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NEUROLOGY > Demyelination and Dementia NeuroPathology > Flashcards

Demyelination and Dementia NeuroPathology Flashcards

1
Q

What 2 structures are damaged during the process of demyelination?

A

Myelin sheath itself

oligodendrocytes/ schwann cells which form myelin sheaths

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2
Q

What is the main Primary demyleinating disease process in adults?

A

Multiple Sclerosis (MS)

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3
Q

What Primary demyelinating diseases are more common in children?

A

Acutedisseminatedencephalomyelitis (self-limiting)

Acutehaemorrhagicleukoencephalitis (rapidly fatal)

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4
Q

What are the secondary causes of demyelination?

A
  • Viral e.g. Progressivemultifocalleukoencephalopathy(PML) •Metabolic e.g. centralpontinemyelinosis
  • Toxic agents e.g. carbon monoxide
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5
Q

Why does MS present as hyperintense lesions in the white matter on MRI?

A

Most axons are concentrated in the white matter

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6
Q

What CSF sign is indicative of MS?

A

IgGoligoclonalbandsinCSF

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7
Q

What focal neurological signs do patients with MS present with if they have optic nerve lsions?

A

opticneuritis

=> Unilateralvisualimpairment

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8
Q

What focal neurological signs do MS patients present with if they have lesions in the spinal cord?

A
  • Motororsensorydeficitintrunkandlimbs
  • Spasticity
  • Bladderdysfunction
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9
Q

What focal neurological signs are present in MS patients with brainstem lesions?

A
  • Cranialnervesigns
  • Ataxia
  • Nystagmus
  • Internuclearopthalmoplegia
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10
Q

Describe the appearance of plaques in MS

A
  • Wellcircumscribed
  • Irregularshaped
  • Glassy/translucentappearance
  • Varyin size
  • Non‐anatomicaldistribution
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11
Q

What areas of the brain are plaques normally found in?

A
  • Adjacenttolateralventricles
  • Corpuscallosum
  • Opticnerves
  • Brainstem
  • Cerebellum
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12
Q

Explain the histological findings in an acute active MS lesion

A
  • Microglia (digest myelin like macrophage)
  • Inflammatorycells around blood vessels
    Plaques grow in a perivascular distribution
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13
Q

Describe the histological findings in a chronic or inactive MS lesion

A

Gliosis (proliferation of glial cells)

Less oligodendrocytes and myelinated axons present

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14
Q

What is the difference in the macroscopic appearance of active and inactive MS lesions

A 
Active = ill defined edge
Inactive = well demarcated
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15
Q

Give examples of degenerative diseases of the cerebral cortex?

A

Alzheimer’sDisease

CJD

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16
Q

What neurodegenerative diseases originate in the basal ganglia and brainstem?

A

ParkinsonDisease
ProgressiveSupranuclearPalsy
HuntingtonDisease

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17
Q

Where else can neurodegenerative diseases present aside from the cerebral cortex, brainstem and basal ganglia?

A 
Cerebellum (Freidrich's ataxia)
Motor neurones (MND)
18
Q

Dementia is an ageing process and not pathological. TRUE/FALSE?

A

FALSE

DementiaisNOTpartofthenormalageingprocess,itisalways pathological

19
Q

Give examples of Primary Dementia

A
  • Alzheimer’sdisease

* Lewybodydementia

20
Q

Give examples of causes of secondary dementia

A

Multi‐infarct(vascular)dementia
•Infection(HIV,syphilis)
•Trauma
•Metabolic

21
Q

What symptoms can alzheimers progress to show in the later stages?

A
  • disorientation
  • worsened memoryloss
  • aphasia
  • profounddisability/immobility
22
Q

Patient’s with alzheimers usually die of the disease. TRUE/FALSE?

A

FALSE

Deathusuallyoccursduetoasecondarycause,like bronchopneumonia

23
Q

Describe the macroscopic appearance of the brain in alzheimers dementia

A

•cortical atrophy
=> in frontal,temporalandparietallobes
(Occipital, brainstemand cerebellumspared)
•Wideningofsulci / Narrowingofgyri
•Dilatationof ventricles

24
Q

What microscopic features are typical of Alzheimers disease?

A
  • neuronalloss (+astrocyteproliferation = gliosis)
  • neurofibrilliary tanngles (in hippocampus and temporal lobe)
  • neuritic plaques due to amyloid angiopathy
25
Q

Why is alzheimers disease thought to be more common in those with Down Syndrome (Trisomy 21)?

A

Genetically have more of amyloid protein precursor (on chromosome 21)
=> more at risk of amyloid angiopathy

26
Q

Describe the microscopic appearance of amyloid angiopathy

A
  • Extracellulareosinophils
  • Polymerisedbetapleated sheats
  • StainswithCongoRed
  • Disruptsbloodbrainbarrier => local oedema
27
Q

What is Lewy Body dementia?

A
  • progressivedementia
  • hallucinations
  • fluctuating levelsofattention/cognition
  • Fluctuationonaday‐to‐daybasis
  • FeaturesofParkinsonism
28
Q

What are the clinical features of Parkinson’s disease

A
  • Lossoffacialexpression
  • stooping
  • shufflinggait
  • slowinitiationof movements
  • tiffness
  • pillrollingtremor
29
Q

Where in the brain does Lewy Body dementia cause degeneration?

A

In the substantia nigra (similar to parkinson’s)

30
Q

Describe how Lewy Body Dementia appears microscopically

A
  • Lossofpigmentedneurons
  • Reactivegliosis
  • RemainingneuronsmayshowLewybodies:
31
Q

When are patient’s with huntington’s disease usually diagnosed and when does dementia develop?

A

Usually diagnosed between 35-50

dementia presents later in disease process

32
Q

How does huntington’s dementia appear macroscopically in the brain?

A

Atrophyofbasalganglia:
-caudate nucleus andputamen

Corticalatrophyoccurslater

33
Q

How does Huntington’s disease appear microscopically in the brain?

A
  • Atrophyofstriatal neuronesthebasalganglia

- astrocyticgliosis

34
Q

What condition is responsible for Fronto-Temporal dementia?

A

Pick’s disease

35
Q

When does Pick’s disease usually present?

A

commencinginmiddlelife(usuallybetween50and60years)

36
Q

What symptoms do patients with Pick’s disease get (relating to damage of frontal/temporal lobes)?

A
  • Personalityandbehaviouralchange •Speechandcommunicationproblems •Changesineatinghabits
  • Reducedattentionspan
37
Q

Pick’s disease is rapidly progressive. How many years do patient’s normally live with this disease?

A

2-10 years

av. = 7

38
Q

How does Pick’s disease appear macroscopically?

A

Extremeatrophyofcerebral cortexinfrontalandlaterin temporallobes
•Brainweight<1Kg

39
Q

How does pick’s disease appear microscopically and what are the hallmark’s of this disease?

A

•Neuronallossandgliosis
Histologicalhallmarks:
•Pick’scells(swollenneurons)
•Intracytoplasmicfilamentous inclusions(Pick’s bodies)

40
Q

What groups of patient’s are most likely to get multi-infarct dementia?

A

Men >women
>60 = common
BUTalsoseeninmiddle‐aged hypertensives

41
Q

What clues can help to distinguish multi-infarct dementia from alzheimer’s disease

A
  • Abruptonset
  • Stepwiseprogression
  • Historyofhypertensionorstroke
  • Evidenceofstroke onCTorMRI

NEUROLOGY (29 decks)

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