CNS degeneration Flashcards

1
Q

Really rapid neurodegeneration

Spongiform vacuolation

A

Suspect Prion disease

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2
Q

Cortical atrophy - widening sulci, gyra shrinkage, ventricular enlargement
Hippocampus affected
Beta-amyloid plaques +Tau tangles
Most common neurodegenerative disease

A

Alzheimer’s disease

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3
Q
Beta-amyloid plaques
Tau tangles (Tau hyper-phosphorylation)
A

Alzheimer’s disease

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4
Q

Pattern of Tau tangle formation

A

Braek staging

Hippocampus (I-III) > temporal (IV) > occipital (peri-striated cortex (V) then striate cortex (VI) ‘HTO’

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5
Q

Braek staging

A

AD staging - based on location of Tau tangles using Tau immunostaining
I-VI

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6
Q

Braek stages I-III

A

Hippocampus only

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7
Q

Braek stage V

A

Occipital lobe (peri-striatal)

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8
Q

Braek stage VI

A

Occipital lobe (striatal)

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9
Q

Braek stage at which symptoms start to appear

A

III or IV

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10
Q

Tau immunostaining in peristriate cortex but not striate cortex is consistent with which Braak grading?

A

Braek V

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11
Q

Contact sports

Tauopathy

A

Chronic traumatic encephalopathy

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12
Q

Alpha-synuclein ‘smooth hyaline inclusions’ in substantia nigra (SN) + cortex
Cortex = cognitive changes
SN = dopaminergic cell death = Parkinson’s symptoms

A

Lewy Body dementia

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13
Q

Function of dopaminergic neurons

A

Project from SN to basal ganglia

Death = Parkinson’s triad (bradykinesia, pill-rolling tremor, rigidity)

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14
Q

Discolouration of SN

A

Dopaminergic cell death

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15
Q

Pattern of Lewy Body formation

A

Also Braek staging:
Medulla (I) > Pons (II) > SN (III) > Basal forebrain (IV) > Cortex (V)
‘Many People See Blue Cats’

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16
Q

What is interesting about the spread of Lewy Body formation?

A

Initial symptoms of LBD are due to SN pathology (Parkinsonism) but LB initially form in medulla. Sleep disorders due to LB in pons may be an initial prodrome to look out for

17
Q

When do symptoms of LBD occur?

A

60-70% SN dopaminergic death

18
Q

Alpha-synuclein (+ ubiquitin) in dopaminergic cells of cortex + SN
Fluctuating cognition, visual hallucinations, Parkinsonism, recurrent falls + syncope

A

Lewy Body Dementia

19
Q

Proteinopathies causing Parkinsonism

A

Lewy Body Dementia (LBD) - Alpha-synuclein
Multiple system atrophy (MSA) - Alpha-synuclein
Progressive Supranuclear Palsy (PSP) - Tau
Corticobasal degeneration (CBD) - Tau

20
Q

Alpha-synuclein
Glial cells
Parkinsonism

A

MSA

Multiple system atrophy

21
Q

Tau

Downward gaze, Parkinsonism

A

PSP (Progressive Supranuclear Palsy)

22
Q

Tau

Unilateral Parkinsonism, myoclonus, alien limbs

A

CBD (Cortico-basal degeneration)

Think weird cannabinoids

23
Q

Parkinsonism symptoms

A

TRAP

Tremor, rigidity, akinesia / bradykinesia, postural instability

24
Q

Tau in fronto-temporal lobes

Loss of executive function - disinhibition, cognition ‘pick up girls’

A

Pick’s disease (type of frontotemporal dementia)

25
Q

Tauopathy classification

A

Pick’s disease (3R)
CBD, PSP (4R)
Alzheimer’s disease (3R and 4R)

26
Q

Tau gene

A

Pro-granulin (chromosome 17)

27
Q

Cat’s eye inclusions

A

Mutations in pro-granulin

28
Q

3R + 4R Tauopathy

A

AD

29
Q

4R Tauopathy

A

CBD, PSP

30
Q

3R Tauopathy

A

Pick’s disease

31
Q

Early onset AD

A

Down’s Syndrome

32
Q

Reduced uptake on DAT scan

A

Parkinson’s

33
Q

Demyelinating plaques
Myelin basic protein
Proteo-lipid protein

A

MS

34
Q

4 stages of MS plaque

A
  1. Acute (oedema)
  2. Early chronic (oedema + macrophages, some myelin breakdown)
  3. Late chronic active (complete myelin loss, macrophages still present)
  4. (complete myelin loss, no macrophages)