Alzheimer's, FTLD, MS, ALS, AT (Exam 1) Flashcards
What is alzheimer’s disease?
slow progressive neurodegenerative disease that causes gradual loss of neurons in the entire cerebral cortex
What is the pathological hallmark of alzheimer’s disease?
amyloid plaques and neurofibrillary tangles!
-deposits of aggregated amyloid beta proteins in the synaptic cleft is the first sign
-neurofibrillary tangles (neurotubules degenerate and tau proteins clump together inside the neuron)
Amyloid protein precursors are normally found in the cell membrane. Normal cleavage of this precursor forms a harmless soluble peptide. Abnormal cleavage forms an insoluble AB peptide that aggregates and causes….
alzheimer’s disease
What disease causes the tau protein to hyperphosphorylate and can no longer hold the microtubule network together, they unravel and tau proteins get tangled up so there is no axoplasmic transport and results in cell death?
alzheimer’s disease
What are the clinical features of alzheimer’s disease?
-initially= forgetfulness and other memory issues
-later= language deficits, loss of math skills, and loss of learned motor skills
-final stages= dementia, incontinence, mute, non-ambulatory
What is the most common cause of dementia in elderly?
alzheimer’s disease
The amount of neurofibrillary tangles is directly proportional to the degree of….
alzheimer’s disease dementia (more tangles= more severe)
Amount of Ach is directly proportional to….
learning and memory recall (AB plaques inhibit acetylcholinesterase activity which decreases choline recovered by presynaptic neuron and reduces the amount available for learning and memory recall)
What is frontotemporal lobar degenerations?
-aggressive degeneration of the frontal and temporal lobes
-young onset
-loss of memory, spatial recognition, emotions, and logic
-can be inherited, sporadic.idiopathic, or infectious like herpes virus
-often misdiagnosed as psychiatric or alzheimer’s
-mutations of tau proteins (can either have TDP43 protein inclusion bodies OR tau protein inclusion bodies)
-has neurofibrillary tangles which damage cortical neurons
What is the most common cause of early onset dementia?
frontotemporal lobar degeneration
What are the clinical features of frontotemporal lobar degenerations?
-aphasia and dementia
-personality, behavior, or language changes before memory loss (which this is opposite for alzheimer’s disease)
What is Pick’s disease?
boards q
-type of frontotemporal lobar degeneration
-shows smooth contoured tau protein inclusion bodies in the neuron cell body
What is another name for amyotrophic lateral sclerosis (ALS)?
lou gehrig’s disease
What is ALS?
-progressive gradual death of voluntary motor neurons
-UMNs affect cerebral cortex
-LMNs affect spinal cord and brainstem
-degeneration of corticospinal tract
-results in denervation of muscles (profound muscle weakness)
-can be sporadic or familial
-toxic protein accumulation in UMN and LMNs
-DOES NOT damage extraocular eye muscles
-TDP43 protein inclusion bodies in motor neurons
-loss of myelinated fibers on corticospinal tracts
-degeneration of anterior roots
-decreases ventral horn motor neurons
What are the clinical features of ALS?
-asymmetric weakness of the hands
-dropping objects
-difficulty in performing fine motor tasks
-cramping and spasticity of the arms and legs
as it progresses
-loss of muscle strength, muscle atrophy and fasciculations (little twitches)
-weakness of respiratory muscles (dysphagia and dysarthria)
