Neuropathology of Dementia Flashcards

1
Q

Identify the protein involved in each disease process (tau, β-amyloid, α-synuclein, PrPp, etc).

A

alpha synuclein are known as lewy bodies, they have a eosinophilic core surrounded by pale halo, found in the substantia nigra in parkinson’s disease, and they are also seen in the cortex in dementia with lewy bodies

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2
Q

Describe the specific microscopic findings associated with each disease process (NFTs, amyloid plaques, inclusions, spongiform change, etc).

A

Neuritic plaques, Aβ peptides aggregate in tissue as β-pleated sheets (amyloid), eliciting a toxic response in astrocytes and microglia
Hyperphosphorylated tau protein is also present in AD

Picks: Neuronal loss and gliosis of outer layers of cortex, caudate nucleus, thalamus
Gliosis of subcortical white matter
Spongiform change
Pick bodies
Intracytoplasmic inclusions
Tau+
“Pick bodies” viewed with Bielschowsky silver stain

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3
Q

Identify the genes involved in familial forms of Alzheimer’s disease.

A

Early: APP gene, Presenilin-1, Presenilin-2; late: ApoE ε4 alleles (chr 19)

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4
Q

Define prions and describe the mechanism by which they induce prion diseases.

A
Abnormal form facilitates conformation change in normal PrP molecules, which accumulate in the tissue
Normal conformation (PrPc) is -helix, which is changed to -pleated sheet in disease state (PrPp)
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5
Q

Recognize the symptoms of Wernicke’s encephalopathy, and describe the etiology and treatment.

A
Clinical syndrome:
Delirium
Gait ataxia
Nystagmus
Alcoholism or other settings associated with poor nutrition
TREAT: 
Thiamine (B1) deficiency
Treat with parenteral thiamine
In thiamine-deficient patients, giving glucose without thiamine can precipitate Wernicke’s encephalopathy
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6
Q

Recognize a wide variety of disease processes that can produce dementia-like symptoms (vascular dementia, metabolic disorders, brain tumors, etc).

A

HIV
Neurosyphilis
Cryptococcal meningitis
Whipple’s disease
Prion diseases
Progressive multifocal leukoencephalopathy (PML)
Subacute sclerosing panencephalitis (SSPE)

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7
Q

A 40 year old male with Down’s syndrome has symptoms of advanced dementia consistent with Alzheimer’s disease. Why is early onset Alzheimer’s disease common in Down’s syndrome?

A

the amyloid precursor protein (APP) is located on chromosome 21

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8
Q

Patients with FTDP-17 have which of the following brain abnormalities?

A

Extensive deposition of intracellular hyperphosphorylated tau protein in neurons and glia

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9
Q

T/F: HIV encephalopathy is frequently treated with HAART therapy

A

HIV associated dementia persists DESPITE HAART therapy:
Microglial nodules and TEST: multinucleated giant cells near blood vessels
Involves white matter, diencephalon, and brainstem

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10
Q

In AD: APP amyloid precursor protein gene is on____
In familial AD there are two types: ____ and _____, the genes responsible for early onset are :
The genes responsible for late onset are:

A

chromosome 21; app gene, presenilin 1, and presenilin 2; apoE E4 alleles chromosome 19: most common form of familial AD

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11
Q

The atrophy is typically more pronounced in the ______

A

The atrophy is typically more pronounced in the medial temporal lobes

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12
Q

Neuritic plaques

A

Composed of dystrophic neurites and mitochondria

Astrocytes and microglia associated with amyloid fibrils with a central core of -amyloid

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13
Q

neuritic plaques tend to aggregate and be distributed in the _________, but you don’t see them in:

A

hippocampus, medial cortex, posterior cingulate gyrus, association cortex; not in motor cortex (hence why you don’t see a lot of sensory motor deficits in patients

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14
Q

neurofibrillary tangles:

A

hyper-phosphorylated tau proteins; difficult to break down and can persist as “ghosts” after neuron dies (and is visible with bielschowsky silver stain)

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15
Q

Pick’s Disease is characterised by:

A
Neuronal loss and gliosis of outer layers of cortex, caudate nucleus, thalamus
Gliosis of subcortical white matter
Spongiform change
Pick bodies
Intracytoplasmic inclusions
Tau+
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16
Q

FTDP-17 is characterised by:

A

Extensive deposition of intracellular hyperphosphorylated tau protein in neurons and glia

17
Q

TEST: Pick’s Disease is characterised by:

A
Neuronal loss and gliosis of outer layers of cortex, caudate nucleus, thalamus
Gliosis of subcortical white matter
Spongiform change
Pick bodies
Intracytoplasmic inclusions
Tau+
18
Q

Cortical Lewy bodies are seen in ______

A

Cortical Lewy bodies are seen in Dementia with Lewy Bodies, not Parkinson’s Disease.

19
Q

vascular dementia is characterised by:

A

2nd most common cause of dementia in the elderly

Cardiac issues, history of stroke, etc

Preventable by treating predisposing conditions:
May also be classified as:
Cortical
Subcortical 
Lacunar
20
Q

vascular dementia is characterised by:

A

2nd most common cause of dementia in the elderly

Cardiac issues, history of stroke, etc

Preventable by treating predisposing conditions:
May also be classified as:
Cortical
Subcortical 
Lacunar
21
Q

Neurosyphilis:

A

Rod-shaped microglia on silver stain (SILVER STAIN MENTIONED CONSTANTLY!)Other symptoms – myoclonus, ataxia, visual abnormalities, mutism

22
Q

CJD is characterised by:

A

myoclonus, ataxia, visual abnormalities, mutism

23
Q

Lewy Bodies are:

A

Positive for alpha-synuclein, and have a:
Eosinophilic core surrounded by pale halo
Found in Substantia nigra in Parkinson’s disease
Also seen in the cortex in Dementia with Lewy bodies

24
Q

TEST: Wernicke’s Encephalopathy: caused by ____- and presents with _____

A

Thiamine B1 deficiency, and presents with Delirium, gait ataxia, nystagmus (result of alcoholism)

25
Q

CJD is characterised by;

A

CSF: 14-3-3 protein
Rapid progression
(death in usually in less than 1 year)
myoclonus, ataxia, visual abnormalities, mutism

26
Q

with CJD, the patient would present with ________

A

abnormal EEG,

27
Q

Neurosyphilis presents histologically with:

A

rod-shaped microglia on silver stain

28
Q

HIV Encephalopathy presents on histo with:

A

microglial nodules and multinucleated giant cells near blood vessels

29
Q

AD on histo has:

A

dystrophic neurites and mitochondria; the astrocytes and microglia associated with amyloid fibrils with a central core of B-amyloid

30
Q

CJD on histo presents with;

A

spongiform change and amyloid plaques in cerebellum

31
Q

Wernicke’s encephalopathy presents with:

A

hermorrhage, neuronal loss, and gliosis in mammillary bodies and periventricular area around the third and fourth ventricles

32
Q

Wernicke’s encephalopathy clinically presents with:

A
Delirium
Gait ataxia
Nystagmus
Alcoholism or other settings associated with poor nutrition
Thiamine (B1) deficiency
33
Q

Wernicke’s encephalopathy with confabulation points to:

A

korsakoff! It is a results of wernicke’s