Neurodegenerative Diseases Flashcards

1
Q

General overview/underlying cause of neurodegenerative disorders

A
  • often due to spontaneous failure w/in body
  • includes spontaneous death of a neuronal population
    • location of population ==> clinical presentation
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2
Q

Typical categories of classification for neurodegenerative diseases

A
  • clinical presentation
  • transmission
  • neurochemistry
  • neuropathology
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3
Q

Common dementia-related problems in neurodegenerative diseases

A
  • memory
  • language
  • executive function
  • visuospatial
  • depression, apathy, sociopathy
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4
Q

Common movement disorder problems in neurodegenerative diseases

A
  • bradykinesia
  • rigidity
  • tremor
  • chorea = involuntary movement disorder
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5
Q

Common motor/muscular-related problems in neurodegenerative diseases

A
  • muscle weakness
  • muscle atrophy
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6
Q

Dementia could indicate…

A
  • Alzheimer’s disease
  • FTD
  • Lewy body
  • HD
  • CJD
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7
Q

Motor problems could indicate…

A
  • ALS
  • ALS/FTD (frontotemporal dementia)
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8
Q

Movement disorders could indicate…

A
  • Parkinson’s
  • PSP (progressive supranuclear palsy)
  • CJD
  • Huntingtons
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9
Q

Alzheimer’s disease: Clinical Features & Transmission

A
  • clinical = early memory and visuospatial problems
  • transmission
    • most = sporadic
    • genetic
      • presenilin 1 mutation
      • trisomy 21
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10
Q

Frontotemporal dementia: Clinical Features & Transmission

A
  • early behavioral, executive, and/or language problems
  • transmitted both genetically and sporadically
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11
Q

Parkinson’s disease: Clinical Features & Transmission

A
  • tremor, rigidity, bradykinesia
    • leads to slow speech and movement
  • genetic and sporadic transmission
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12
Q

Lewy Body Dementia: Clinical Features & Transmission

A
  • early parkinsonian features, psychosis, fluctuating consciousness
  • genetic and sporadic transmission
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13
Q

Progressive supranuclear palsy: Clinical Features & Transmission

A
  • bradykinesia, rigidity, falls
  • abnormal vertical eye movements
  • genetic and sporadic transmission
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14
Q

ALS: Clinical Features & Transmission

A
  • weakness and atrophy
  • fasciculations
  • UMN & LMN signs
  • genetic and sporadic transmission
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15
Q

CJD: Clinical Features & Transmission

A
  • rapidly progressing dementia
  • myoclonus (jerky muscle contraction) developing over months
  • (spontaneous) onset usually 55-65yo
  • genetic, spontaneous, and infectious transmission
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16
Q

Huntington’s disease: Clinical Features & Transmission

A
  • dementia
  • depression, sociopathy (aggression)
  • chorea
  • inherited: autosomal dominant
17
Q

Kuru: Clinical Features & Transmission

A
  • early tremor and ataxia
  • caused by canabalism
18
Q

Gertsmann Straussler Scheinker Syndrome (GSS): Clinical Features & Transmission

A
  • clumsiness, incoordination, ataxia
  • NO myoclonus
  • varied presentations
  • genetic only: autosomal dominant
19
Q

Fatal Familial Insomnia disease: Clinical Features & Transmission

A
  • progressive insomnia
  • memory loss, confusion
  • hallucinations
  • genetic: autosomal dominant
20
Q

Alzheimer’s disease: Neurochemistry & Neuropathology

A
  • ACh deficit (cholinergic hypothesis)
  • Amyloid plaques and neurofibrillary tangles
  • diffuse atrophy globally
  • cortex and hippocampus involved
21
Q

Frontotemporal dementia: Neurochemistry & Neuropathology

A
  • tau proteins vs. seratonin deficit
  • frontal and temporal lobe most affected
  • neurofibrillary tangles
  • ubiquitin inclusions
  • pick bodies
  • no distinct histology
22
Q

Parkinson’s disease: Neurochemistry & Neuropathology

A
  • Chemistry
    • Synuclein
    • Dopamine deficit
  • Pathology
    • Lewy bodies
23
Q

Lewy Body Dementia: Neurochemistry & Neuropathology

A
  • ACh deficit
  • Dopamine deficit
  • Lewy bodies
24
Q

Progressive supranuclear palsy: Neurochemistry & Neuropathology

A
  • tau proteins
25
Q

ALS: Neurochemistry & Neuropathology

A
  • ubiquitin
26
Q

CJD: Neurochemistry & Neuropathology

A
  • prion protein
27
Q

Huntington’s disease: Neurochemistry & Neuropathology

A
  • polyglutamine
  • characteristic gross changes w/out microscopic changes
  • caudate nucleus atrophy
  • ventricles ==> butterflies
28
Q

Kuru disease: Neurochemistry

A
  • prion protein
29
Q

GSS: Neuropathology

A
  • cerebellar problems
30
Q

Fatal familial Insomnia: Neuropathology

A
  • thalamus problems
31
Q

Characteristics of prion diseases

A
  • can be sporadic, heritable, or transmissible/infectious
  • caused by proteinaceious infectious particle w/out nucleic acid
    • natural protein ==> deforms to beta pleated sheet
  • uniformly fatal diseases