Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neuroscience > Neurodegenerative diseases > Flashcards

Neurodegenerative diseases Flashcards

1
Q

neurodegenerative disorders

A

characterized by progressive dysfunction and death of neurons
- Abnormal accumulations of specific proteins in the brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

degeneration repercussions

A

often affects specific systems, implying selective vulnerability ⇒ not all brain regions are the same and some may be affected by certain toxins or not

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

pathophysiology of neurodegenerative diseases (3)

A
  • Aging is generally the most important risk factor
  • Interaction between genetic and environmental factors ⇒ Parkinson’s and MS
  • Genetic and sporadic forms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

prevalence

A

proportion of people in the population affected by each disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

incidence

A

rate or frequency of the disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what % of neurodegenerative disease is AD?

A

75% followed by parkinson’s and MS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

types of neurodegenerative diseases (2)

A
  1. cognitive
  2. motor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

cognitive disorders

A

degeneration affects the cerebral cortex, leading to dementia
- Alzheimer’s disease (AD), frontotemporal dementia (FTD), Pick’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

motor disorders (4)

A
  1. Degeneration affects motor neurons leading to motor weakness
    - Amyotrophic Lateral Sclerosis (ALS) aka Lou Gherigs disease, spinal muscular atrophy (SMA)
  2. Degeneration affects cerebellum and tracts leading to Cerebellar ataxia
    - Spinocerebellar ataxias (SCA)
  3. Degeneration affects substantia nigra and basal ganglia leading to akinetic and rigid movements
    - Parkinson’s disease (PD), progressive supranuclear palsy (PSP) => akinetic
  4. Degeneration affects basal ganglia leading to hyperkinetic movements
    - Huntington’s disease (HD) => hyperkinetic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Ataxia

A

lack of voluntary coordination of muscle movement and ability to maintain proper gait when standing and walking ⇒ can translate into speech abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Akinetic

A

Akinetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

hyperkinetic

A

high tuned and sometimes uncontrolled muscle movements

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

brain structures affected in Huntingtons

A

caudate nucleus, striatum, and frontal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

brain structures affected in AD

A

hippocampus, entorhinal, and frontal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

brain structures affected in frontotemporal dementia

A

frontal and temporal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

brain structures affected in PD

A

substantia nigra

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

brain structures affected in cerebellar ataxia

A

cerebellum neuron loss
- all brain cells may express mutant proteins but certain brain regions will degrade first

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

brain structures affected in Amyotrophic lateral sclerosis

A

motor neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

protein aggregates

A

occur within cells (protein inclusions) and occur outside the cells in extracellular space (plaque)
- There can be misfolded proteins that form aggregated inside and outside of cells
- there are both neuron and glial inclusions in the disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

how does inflammation work in neurodegenerative diseases?

A

we often see inflammation due to the substances which the body will attack as if it is foreign
- protein aggregates will form in cells and the neurons will be harmed as they become larger
- The neurons will release molecules called DAMPS ⇒ damage associated molecular patterns

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

DAMPS

A

molecules that can activate certain receptors in immune cells
- Microglia share same origin as immune cells which express pattern recognition receptors that sense the presence of DAMPS to start immune response
- This triggers an inflammation cascade
- Particularly in areas where the blood brain barrier is compromised ⇒ macrophages will invade the brain and contribute to further information which creates worse symptoms

22
Q

Alzheimer’s disease

A

progressive neurodegenerative dementia, whose duration (death) of the disease can vary from 3 years to 20 years ⇒ after clinical manifestation
- Most common type of dementia in the elderly ⇒ decline in mental ability severe enough to interfere with daily life
- Difficult to perform activities of daily living

23
Q

ow many Americans have dementia in 2024

A

7 million

24
Q

classifications of AD (% genetic)

A
  • 98% sporadic ⇒ late onset AD
  • 2% familial ⇒ early onset AD (as early as 24)
25
Q

genes in AD (3)

A
  • APP ⇒ chromosome 21 (Amyloid Precursor Protein)
  • PSEN1 ⇒ chromosome 14 (Presenilins)
  • PSEN2 ⇒ chromosome 1 (Presenilins)
26
Q

histological features of AD (4)

A
  • Gyri are smaller and sulci are larger (loss of 30%)
  • the lateral ventricles are larger and so is 3rd ventricle
  • hippocampus is mostly gone and the surrounding enteral cortex is also gone
  • extracellular accumulation of amyloid-beta (Abeta) in amyloid plaques (brown) and intracellular accumulation of tau inclusions forming neurofibrillary tangles (black)
27
Q

MCI AD; characterization?

A

mild cognitive impairment which leds to AD ⇒ characterized by neuronal death (first phase)

28
Q

Preclinical AD

A

misfolding and aggregation of ABeta and Tau leading to plaques and NFT’s
- 2-3 decades long ⇒ can tell us there may be identifiable signs

29
Q

clinical presentation of Alzheimers early on (3)

A
  • Memory loss that disrupts daily life
  • Confusion with time or place
  • Trouble with visual and spatial
30
Q

clinical presentation of Alzheimers later on (4)

A
  • Challenges in planning or solving problems
  • New problems with word finding and speaking
  • Decreased or poor judgment
  • Changes in mood or personality (second phase)
31
Q

Apolipoprotein E (APOE4)

A

depending on how many copies your risk will increase for AD
- 2, 3, or 4 copies
- 2 copies makes you 12x more likely
- 1 copy makes you 3x more likely

32
Q

symptomatic treatments for AD (2)

A
  1. Cholinesterase inhibitors ⇒ the original brain area that degenerates characteristically has cholinesterase
    - Donepezil (Aricept)
    - Rivastigmine (Excelon)
    - Galantamine (Razadyne)
  2. NMDA receptor antagonists ⇒ prevent neuron loss
    - Memantine-HCl (Namenda)
33
Q

disease modifying agents for AD

A

Aggregated ABeta antibodies ⇒ antibodies targeting ABeta only for those in MCI stage
- Donanemab (Kisunla)
- Lecanemab (Lequembi)

34
Q

what happens to some people taking the ABeta antibodies?

A

may have swelling/bleeds in the brian which can cause seizures and other symptoms (rare)
- APOE4 carriers are at greater risk of complications
- only for MCI and mild AD with relatively modest effects of 20%

35
Q

Parkinson’s disease

A

belongs to a group of conditions called motor system disorders which are the result of the loss of dopamine-producing brain cells in the substantia nigra (>50-70%)
- Named after physician James Parkinson

36
Q

classifications of PD (% genetic)

A
  • 95% sporadic and occurs late onset PD
  • 5% familial for early onset PD
37
Q

genes for PD (5)

A
  • SNCA (alpha-synuclein)
  • Parkin
  • DJ-1
  • PINK1 (PTEN-induced kinase 1)
  • LRRK2 (Leucine-Rich repeat kinase 2)
38
Q

Sporadic PD

A

associated with risk factors, notably aging (>50 years) and exposure to pesticides (rotenone) or herbicides (Agent orange) ⇒ why midwest has a lot of cases particularly

39
Q

histological features of PD

A

loss of neurons in Substanta nigra
- Lewy bodies and Lewy neurites are intracellular aggregates of alpha-synuclein in the soma and neurites respectively

40
Q

symptoms of PD (4)

A
  • Tremor: trembling in hands, arms, legs, jaw, and face ⇒ resting tremor
  • Rigidity: stiffness of the limbs and trunk
  • Bradykinesia: slowness of movement ⇒ patients usually cannot turn while walking
  • Postural instability: impaired balance and coordination
41
Q

PD onset symptoms that are non-motor (5)

A
  • Sleep disorders
  • Autonomic function
  • Sensation
  • Mood disorders
  • Cognitive impairment
    → in the advanced stages of the disease, cognitive and behavioral problems may arise, with dementia commonly occurring ⇒ 30-80% of subjects with PD
42
Q

symptomatic treatments of PD (3)

A
  • L-Dopa ⇒ complement the loss of dopamine cells
  • Dopamine receptor agonist (loss dop cells)
  • Deep brian stimulation (DBS) ⇒ UMN does this which introduces a pacemaker in the brain to stimulate regions of specific neurons
    Note: there is currently no cure but treatments exist to drastically relieve motor symptoms
43
Q

Multiple Sclerosis

A

chronic, inflammatory, demyelinating and neurodegenerative disease of the central nervous system (CNS) ⇒ likely affecting glial cells
- MS is a heterogenous, multifactorial, immune mediated (autoimmune) disease that is caused by complex gene environment interactions
- Immune cells attack the glial cells myelinating in the CNS

44
Q

4 clinical courses of MS

A
  1. Relapsing remitting MS (RRMS) ⇒ 85%
  2. Secondary progressive MS (SPMS) ⇒ 85%
  3. Primary progressive MS (PPMS) ⇒ 10-15% (gradually progressive)
  4. Progressive relapsing MS (PRMS) ⇒ rare
45
Q

symptoms of MS (4)

A

Immune mediated inflammatory demyelinating disease of the CNS
- Optic neuritis (early)
- Clumsiness and muscle weakness
- Paresthesias ⇒ abnormal feeling in skin
- Lhermitte sign ⇒ discharge feelings when you do movements of your neck

46
Q

treatment options of MS (3)

A
  • corticosteroids
  • Beta interferons 1A and 1B
  • glatiramer acetate
47
Q

pathological features of MS (6)

A
  • white matter ⇒ myelin pathology (blue arrowheads) by antibody labeling (brown) or by MRI
  • Retina ⇒ thinning of the peripapillary retinal nerve fiber layer
  • grey matter ⇒ myelin pathology (blue arrowheads) by antibody labeling (brown) or by MRI
  • meninges ⇒ vascular pathology (blue arrowheads) by MRI or by histology staining pink
  • optic nerve ⇒ myelin pathology by MRI (pink circle) and antibody labeling (brown)
  • thalamus and pons ⇒ myelin pathology (blue arrowheads) by antibody labeling (brown) or by MRI
48
Q

pathological hallmarks of MS plaques (4)

A
  • Inflammation
  • Demyelination
  • Oligodendrocyte death
  • Axon degeneration
49
Q

symptomatic treatments for MS (2)

A
  1. Corticosteroids
    - Reduce inflammation
  2. Plasma exchange
    - Slows down functional decline (breathing)
50
Q

Disease modifying treatments for MS

A
  1. Ocrelizumab (Ocrevus)
    - For primary progressive MS and only FDA approved disease modifying therapy ⇒ those receiving the treatment are slightly less likely to progress than untreated people
  2. Many treatment options available
    - For relapsing remitting Ms, several disease modifying therapies are available
  3. New treatments soon possibly
    - For secondary progressive MS there is tolebrutinib (Sanofi) which is a BTK inhibitor ⇒ 30% delay in onset of disability progression
51
Q
A

Neuroscience (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions