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Pathology > Neurodegenerative Disorders > Flashcards

Neurodegenerative Disorders Flashcards

1
Q

Neurodegenerative Disease

A

disorders w/ progressive loss of neurons which have shared function

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2
Q

Etiopathogenesis of Neurodegenerative disease

A
  • Not understood
  • MOST HAVE ACCUMULATION OF PROTEIN AGGREGATES
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3
Q

CFs of Alzheimer

A
  • Memory impairment (Short-term –> long-term)
  • Altered mood & behavior
  • Impaired cognition
  • Aphasia & disorientation

NB: motor & sensory system usually intact

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4
Q

Diagnosis & Cause of death in Alzheimer

A
  • MSE
  • Histo (autopsy)

COD= Intercurrent pneumonia or other infection

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5
Q

Etiopathogenesis of Alzheimer

A

Accumulation of AB & Tau proteins as plaques & tangles –> Neuronal death & inflammation

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6
Q

Formation of plaques in Alzheimer Disease

A

Amyloid precursor protein (APP) mutation –> AB monomer –> AB oligomers –> AB peptide aggregation –> Neuronal damage

  • Extracellular
  • Gene on Chr 21 (down syndrome association)
  • APP broken down into fragments by Secretase
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7
Q

Formation of Neurofibrillary tangles in Alzheimer

A

Aggregation of Tau proteins (microtubule-associated proteins) in axons

  • AB oligomers activates kinases –> Hyper-phosphorylate tau –> Loses ability to bind microtubules –> Aggregation
  • INTRACELLULAR then goes EXTRACELLULAR after neuronal death
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8
Q

Types o Alzheimer Disease

A

Inherited (20%)
1. Early onset
- APP on Chr 21 –> AB amyloid formation
- Presenilin gene on Chr 1 & 14 and y-secretase
2. Late onset
- Tau protein
- Apo E on Chr 19 – 3 allelic form (e2, e3 & e4 )
Homozygosity of e4 is worst
Sporadic (80%)

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9
Q

Gross morphology of brain w/ Alzheimer

A
  • Atrophy - frontal, temporal & parietal lobes
  • Cerebral sulci widening
  • Ventricular enlargement - Hydrocephalus ex vacuo
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10
Q

Microscopic changes in brain w/ Alzheimer Disease

A
  • Neuronal loss in cerebral cortex
  • Gliosis - Astrocyte proliferation & hypertrophy
  • Neurofibrillary tangles & neuritic plaques
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11
Q

Frontotemporal Lobar Degeneration (FTLD)

A

disorder affecting the frontal and/or temporal lobes

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12
Q

CFs of FTLD

A
  • Progressive behavioral changes OR
  • Language problems
  • Memory disturbance (late - differentiate from Alzheimer)
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13
Q

Epidemiology of FTLD

A

Younger ages (differentiate from Alzheimer)

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14
Q

Etiopathogenesis of FTLD

A
  • FTLD-tau (Tau accumulation - Also in Alzheimer)
  • FTLD-TDP
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15
Q

Gross morphology of FTLD

A
  • Temporal cortex atrophy

NB: Relative frontal cortex preservation

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16
Q

Microscopic changes in FTLD

A

Cytoplasmic inclusion w/ TDP43 proteins

17
Q

Parkinson Disease

A

NDD w/ hypokinetic movement due to loss of dopaminergic neurons in Substantia nigra

18
Q

Etiopathogenesis of Parkinson

A
  • Substantia Nigra - Protein accumulation, mitochondrial abnormalities & dopaminergic neuronal loss
  • Mutation in a-synuclein gene (autosomal dominant)
19
Q

CFs of Parkinson

A
  • Tremor (asymmetric pill rolling)
  • Rigidity (cogwheel)
  • Bradykinesia/ Akinesia (shuffling gait)
  • Postural instability
  • Masked facies, hypokinetic dysarthria, micrographia & sleep disorder
  • Dementia (fluctuates) & hallucination (LATE)
20
Q

Gross morphology of Parkinson

A
  • Substania nigra - Pallor & atrophy
21
Q

Microscopic changes in parkinson

A
  • Loss of pigmented neurons w/ gliosis
  • Lewy bodies (cytoplasmic eosinophilic inclusions - filaments composed of a-synuclein)
22
Q

Progression & Cause of death in Parkinson

A

progress over 10-15 year

COD = aspirational pneumonia OR trauma from falls

23
Q

Lewy body Dementia

A

When dementia & hallucinations occurs within 1 year of motor symptoms in Parkinson

24
Q

Amyotrophic Lateral Sclerosis

A

NDD due to death of LMN in the spinal cord & brain stem AND UMN in the motor cortex

25
Q

Pathogenesis of ALS

A
  • Uncertain
  • Abnormal RNA and/or Protein processing

Mutations
- Chr 9 Hexanucleotide repeat
- Superoxide Dismutase gene

26
Q

Epidemiology of ALS

A
  • M>F
  • 50 years
  • Life expectancy = 2-5 years after onset
  • Sporadic (mostly) or Familial (10%) - AD inheritance)
27
Q

CFs of ALS

A
  • Asymmetric distal extremity weakness
    Loss of LMN –> Denervation of muscles –> Atrophy
  • weakness & fasciculations
    Loss of UMN
  • Paresis, spasticity, hyperreflexia, Babinski sign & Degeneration of corticospinal tract in lateral portion of spinal cord

Cognition may be impaired.
But intellect, sensation, sphincter control & eye movement are SPARED.

28
Q

Cause of Death in ALS

A

Recurrent Pulmonary Infections

29
Q

Gross morphology of ALS

A
  • Thinning of Anterior roots of spinal cord
  • Atrophy of Precentral motor cortex
30
Q

Microscopic changes in ALS

A
  • Dec # of neurons in anterior horn in spinal cord
  • Neurogenic atrophy in skeletal muscles
  • Reactive gliosis
  • Loss of anterior root myelinated fibers
31
Q

Huntington Chorea/Disease

A

Movement disorder associated with degeneration of Striatum (Caudate & Putamen)

32
Q

CFs of Huntington

A

MOTOR
- Chorea
- Athetosis
- Dystonia
- Impaired gait, posture & balance
- Difficulty in speech & eye movements

COGNITION & PHYCHIATRY
- Forgetfulness (early)
- Difficulty organizing tasks & lack of impulse control
- Dementia
- Increase risk of suicide

33
Q

Etiopathogenesis of Huntington

A
  • CAG repeats ( 39-250 repeats)
  • Gain of function mutation of Huntingtin gene on Chr 4
    -Mutant protein + ubiquitination + proteolysis –> Intranuclear fragments –> Disrupts protein degradation & mitochondrial function & sequester transcription factors
  • ANTICIPATION- Paternal transmission results in earlier onset in next generation
34
Q

Gross morphology of Huntington

A
  • Atrophy of Caudate nucleus & Putamen
  • Dilated lateral & third ventricles
35
Q

Microscopic changes in Huntington

A
  • Neuronal loss w/ gliosis
  • Intranuclear inclusions w/ ubiquitinated Huntington protein
36
Q

Progression & Cause of death in Huntington

A
  • Progressive
  • Death after about 15 years

Pathology (7 decks)

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