxia
Motor neurons: weakness (e.g ALS)
Cortex: memory, language, insight/planning

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3
Q

What is dementia?

A

Impaired memory and cognition along with personality changes; decreased overall fxnality. Maintain consciousness, common feature of neurodegeneration.

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4
Q

What is the MC cause of demntia?

A

Alzheimer disease (AD). Strongly associated with age

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5
Q

What acummulats in the brain with AD?

A

Beta-amyloid proteins (plaques)

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6
Q

What do B-amyloid palques cause in the brain?

A

Neurotoxic, decreased neurotransmission, cortical atrophy. Breakdown = tau proteins (tangles).

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7
Q

What do tau proteins indicate?

A

Breakdown of neurons. General neurodegeneration.

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8
Q

How does AD progress?

A

Insidious/progressive: altered mood/behavior decreased cognition, disorientation, amnesia

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9
Q

What is seen within 5-10 years with AD?

A

Aphasia, immobility. Lethal infx: pneumonia (MC)

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10
Q

What is seen within 5-10 years with AD?

A

Aphasia, immobility. Lethal infx: pneumonia (MC)

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11
Q

Is there a genetic component to AD?

A

10% genetic. Linked with down syndrome bc of chromosome 21

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12
Q

What is parkinsonism?

A

Abormal motor fxn., tremor, rigidity, bradykinesia, instability

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13
Q

What is the MC cause of parkinsonism?

A

Parkinson disease (But not always)

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14
Q

What is damaged in parkinson disease?

A

Damage to dopaminergic neuron. Altered CNS synaptic transmission. Substantia nigra **, cortex, medulla, pons.

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15
Q

What kind of inclusions are associated with Parkinson’s disease?

A

Alpha-synuclein = Lewy body (round/oval, dense core, pale halo).

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16
Q

What age group is MC affected by Parkinson’s?

A

Onset: Age 45-65

17
Q

What kind of discoloration is noted with Parkinson’s?

A

Pallor of the substantia nigra

18
Q

How progressive is Parkinsons?

A

Immobility 10-15 years

19
Q

What are some physical features of Parkinson’s?

A

“Cogwheel” rigidity & festinating (shuffling) gait
“Pill-rolling” of thumb & index fingers
Exressionless (poker/mask face)

20
Q

What are some behavior disorders in advanced stages of PD?

A

Dementia, hallucinations (cortex) within 1 year of motor symptoms (Lewy body dementia).
Possible autonomic dysfunction

21
Q

What are some conditions associated with death in PD?

A

Frequent falls, infection (pneumonia).

22
Q

What is the Tx for Parkinson’s?

A

L-DOPA (increased dopamine), deep brain stim.

Manages movement dysfunction, does not slow progression, becomes less effective

23
Q

What is Huntington disease?

A

Progressive neurodegeneration. Severe cerebral atrophy. Prominent at caudate and putamen nuclei

24
Q

What are the genetic implications of HD?

A

Autosomal dominant, Huntington gene. Trinucleotide repeat: CAG (anticipation).

25
Q

What are the inclusions associated with HD?

A

Ubiquinated Huntington protein

26
Q

What is the onset for HD?

A

Delayed onset: 30-40 years

27
Q

What are the features of HD?

A

Chorea (jerky, dance-like) of entire body.
Altered congnition: severe dementia, insanity
MC lethal within 15 years.

28
Q

What is associated with death in HD?

A

Pneumonia
Injuries
Choking
Suicide

29
Q

What is ALS?

A

Denervation atrophy of motor neurons. Unique combination of LMN and UMN death.

30
Q

What are the areas of LMN and UMN that are killed with ALS?

A

LMN: Cord, brainstem. Corticospinal and corticobulbar tracts. Anerior horn cells, ventral nerve roots.
UMN: Betz cells (posterior frontal lobe)

31
Q

What is the onset of ALS?

A

Insidious: asymmetric distal extremity weakness. MC among males, age: 40-50 years.

32
Q

What kind of atrophy is noted with ALS?

A

Progressive muscle atrophy and decreased strength. Fasciculation: involuntary muscle contractions

33
Q

What are the features of ALS?

A

Weakness, hyperreflexia (bc of betz cells), spasticity. Preserves sensation and extraocular motor fxn. Difficulty speaking, swallowing, breathing

34
Q

What is the onset like of ALS?

A

Rapid progression (Stephen Hawking: atypical)

35
Q

What causes death with ALS?

A

Respiratory paralysis (pneumonia)

36
Q

What causes ALS?

A

90% sporadic. 10% are familial, autosomal dominant (earlier onset) - associated with superoxide dismutase abnormalities.

37
Q

What causes ALS?

A

90% sporadic. 10% are familial, autosomal dominant (earlier onset) - associated with superoxide dismutase abnormalities.

Systems Path 1 (9 decks)

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