Flashcards in Degenerative Disorders: Krafts Deck (23):
What is death usually from in an Alzheimer's patient?
Name key features of Alzheimer disease
Cortex degenerated, Brain atrophy, dementia, Plaques and tangles, 3-20 year prognosis
What is within the amyloid plaques?
A Beta Peptide
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)
What contains Tau protein?
Are tangles intra or extracellular?
intracellular (cell body)
Early on, what two centers are affected in Alzheimer disease?
Language and memory centers
Tau accumulation seems to be secondary to what?
A Beta peptide accumulation
What is the normal function for tau protein?
Helps keep microtubules together in an axon
What lobes are degenerated in Pick Disease and what is the result?
Frontal lobe: personality changes
Temporal lobe: language disturbances
In Pick disease, what cytoplasmic neuronal inclusions do you see and what do they contain?
Pick bodies: contain Tau protein
In what area are neurons degenerated in Parkinson disease?
Substantia nigra (dopaminergic neurons that project to striatum)
What do you see microscopically in Parkinson disease?
What is the life expectancy of a Parkinson's patient?
not really shortened, but not a great quality
What is a Parkinsonian gait called?
Where do they place the electrodes in the brain of a Parkinson Disease patient?
What is degenerated in Amyotrophic Lateral Sclerosis?
motor neurons (upper and lower)
What happens to the patient's muscles in ALS?
Atrophy (LMN issue)
Are sensory and cognition affected in ALS?
How long does an ALS patient usually live and why do they die?
2-3 years, due to respiratory compromise
What are the clinical features (chronologically) of ALS?
Early: Asymmetric hand weakness, twitching, arm/leg spasticity, slurred speech
Then: muscle atrophy, fasiculations, creeping paralysis
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.)