Module 4 Flashcards

Question

What is spinocerebellar Ataxias?

Answer 1

- Affects spinal cord and/or cerebellum -> impairment of balance and coordination of movement called "ataxia"

Answer 2

Affects peripheral neurons system, PNS

Answer 3

- Older age: higher risk factor -> defects in mitochondrial metabolism -> higher production of reactive oxygen (unstable o2 that can damage cells), cell injury, cell death - Protein misfolding: "aggregation" - Synaptic dysfunction: disturbance in the structure or function of the synapse, EARLY EVENT - Neuronal cell death: neurons are stressed and maladapted under a disorder-> treatment: prevent neuronal death (limited clinical benefits)

Answer 4

- Decline in cognitive (conscious intellectual activity) function: sufficient to interfere with the activities of daily living - Decline in: memory, thinking skills, reasoning, complex motor skills (caused by neurodegenerative diseases) - The term dementia does NOT imply an underlying cause - AD and Lewy Body dementia are the 1st and the 3rd most common cause of dementia - Incurable - Treatment for: slowing progression of symptoms and prolong cognitive function (Memory problems, characteristic of dementia, may be caused by other conditions that are treatable and curable, such as vitamin deficiency, thyroid problems, viral or bacterial infections, medication side effects, stress, or depression. If treated, patients may regain some or all of their cognitive function)

Answer 5

- Age (not apart of a normal aging but a form of cognitive decline) - Health factors: genetic diabetes, high blood pressure, unhealthy cholesterol levels - Lifestyle/ dietary factors: smoking, physical inactivity, obesity, drug and alcohol abuse, poor access to goof diet Mental health: PTSD, depression, schizophrenia

Answer 6

- Up to 10 years earlier - To the point where to them dementia (memory loss, confusion, and forgetfulness) is considered a normal part of aging - Rather, terms used to describe dementia are gentle and humorous such as “mind changes”, “buried memories”, “forgetful”, etc. Symptoms related to dementia have been described by Indigenous Peoples as a “second childhood” and a time when one is “closer to the Creator.” Through this view, memory loss is not seen as a negative experience, but as another part of the circle of life. - Memory loss is not associated with the illness ---> late dignoses

Answer 7

I-CAARE: The Indigenous Cognition and Aging Awareness Research Exchange: use of medicine wheel - look at the examples of intregating the medicine wheel

Answer 8

used to evaluate judgement, reasoning, memory, problem solving… to detect cognitive impairment - They vary in length, and combine written, oral and listening components - These tests assess a variety of skills and ability such as orientation, short-term memory, and visuoconstructive skills (The ability to organize and manually manipulate spatial information to make a design).

Answer 9

- Orientation: patients are asked to state the current date, their address or city - Short term memory: patients are given 5 words and asked to repeat them. They are then asked to perform a separate task before repeating the words again. - Visuoconstructive: patients are asked to draw a clock indicating a given time. This task also requires auditory comprehension and memory. - MOST COMMON -> Montreal Cognitive Assessment (MoCA) (the clock)

Answer 10

- Progressive neurodegenerative disease - Most common cause of dementia (50-75%) - Average life span after diagnoses (4-8 years) but sometimes 20 years - Global crisis (As our population continues to age, the number of people living with A D is expected to double by 2030 and more than triple by 2050 - double in Indigenous people)

Answer 11

- Genetics: caused by inherited mutations that affect amyloid-B processing + other mutation developing the more common, sporadic form of A D - Sex: higher in women (double) -> bc of hormones and grater life expectancy Lifestyle: modifiable risk factor (diabetes, obesity, depression, smoking, and low educational attainment)

Answer 12

(not fully understood), alteration in biology of two proteins (Amyloid-B and tau proteins) causes their misfolding and aggregation, resulting in injury and ultimate death of neurons

Answer 13

- Amyloid-B: small protein formed by cleavage (cutting of a protein at a specific site) of a larger protein called amyloid precursor protein (APP) found in the cell membrane of neurons Ø AD: AB easily aggregates is cleaved from APP -> aggregates/oligomers -> concentrate at synapse and disrupt their function Ø Outside the neuron: the AB oligomers further aggregate into larger structures called AB plaques -> activation of microglia and release of inflammatory mediators -> neuronal injury It can aggrate and damage the walls of the brain blood vessels (this condition is known as Cerebral Amyloid Angiopathy (CAA), can increase the risk of a stroke)

Answer 14

- Tau: Protein that binds to microtubules, which are important for the transport of vesicles and mitochondria along axons Ø Normal tau: binds to microtubules and promotes their stability Ø AD: it misfolds -> binds less strongly to microtubules -> disassembles and disrupt proper neurotransmission Ø The misfolds: forms oligomers and aggrates in the neuron as neurofibrillary tangles (tau tangles), which are toxic to neurons Ø Misfolded tau can also pass through synapses and "spread" to other neurons -> misfolding across the brain

Answer 15

how AD develops - Accumulation and deposition of misfolded AB in the brain is primary the cause of AD Neurofibrillary tangles occur later in this processes

Answer 16

1. A β is produced by the cleavage of A P P in the cell membrane of neurons. 2. In the space between neurons, A β oligomers are thought to disrupt the function of synapses. 3. A β oligomers aggregate into plaques outside the cell, disrupting neuron functions. 4. A β deposits outside the cell activate microglial cells triggering a harmful inflammatory response. 5. Misfolded tau aggregate into neurofibrillary tangles inside neurons, displacing intracellular organelles. 6. Misfolded tau can pass through synapses into other neurons, where it catalyzes further misfolding of tau.

Answer 17

(caused by the increasing amount of tangles over time) - Early stage: AD begins in or near the hippocampus (involved in encoding and retrieval of memory and spatial navigation) located on the medial surface of each lobe of the brain - Middle stage: large areas of the cerebral cortex are involved by amyloid-B plaques and neurofibrillary tangles -> progressive death of neurons Late stage: takes several years to become severe

Answer 18

Moderate to severe changes in the structure of the brain are obvious to the naked eye - Cerebral cortical atrophy: outermost layer of brain cell (cerebral cortex)-> atrophy, decreases the overall size and weight of the brain - Enlarged ventricles (brain fluid filled space): faster degeneration of the brain tissue (process: ex-vacuo hydrocephalus) Hippocampal (region where early pathological changes of AD are first seen) Atrophy: hippocampus progressively atrophies (shrinks) as a result of neuronal death

Answer 19

- Tests the function of electrical signals in the nerves - By placing a small electrodes on the skin + transmitting a small amount of electricity to the electrodes Weak response may indicate an issue with signal transmission

Answer 20

- Inserting a thin needle into one of the muscles, this needle is attached to a wire that connects to a machine The doctor will ask the patient to flex and release the muscle -> machine measures the muscle's electrical activity

Answer 21

- Checks for hereditary neuropathies (damages or dysfunction of one or more neurons) Performed using a sample of blood sent for DNA analysis

Answer 22

- Most common inherited neurological disorder - Onset is usually in childhood - Characterized by a progressive weakness and atrophy (muscle wasting due to reduction in the size of the cell, organ or tissue) beginning in legs - Will lead to diminished or absent muscle stretch reflexes - Sensory deficits can also occur (mild) - Slow-progressing disease No impact on life span

Answer 23

- There is no single gene associated with it More than 100 genes have been linked to CMT

Answer 24

- Demyelination neuropathy - Mutations affect Schwann cells - Slowly progression - Patients have weakness/ atrophy in the lower legs starting in childhood - Later may experience hand weakness and loss of sensation, along with foot and leg problems

Answer 25

- Axonal neuropathy - Mutation cause the degeneration of neuronal axons -> loss of nerve supply to muscles and atrophy of skeletal muscle - Similar symptoms to CMT 1 but milder!!!!! Less disability and sensory loss - Noticed in childhood or adolescence

Answer 26

Physiotherapy HELPS: to maintain muscle and prevent further atrophy and the routine is unique to the patients