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USMLE Neurology > Neurodegenerative disorders > Flashcards

Neurodegenerative disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What contaminent in illegal drugs can cause Parkinson’s -> toxicity of substantia nigra?

A

MPTP which is metabolised to MPP+

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2
Q

What will be seen on histology / grossly in the brain in Parkinsons disease?

A
  • Depigmentation of SN (loss of dopamenergic neurons)
  • Lewy bodies: composed of alpha-synuclein (intracellular eosinophilic inclusions)
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3
Q

What is the trinucletide repeat in Huntingtons disease?

A

CAG repeated in the Huntingtin (HTT) gene

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4
Q

What chromsome is affected in Huntingtons?

A

Chromosome 4

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5
Q

What are the symptoms of Huntington disease?

A
  • Chorea
  • Athetosis
  • Aggression
  • Depression
  • Dementia
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6
Q

What do neurons die via in Huntingtons?

A

NMDA-R binding and glutamate excitotoxicity

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7
Q

What atrophies in Huntingtons?

A

Caudate and putamen

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8
Q

What NTs are affected in Huntingtons?

A
  • Increased dopamine
  • Decreased GABA
  • Decreased Ach
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9
Q

What structures are atrophied in Huntingtons?

A

Caudate and putamen (dorsal striatum)

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10
Q

Why are those with Downs syndrome at increased risk of Alzheimers?

A

APP is located on chr 21.
- APP synthesises Amyloid-Beta -> ALzheimers

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11
Q

What NT is decreased in Alzheimers?

A

Ach

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12
Q

What proteins are linked to increased risk of Alzheimers?

A
  • ApoE-2 (decr risk of sporadic) and ApoE4 (decr risk of sporadic)
  • APP, Presenilin-1, Presenilin-2
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13
Q

What proteins are linked to familial early onset Alzheimers?

A
  • APP
  • Presenilin-1
  • Presenilin-2
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14
Q

What are the gross findings on the brain in Alzheimers?

A
  • Widespread cortical atrophy
  • Hippocampus atrophy
  • Narrowing of gyri and widening of sulci
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15
Q

What are the histological findings in Alzheimers?

A
  • Senile plaques in gray mater: B amyloid core; may cause amyloid angiopathy
  • Neurofibrillary tangles: intracellular, hyperphosphorylated tau protein = insoluble cytoskeletal elements
  • Hirano bodies - intracellular eosinophilic proteinaceous rods in hippocampus
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16
Q

What protein are Amyloid-B synthesised by?

A

APP

17
Q

How can Alzheimers cause intracranial hemorrhage?

A

Beta amyloid may cause amyloid angiopathy -> intracranial hemorrhage

18
Q

What are neurofibrillary tangles, where are they found/

A

Intracellular, hyperphosphorylated tau protein - insoluble cytoskeletal elements

19
Q

What will frontotemporal dementia present with?

A

Changes in personality/behaviour and aphasia

20
Q

What will be seen on histology in Frontotemporal dementia?

A
  • Inclusions of hyperphosphorylated tau (round Pick bodies)
  • Ubiquinated TDP-43
21
Q

What symptom can differentiate Lewy body dementia from others?

A

Visual halucinations

22
Q

Where are Lewy bodies found?

A

Intracellular in cortex

23
Q

What cvan be seen on imaging (MRI/CT) in vascular dementia?

A

Multiple cortical and/or subcortical infarcts

24
Q

What is 2nd most common cause of dementia in the elderly?

A

Vascular

25
Q

What are the major symptoms of Creutzfeld Jakob disease?

A

Rapidly progressive dementia with myoclonus and ataxia

26
Q

What will be seen on EEG in CJD?

A

Periodic sharp waves

27
Q

What will be found in CSF in CJD?

A

14-3-3 protein

28
Q

What will be seen in the cortex grossly in CJD?

A

Spongiform cortex (vacuuolation w/o inflammation)

29
Q

What happens to the prions in CJD?

A

the normal prion is transformed to a Beta-peated sheet resistant to proteases (PrPc -> PrPsc)

30
Q

HIV-associated dementia will lcause what grossly on the brain?

A

Diffuse gray matter and subcortical atrophy

31
Q

What will be seen histologically in HIV-associated dementia?

A

Microglial nodules with multinucleated giant cells

32
Q

What are the symptoms of HIV-associated dementia?

A
  • Cognitive deficits
  • Gait disturbances
  • Irratibility
  • Depressed mood

USMLE Neurology (19 decks)

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