Degenerative Disorders: Krafts Flashcards Preview

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Flashcards in Degenerative Disorders: Krafts Deck (23):
1

What is death usually from in an Alzheimer's patient?

Infection (Pneumonia)

2

Name key features of Alzheimer disease

Cortex degenerated, Brain atrophy, dementia, Plaques and tangles, 3-20 year prognosis

3

What is within the amyloid plaques?

A Beta Peptide

4

Where does A Beta peptide come from?

abnormal processing of Amyloid Precursor Protein (APP) via Beta-secretase (instead of alpha-secretase which normally cleaves it)

5

What contains Tau protein?

Neurofibrillary tangles

6

Are tangles intra or extracellular?

intracellular (cell body)

7

Early on, what two centers are affected in Alzheimer disease?

Language and memory centers

8

Tau accumulation seems to be secondary to what?

A Beta peptide accumulation

9

What is the normal function for tau protein?

Helps keep microtubules together in an axon

10

What lobes are degenerated in Pick Disease and what is the result?

Frontal lobe: personality changes
Temporal lobe: language disturbances

11

In Pick disease, what cytoplasmic neuronal inclusions do you see and what do they contain?

Pick bodies: contain Tau protein

12

In what area are neurons degenerated in Parkinson disease?

Substantia nigra (dopaminergic neurons that project to striatum)

13

What do you see microscopically in Parkinson disease?

Lewy bodies

14

What is the life expectancy of a Parkinson's patient?

not really shortened, but not a great quality

15

What is a Parkinsonian gait called?

Festinating

16

Where do they place the electrodes in the brain of a Parkinson Disease patient?

Thalamus

17

What is degenerated in Amyotrophic Lateral Sclerosis?

motor neurons (upper and lower)

18

What happens to the patient's muscles in ALS?

Atrophy (LMN issue)

19

Are sensory and cognition affected in ALS?

NO

20

How long does an ALS patient usually live and why do they die?

2-3 years, due to respiratory compromise

21

What are the clinical features (chronologically) of ALS?

Early: Asymmetric hand weakness, twitching, arm/leg spasticity, slurred speech
Then: muscle atrophy, fasiculations, creeping paralysis

22

What is meant by "progressive bulbar palsy" vs "progressive muscular atrophy" in ALS?

Muscular atrophy is due to lower motor neuron degeneration, progressive bulbar palsy is due to degeneration of brainstem motor nuclei (affects swallowing and speaking etc.)

23

What is the ALS morphology?

Thin anterior spinal roots, reduction in anterior horn neurons, atrophied skeletal muscle, degeneration of corticospinal tracts