Degenerative Disorders: Krafts

Question 1

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

Answer 1

Infection (Pneumonia)

Question 2

Name key features of Alzheimer disease

Answer 2

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

Question 3

What is within the amyloid plaques?

Answer 3

A Beta Peptide

Question 4

Where does A Beta peptide come from?

Answer 4

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

Question 5

What contains Tau protein?

Answer 5

Neurofibrillary tangles

Question 6

Are tangles intra or extracellular?

Answer 6

intracellular (cell body)

Question 7

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

Answer 7

Language and memory centers

Question 8

Tau accumulation seems to be secondary to what?

Answer 8

A Beta peptide accumulation

Question 9

What is the normal function for tau protein?

Answer 9

Helps keep microtubules together in an axon

Question 10

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

Answer 10

Frontal lobe: personality changes

Temporal lobe: language disturbances

Question 11

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

Answer 11

Pick bodies: contain Tau protein

Question 12

In what area are neurons degenerated in Parkinson disease?

Answer 12

Substantia nigra (dopaminergic neurons that project to striatum)

Question 13

What do you see microscopically in Parkinson disease?

Answer 13

Lewy bodies

Question 14

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

Answer 14

not really shortened, but not a great quality

Question 15

What is a Parkinsonian gait called?

Answer 15

Festinating

Question 16

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

Answer 16

Thalamus

Question 17

What is degenerated in Amyotrophic Lateral Sclerosis?

Answer 17

motor neurons (upper and lower)

Question 18

What happens to the patient’s muscles in ALS?

Answer 18

Atrophy (LMN issue)

Question 19

Are sensory and cognition affected in ALS?

Answer 19

NO

Question 20

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

Answer 20

2-3 years, due to respiratory compromise

Question 21

What are the clinical features (chronologically) of ALS?

Answer 21

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

Question 22

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

Answer 22

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.)

Question 23

What is the ALS morphology?

Answer 23

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