Neurodegenerative disorders

Question 1

What are some general features of neurodegenerative disorders?

Answer 1

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

Question 2

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

Answer 2

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

Question 3

What is dementia?

Answer 3

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

Question 4

What is the MC cause of demntia?

Answer 4

Alzheimer disease (AD). Strongly associated with age

Question 5

What acummulats in the brain with AD?

Answer 5

Beta-amyloid proteins (plaques)

Question 6

What do B-amyloid palques cause in the brain?

Answer 6

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

Question 7

What do tau proteins indicate?

Answer 7

Breakdown of neurons. General neurodegeneration.

Question 8

How does AD progress?

Answer 8

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

Question 9

What is seen within 5-10 years with AD?

Answer 9

Aphasia, immobility. Lethal infx: pneumonia (MC)

Question 10

What is seen within 5-10 years with AD?

Answer 10

Aphasia, immobility. Lethal infx: pneumonia (MC)

Question 11

Is there a genetic component to AD?

Answer 11

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

Question 12

What is parkinsonism?

Answer 12

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

Question 13

What is the MC cause of parkinsonism?

Answer 13

Parkinson disease (But not always)

Question 14

What is damaged in parkinson disease?

Answer 14

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

Question 15

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

Answer 15

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

Question 16

What age group is MC affected by Parkinson’s?

Answer 16

Onset: Age 45-65

Question 17

What kind of discoloration is noted with Parkinson’s?

Answer 17

Pallor of the substantia nigra

Question 18

How progressive is Parkinsons?

Answer 18

Immobility 10-15 years

Question 19

What are some physical features of Parkinson’s?

Answer 19

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

Question 20

What are some behavior disorders in advanced stages of PD?

Answer 20

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

Question 21

What are some conditions associated with death in PD?

Answer 21

Frequent falls, infection (pneumonia).

Question 22

What is the Tx for Parkinson’s?

Answer 22

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

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

Question 23

What is Huntington disease?

Answer 23

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

Question 24

What are the genetic implications of HD?

Answer 24

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

Question 25

What are the inclusions associated with HD?

Answer 25

Ubiquinated Huntington protein

Question 26

What is the onset for HD?

Answer 26

Delayed onset: 30-40 years

Question 27

What are the features of HD?

Answer 27

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

Question 28

What is associated with death in HD?

Answer 28

Pneumonia Injuries Choking Suicide

Question 29

What is ALS?

Answer 29

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

Question 30

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

Answer 30

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

Question 31

What is the onset of ALS?

Answer 31

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

Question 32

What kind of atrophy is noted with ALS?

Answer 32

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

Question 33

What are the features of ALS?

Answer 33

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

Question 34

What is the onset like of ALS?

Answer 34

Rapid progression (Stephen Hawking: atypical)

Question 35

What causes death with ALS?

Answer 35

Respiratory paralysis (pneumonia)

Question 36

What causes ALS?

Answer 36

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

Question 37

What causes ALS?

Answer 37

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