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BB Test 4 > Neurodegenerative Diseases > Flashcards

Neurodegenerative Diseases Flashcards

1
Q

What is the definition of dementia?

A
  • A progressive loss of cognitive function
  • Multiple cognitive domains affects (language, motor skills, calculations, spatial reasoning, judgment, memory (short and long term)
  • May be gradually progressive or stepwise
  • NOT a function of normal aging
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2
Q

What are some treatable causes of dementia?

A
  • Vitamin B12 (cobalamin) or B1 (thiamine) deficiency
  • Toxins (lead, mercury, alcohol)
  • Depression (pseudodementia)
  • Infections (syphilis, HIV, etc)
  • Endocrine problems (adrenal disease, thyroid disease)
  • Inflammation (vasculitis)
  • Structural problems (brain tumors, NPH)
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3
Q

Which gender is at greatest risk of Alzheimer’s?

A

Women (2:1 ratio, but might be due to larger women population)

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4
Q

What is Mild Cognitive Impairment?

A
  • Memory complaints with objective evidence of impaired recent memory, but intact daily function and intact non-memory cognitive function
    • Not all MCI develop into AD
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5
Q

What are some risk factors for developing Alzheimer’s?

A
  • Old age
  • Lower baseline intelligence and/or education level
  • Smaller head size
  • Family history of AD
  • History of significant head injury (in males)
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6
Q

What is the clinical presentation of Alzheimer’s?

A
  • Primary symptom is progressive memory loss (initially short term, long term later on)
  • Language disturbances
  • Apraxia (loss of ability to perform learned tasks)
  • Acalculia (trouble with calculations)
  • Visuospatial difficulty
  • Sequencing problems
  • Behavioral problems
  • Neurological problems (EPS, loss of frontal lobe inhibition, urinary incontinence, seizures, abulia, death)
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7
Q

What is diagnostic of Alzheimer‘s disease (AD)?

A
  • Neurofibrillary tangles upon autopsy
  • β-amyloid plaques
  • Sulci widening due to atrophy of the brain
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8
Q

What area of the brain and what neurotransmitter is affects in Alzheimers?

A
  • Basal Nucleus of Meynert (BNM)
  • ACh is decreased in AD
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9
Q

What is the result of β-amyloid accumulation?

A
  • May stimulate free radical production resulting in neuronal apoptosis (programmed cell death)
  • Free radicals result in MLP (membrane lipid peroxidatoin)
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10
Q

Describe the way β-APP is cleaved correctly?

A
  1. α-secretase cleaves β-APP (an integral membrane protein) and releases sAPP (secretory form)
  2. sAPP activates receptor R which increases cGMP
  3. cGMP activates PKG
  4. PKG activates NF-κB (survival factor)
  5. PKG also promotes K+ efflux (hyperpolarization) a protective measure
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11
Q

Describe the way β-APP is cleaved incorrectly? (secretase pathway)

A
  1. β-secretase and γ-secretase cleave β-APP which releases β-amyloid (Aβ)
  2. Aβ aggregates into insoluble plaques that induce membrane lipid peroxidation (MLP)
  3. MLP impairs Na+, Ca<strong>2</strong>+ and glucose transport resulting in apoptosis
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12
Q

Describe the defect when there is a Presenilin-1 (PS-1) mutation.

A
  1. Presenilin-1 (PS-1) is an ER membrane protein
  2. Mutations in PS-1 increases Ca2+ release via ryanodine receptors (RYRs) and IP3 receptors
  3. Enhanced Ca2+ release triggers further Ca2+ influx
  4. Altered Ca2+ homeostasis leads to apoptosis and excitotoxicity
  5. Increased Ca2+ also alters APP processing to increase Aβ production

Increases risk of AD!

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13
Q

Mutations in what genes/chromosomes are associated with increased risk of familial early-onset AD?

A
  • 21 (β-APP) - Down’s syndrome patients almost always develop AD
  • 14 (PS-1)
  • 1 (PS-2)

PS mutations responsible for most familial early onset cases

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14
Q

Mutations in what genes/chromosomes are associated with increased risk of sporadic onset AD?

A
  • ApoE4 (ε4) - Chromosome 19 ApoE is a protein modulator of phospholipid transport and may have a role in synaptic remodeling
  • IL-A & IL-B - Chromosome 2
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15
Q

What gene/chromosome is slightly protective of AD?

A

ApoE2 (ε2) - Chromosome 19

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16
Q

Describe neurofibrillary tangles.

A
  • Intracellular (cytoplasmic) inclusions
  • Composed of tau protein (hyperphosphorylated) and microtubule associated protein (MAP)
  • Paired helical filaments (PHF) are composted of tau
  • Found in hippocampus and medial temporal lobe, parieto-temporal regtions, and the frontal association cortices leading to cell death
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17
Q

What is shown in this image?

A
  • Neurofibrillay tangles
  • Seen in Alzheimer‘s disease (AD) Also seen in
  • Progressive Supranuclear Palsy (PSP) in the brainstem
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18
Q

What is the result of hyperphosphorylated tau protein in AD?

A
  • Tau is a microtubule associated protein that causes microtubules to fall apart and create aggregates
  • Aggregates form neurofibrillary tangles
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19
Q

What stimulates the hyperphosphorylation of tau protein?

A

Aβ fibrils or aggregates

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20
Q

Describe what accompanies neurofibrillary tangles and plaques.

A
  • Neuronal death and synapse loss occurs near regions of neurofibrillary tangles and plaques
  • Results in granulovacuolar degeneration - Cytoplasmic clearing with granular deposits
  • Also effects cholinergic neurons in BNM leading to decrease ACh
  • Also results in loss of serotonergic neurons in median raphe and adrenergic neurons in locus ceruleus leading to deficits in 5-HT and NE
21
Q

(T/F): Inflammation may play a role in developing Alzheimer’s.

A

True. Inflammation may play a causative role

People who take NSAIDs are less likely to develop AD than people that don’t

22
Q

What drugs are used to treat Alzheimer’s?

A
  • Anticholinesterases (increase ACh at synapse)
    • Donepezil
    • Rivastigmine
    • Galantamine
    • Tacrine
  • NMDA receptor antagonist (Glu antagonist)
    • Memantine
  • New ideas: Antibody or vaccination against Aβ or γ-secretase inhibitors
23
Q

What is vascular dementia?

A
  • Progressive loss of cognitive function after strokes or other vascular diseases
  • Abrupt onset
  • Stepwise decline
  • Physical complaints
  • Emotional incontinence (sudden bursts of tears or laughter)
24
Q

What conditions increase the patients risk for Vascular dementia?

A
  • Hypertension
  • History of stroke
  • Focal neurologic signs and symptoms
25
Q

What are the symptoms of Parkinson’s disease?

A
  • Tremor (at rest)
  • Rigidity (cogwheel)
  • Akinesia (or bradykinesia)
  • Postural instability (late)
  • Shuffling gait

Dementia develops in about 40% of Parkinson’s patients

26
Q

What neuron changes are associated with Parkinson’s?

A
  • Loss of dopaminergic neurons in substantia nigra (pars compacta)
  • Results in decrease Dopamine
27
Q

What is seen histologically in Parkinson’s patients?

A

Lewy bodies - Cytoplasmic inclusions composed of α-synuclein

28
Q

What is Dementia with Lewy Bodies (DLB)?

A
  • Essentially Parkinson’s patients that have cortical LB as well as LB in the substantia nigra
  • Fluctuating levels of alertness or cognitive function
  • Frequent visual hallucinations (commonly of little people)
  • Other signs of dementia and/or PD
29
Q

What is the Braak Hypothesis?

A
  • Explains early constipation associated with REM behavior disorders prior to Parkinson’s and progression to dementia
  • α-synuclein deposits first occur in enteric nervous system and olfactory bulb in asymptotic patients
  • Then spread rostrally into medulla and up the brainstem (stereotypic temporal pattern) progressing through substania nigra in midbrain and eventually to amygdala and cerebral cortex
  • Disease starts in periphery and gains access to CNS through retrograde transport along projection neurons from GI tract
30
Q

What is frontotemporal dementia (FTD)?

A
  • Atrophy involving the frontal and temporal lobes
  • Disordered personality and social conduct
  • Perception, spatial skills, skilled motor tasks and memory are relatively well preserved
31
Q

What are the signs and symptoms of frontotemporal dementia (FTD)?

A
  • Decline in social interpersonal conduct, inappropriate behaviors, disinhibition, abnormal joking, inappropriate sexual comments or behaviors
  • Emotional blunting and loss of insight
  • Decline inperonsal hygeine and grooming
  • Mental rigidity and inflexibility
  • Distractibility and impresistence
  • Hyperorality
  • Perservative and stereotyped behavior
32
Q

What is seen histologically with Pick‘s Disease?

A
  • Swollen (ballooned) neurons (Pick cells)
  • Argentophilic neuronal inclusions (Pick bodies) composed of tau proteins
33
Q

What is the most common frontotemporal dementia (FTD)?

A

Pick’s Disease

34
Q

What is shown in this image?

A

Pick bodies (tau protein inclusions)

35
Q

What is shown in this image?

A

Pick cells (pale, swollen neurons)

36
Q

Familial form of Picks disease is linked to what chromosome?

A

17q21-22 (associated with tau protein)

37
Q

What is the primary presentation of Pick’s Disease?

A

Loss of language is main symptom (known as Primary Progressive Aphasia)

38
Q

What is the cause of Huntington’s Disease (HD)?

A

Increased CAG repeats in huntingtin gene of chromosome 4

39
Q

What is the result of Huntington’s Disease (HD)?

A
  • Atrophy of the caudate nucleus and putamen (due to loss of cholinergic and GABAergic neurons)
  • Inclusions of huntingtin protein aggregates seen in cytoplasm or nucleus
40
Q

What is the juvenile form of Huntington’s Disease (HD)?

A

Westphal Varient

41
Q

What are the symptoms of Huntington’s Disease (HD)?

A
  • Choreiform movements
  • Aggression
  • Depression
  • Dementia
42
Q

What are the symptoms of Creutzfelt-Jacob Disease (CJD)?

A
  • Rapidly progressive dementia, universally fatal
  • Memory loss, personality changes, hallucinations, speech impairment (mutism)
  • Myoclonus (sudden jerks), balance coordination and walking problems (ataxia), rigid posture, seizures
  • Infectious partical is a protein called a prion
43
Q

What is seen histologically in CJD?

A
  • Transmissible spongiform encephalopathy
  • “Spongy” due to holes left by dead/dying neurons
44
Q

What is shown in this image and what disease is it characteristic of?

A

Spongiform Enecephalopathy Seen in CJD

45
Q

What is Kuru?

A
  • A degenerative dementia in New Guinea due to ritual cannibalism of deceased elders
  • Prion disease
46
Q

What disease in animals are prion diseases similar to?

A

Similar to Scrapie disesae in cows and sheep

47
Q

How is CJD transported in humans?

A
  • HGH (human growth hormone)
  • Pituitary extracts
  • Transplanted tissues
48
Q

What is Gerstmann-Straussler-Scheinker (GSS) disease?

A

A similar disease to CJD but with hereditary transmission

49
Q

What is the changes in the protein that causes Prion disease?

A

PrPc (alpha helix) is misfolded to PrPsc (beta sheet) in post-translational modification

BB Test 4 (14 decks)

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