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Pathoma > 17.7 Dementia and Degenerative Disorders > Flashcards

17.7 Dementia and Degenerative Disorders Flashcards

1
Q

Degeneration in what area of the brain will lead to movement problems?

A

Basal Ganglia - sometimes in the brainstem

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2
Q

What does degeneration of the cortex cause?

A

Dementia

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3
Q

Alzheimer’s Disease

A

Degenerative disease of the cortex

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4
Q

What is the most common cause of dementia?

A

Alzheimer’s Disease

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5
Q

The deposition of ______ leads to Alzheimer’s disease?

A

Aß-amyloid - cleaved form of amyloid precursor protein

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6
Q

What are the clinical features of Alzheimer’s disease?

A
  • Slow memory loss
  • Progressive disorientation
  • Loss of learned motor skills
  • Personality changes
  • NO early focal neurologic deficits
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7
Q

Most cases of Alzheimer’s are ______

A

Sporadic

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8
Q

What is a genetic risk factor of Alzheimer’s?

A

Epsilon4-ApoE

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9
Q

What is a genetic decreased risk factor of Alzheimer’s?

A

Epsilon2-ApoE

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10
Q

What gene mutation is early onset Alzheimer’s associated with?

A

Presenlin-1 and 2

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11
Q

What is Alzheimer’s associated with?

A

Down Syndrome - amyloid precursor is found on Ch21 so there is increased Aß-amyloid

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12
Q

What will be seen on the brain of an Alzheimer’s patient?

A

Diffuse cortical atrophy - with narrowing of the gyri and widening of the sulci

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13
Q

Hydrocephalus Ex Vacuo

A

Consequence of brain atrophy where the ventricles of the CNS dilate

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14
Q

Cerebral Amyloid Angiopathy

A

Deposition of Aß-amyloid into the blood vessels of the brain

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15
Q

What protein is present in neurofibrillary tangles?

A

Tau Protein that is hyperphosphorylated

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16
Q

Vascular Dementia

A

Multifocal infarction and injury that can be due to HTN, atherosclerosis or vasculitis that decreases blood flow to brain

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17
Q

What is the second most common cause of dementia?

A

Vascular Dementia

18
Q

Pick Disease

A

Degenerative disease of the frontal and temporal lobe that spares the parietal and occipital lobes - leads to behavioral and language symptoms

19
Q

What is found in Pick disease?

A

Round aggregates of tau protein in neurons of the cortex

20
Q

Parkinson Disease

A

Degenerative loss of dopaminergic neurons in the substantia nigra (pars compacta)

21
Q

What is the effect of dopamine from the substantia nigra - pars compacta on the striatum?

A
  • Increases stimulation with D1 receptor binding
  • Decreases inhibition with D2 receptor binding

Overall increases cortical function and increases movement

22
Q

Exposure to what chemical has been known to lead to Parkinson’s?

A

MPTP

23
Q

What are the clinical features of Parkinson’s?

A

Tremor
Rigidity
Akinesia
Postural Instability

24
Q

What are the round eosinophilic bodies seen in Parkinson’s?

A

Lewy Bodies

25
Q

What are Lewy Bodies composed of?

A

Alpha synuclein

26
Q

What does early dementia in Parkinson’s suggest?

A

Lewi Body Dementia - Lewy bodies in the cortex

27
Q

Huntington’s Disease

A

Degeneration of the GABAergic neurons in the caudate nucleus of the basal ganglia

28
Q

What is the function of the caudate?

A

The GABA neurons of the caudate send inhibitory signals to the cortex.

29
Q

What is a main feature of Huntington’s?

A

Chorea

30
Q

What is the inheritance pattern of Huntington’s?

A

Autosomal Dominant

31
Q

What is the mutation seen in Huntington’s?

A

CAG repeats in the huntingtin gene

32
Q

What is a common cause of death with Huntington’s?

A

Suicide

33
Q

Normal Pressure Hydrocephalus

A

Increased CSF results in dilated ventricles and can cause dementia

34
Q

What are the clinical features of normal pressure hydrocephalus?

A
  • Urinary incontinence
  • Gait instability
  • Dementia
35
Q

What is the treatment for normal pressure hydrocephalus?

A

Ventricle-Peritoneum Shunt

36
Q

Spongiform Encephalopathy

A

Degenerative disease due to a prion protein

37
Q

What is the pathologic protein in spongiform encephalopathy?

A

Prp c in a beta-pleated sheet configuration that is non-degradable

38
Q

What is the hallmark of spongiform encephalopathy?

A

Intracellular vacuoles in neurons and glial cells

39
Q

What are the 3 mechanisms for spongiform encephalopathy?

A
  • Sporadic
  • Inherited
  • Transmitted
40
Q

Creutzfeldt–Jakob Disease (CJD)

A

Usually sporadic that can rarely arise form exposure to prion infected human tissue and presents as rapidly progressive dementia associated with ataxia and startle myoclonus - fatal within 1 year

41
Q

vCJD

A

Related to exposure to bovine spongiform encephalopathy

42
Q

Familial Fatal Insomnia

A

Inherited prion disease characterized by insomnia and an exaggerated startle response

Pathoma (119 decks)

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