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Neurology > Neurodegenerative Diseases Pt. 1 > Flashcards

Neurodegenerative Diseases Pt. 1 Flashcards

1
Q

What are the 3 poles of the cerebral cortex?

A
  • Frontal
  • Temporal
  • Occipital
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2
Q

What are the 6 layers of the neocortex?

A
  • Molecular layer
  • External granular layer
  • External pyramidal layer
  • Internal granular layer
  • Internal pyramidal layer
  • Multiform layer
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3
Q

What are the primary cortices?

A
  • Somatosensory
  • Motor
  • Visual
  • Auditory
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4
Q

What is the difference between unimodal and heteromodal association cortices?

A
  • Unimodal: concerned with integration of function from a single area
  • Heteromodal: higher order information processing: integration of function from multiple sensory and/or motor modalities
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5
Q

What is Papez circuit?

A

Limbic structures: Cingulate to hippocampus to fornix to mammillary bodies to thalamus

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6
Q

What are the Outer Core-Cortical Components of the brain? (what functions do they serve?)

A
  • Cingulate cortex (emotional and motor; visual spatial and memory)
  • Orbital frontal lobe (personality, behavior)
  • Temporal lobe (memory)
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7
Q

What are the inner core subcortical components? (what function do they serve)

A
  • Hypothalamus (pleasure center, autonomic, endocrine integration)
  • Amygdala (preservation of self behaviors)
  • Septum (preservation of species behaviors - sex)
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8
Q

Lesions in hippocampus, dorsal medial nucleus of the thalamus, mammillary nuclei alone or in combination can lead to _______ states

A

Amnestic

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9
Q

What are the two parts of declarative memory (hippocampus)?

A
  • Episodic - personal events in one’s life, actively remembered
  • Semantic - facts, known rather than actively remembered
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10
Q

What are the two forms of longterm memory?

A

Explicit (declarative)

Implicit (nondeclarative)

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11
Q

What are the functions of the following brain regions?

  • Frontal cortex:
  • Hypothalamus:
A
  • Frontal cortex:
    • Highest cognitive functions; emotional control
  • Hypothalamus:
    • Primitive emotional responses
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12
Q

What two different types of output neurons (dopamine receptors) in the striatum are affected by dopamine?

A
  • Neurons with D1 dopamine receptors (excite direct pathway and facilitate movement)
  • Neurons with D2 dopamine receptors (inhibit indirect pathway → disinhibition; facilitate movement)
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13
Q

What is the difference between the direct and indirect nigrostriatal pathway in terms of thalamus function?

A
  • Indirect pathway: thalamus is inhibited
  • Direct pathway: Thalamus is disinhibited (excited)
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14
Q

Fill in the blanks

A
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15
Q

What are common features of Neurodegenerative diseases?

A
  • Selective vulnerability of specific neurons and systems
  • Misfolded and/or aggregated proteins
  • Sporadic and familial forms
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16
Q

What are characteristics of gray matter diseases? (Selective vulnerability)

A
  • Progressive loss of neurons
  • Groups of neurons and associated fiber tracts
  • Functionally related and relatively symmetric
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17
Q

What are common cellular hallmarks in many degenerative diseases? (Misfolded or aggregated proteins)

A
  • Resistance to normal degradation processes (ubiquitin proteosome system)
  • Often form inclusions
  • Cytotoxic to neuron
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18
Q

What are possible etiologies of neurodegenerative diseases?

A
  • Genetic mutations
  • Genetic polymorphisms
  • Aging
  • Environmental toxins
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19
Q

What are the cellular mechanisms of neurodegenerative diseases?

A
  • Oxidative stress (ROS)
  • Inflammation
  • Disruption of axonal transport and synaptic function
  • Dysfunctional waste clearance
  • Mitochondrial dysfunction
  • Programmed cell death (apoptosis)
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20
Q

What are the three main causes of neurodegeneration?

A

Environmental toxins

Neuronal metabolism

Aging

21
Q

How can ROS, oxidative stress, and exitotoxicity lead to cell death?

A
  • DNA damage
  • Lipid peroxidation
  • Protein damage
22
Q

How does cell membrane damage arise from dysfunctional mitochondria?

A
  • Toxins and aging lead to loss of mitochondrial function
  • Inefficient mitochondrial electron transport “leaks out” electrons; oxygen radicals
  • Complex 1 is vulnerable to injury in response to free radicals
  • Free radicals → lipid peroxidation → loss of membrane integrity
23
Q

What reactive molecules cause oxidative stress?

A

Hydrogen peroxide and superoxide

24
Q

How does superoxide perpetuate excitotoxicity (damage of neurons)?

A

Superoxide → **Persistent activation of NMDA receptors **→Excess intracellular calcium → ATP depletion (so cells can’t get rid of calcium) → Cell death → excess glutamate → Persistent activation of NMDA receptors

25
Q

What mechanisms does the cell have to deal with oxyradicals?

A
  • Ascorbate
  • Glutathione
  • Superoxide dismutase
  • Catalase (inactivated hydrogen peroxide)
26
Q

What is the most common form of dementia?

A

Alzheimer’s Disease

27
Q

What is the clinical definition of Alzheimer’s Disease?

A

Gradual and progressive decline in cognitive funciton with impairments in recent memory and one additional cognitive domain that is not due to other medical or psychiatric illness, and results in a functional impairment socially or occupationally

28
Q

What Cognitive Domains can be affected in Alzheimer’s disease?

A
  • Memory
  • Language
  • Abstract thinking and judgment
  • Visuo-spatial or perceptual skills
  • Praxis
  • Executive function
29
Q

What are the diagnostic criteria for:

  • Definite DAT (dementia of Alzheimer type):
  • Probable DAT:
A
  • Definite DAT (dementia of Alzheimer type):
    • Criteria for Probable DA and Histopathalogic evidence
  • Probable DAT:
    • Dementia
    • Two areas of cognitive impairment
    • Progression over time
    • Normal sensorium
    • Age of onset between 40-90 years
    • No other disease causing the dementia
30
Q

What are the diagnostic criteria for Possible DAT?

A
  • Atypical onset, presentation, or progression without known etiology
  • Systemic or other brain disease capable of producing dementia
  • Gradually progressive decline in a single intellectual function in the absence of any other cause
31
Q

What is seen in Stage 1 of DAT? What is the duration

A

1-3 years

  • Memory (new learning defective)
  • Visuospatial skills (topographic disorientation)
  • Language (poor wordlist generation, anomia – inability to name 17 things in a category)
  • Psychiatric features (depression/apathy and delusions)
32
Q

What is seen in Stage 2 of DAT? What is the duration?

A

2-10 years

  • Memory (recent and remote recall more severely impaired)
  • Visuospatial skills (poor construction; spatial disorientation)
  • Calculation (acalculia)
  • Psychiatric features (delusions)
33
Q

What is seen in Stage 3 of DAT? What is the duration?

A

8-12 years

  • Intellectual funcitons severely impaired
  • Sphincter control - urinary and fecal incontinence
  • Motor - limb rigidity and flexion posture
34
Q

What are the two types of senile plaques associated with Alzheimer’s disease?

A
  • Diffuse plaque (senile plaques) – extracellular accumulation of β-amyloid protein
  • Neuritic plaque – extracellular accumulation of Aβ protein and tau containing neurites (frontal cortex → temporal cortex → neocortex)
35
Q

What gross changes can be seen in the brain with Alzheimer’s disease?

A
  • Atrophy of gyri and widening of sulci
  • Increased size of lateral ventricles
36
Q

Which type of senile plaque is more closely associated with cognitive decline?

A

Neuritic plaque

37
Q

How do you stain a cerebral amyloid angiopathy (almost always found in AD)?

A

Congo red stain

38
Q

What are Neurofibrillary tangles (NFT)? Are they unique to AD?

A
  • NFT: Intraneuronal accumulation of an abnormally phosphorylated form of tau (normal microtubule associated protein)
  • Not unique to AD – also found in degenerative diseases
39
Q

What is the biggest risk factor for AD?

What are the current factors used in the pathological diagnosis of AD?

A
  • What is the biggest risk factor for AD?
    • Age
  • What are the current factors used in the pathological diagnosis of AD?
    • Density fo neuritic plaques
    • Staging scheme for neurofibrillary tangles
40
Q

What are the three patterns of inheritance for Alzheimer’s Disease? What is the prevalence of each?

A
  • Sporadic (75%)
  • History of affected relatives (20-25%)
  • Prominent family history – usually autosomal dominant (1-5%)
41
Q

What are the differences between early onset and late onset AD?

A
  • Early onset:
    • <60-65 years old
    • Often autosomal dominant mutation - highly penetrant
  • Late onset
    • >60-65 years old
    • No Mendelian pattern of inheritance
42
Q

What is Amyloid precursor protein?

How is it related to Down Syndrome?

A
  • Amyloid precursor protein (APP) – transmembrane glycoprotein (on chromosome 21)
    • Normal functions not fully understood
    • Amyloid plaques (senile plaque) derived from APP
  • Older individuals with Down’s syndrome develop AP in late 30s
43
Q

What are the two ways in which Amyloid Precursor Protein is processed?

A

APP processing: two pathways

  • α secretase cleaves Aβ sequence; no Aβ produced
  • Cleavage at β and γ sites of APP: Aβ produced, β secretase enzyme identified, γ secretase enzyme unknown
44
Q

What genetics are associated with AD?

A
  • Presenilin 1 (PSEN1 gene) on chromosome 14 (complete penetrance)
    • Most common genetic mutation at this time (50% of familial AD)
  • Amyloid precursor protein (APP gene) on chromosome 14 – rare
  • Presenilin 2 (PSEN2 gene) on chromosome 1 (very rare)
45
Q

How do Apolipoprotein E and the APOE gene contribute to AD? What is their normal function?

A
  • Established genetic risk factor for late-onset AD
  • Three alleles at gene locus on chromosome 19: ε2, ε3, ε4
    • Code three protein isoforms, E2, E3, and E4
    • Apo E protein involved in cholesterol transport, metabolism, and storage
46
Q

How does the ε4 allele of the APOE gene modify genetic risk?

A
  • Dose-dependent: people with one ε4 allele have approx. 3x increased risk of AD, people with two ε4 alleles have approx. 15x increased risk of AD
  • Association robust but not specific
  • Presence of ε4 necessary but not sufficient
47
Q

What types of dementia are associated with Cholinergic signaling deficiency?

A
  • Alzheimer’s disease
  • Dementia with Lewy bodies
  • Vascular Dementia
48
Q

What are the four approved centrally acting cholinesterase inhibitors for treating Dementia?

A
  • Tacrine (not used much - worst side effects)
  • Donepezil
  • Rivastigmine
  • Galantamine
49
Q

What is an NMDA channel blocker with some efficacy in slowing AD disease progression?

A

Memantine

Neurology (26 decks)

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