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NEUROLOGY > Demyelination and Dementia NeuroPathology > Flashcards

Demyelination and Dementia NeuroPathology Flashcards

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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?

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

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

Study These Flashcards
A

FALSE

DementiaisNOTpartofthenormalageingprocess,itisalways pathological

19
Q

Give examples of Primary Dementia

Study These Flashcards
A
  • Alzheimer’sdisease

* Lewybodydementia

20
Q

Give examples of causes of secondary dementia

Study These Flashcards
A

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

21
Q

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

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

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

Study These Flashcards
A

FALSE

Deathusuallyoccursduetoasecondarycause,like bronchopneumonia

23
Q

Describe the macroscopic appearance of the brain in alzheimers dementia

Study These Flashcards
A

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

24
Q

What microscopic features are typical of Alzheimers disease?

Study These Flashcards
A
  • neuronalloss (+astrocyteproliferation = gliosis)
  • neurofibrilliary tanngles (in hippocampus and temporal lobe)
  • neuritic plaques due to amyloid angiopathy
25
Why is alzheimers disease thought to be more common in those with Down Syndrome (Trisomy 21)?
Genetically have more of amyloid protein precursor (on chromosome 21) => more at risk of amyloid angiopathy
26
Describe the microscopic appearance of amyloid angiopathy
- Extracellular eosinophils - Polymerised beta pleated sheats - Stains with Congo Red - Disrupts blood brain barrier => local oedema
27
What is Lewy Body dementia?
- progressive dementia - hallucinations  - fluctuating  levels of attention / cognition - Fluctuation on a day‐to‐day basis  - Features of Parkinsonism 
28
What are the clinical features of Parkinson's disease
- Loss of facial expression - stooping - shuffling gait - slow initiation of  movements - tiffness  - pill rolling tremor
29
Where in the brain does Lewy Body dementia cause degeneration?
In the substantia nigra (similar to parkinson's)
30
Describe how Lewy Body Dementia appears microscopically
- Loss of pigmented neurons - Reactive gliosis - Remaining neurons may show Lewy bodies:
31
When are patient's with huntington's disease usually diagnosed and when does dementia develop?
Usually diagnosed between 35-50 | dementia presents later in disease process
32
How does huntington's dementia appear macroscopically in the brain?
Atrophy of basal ganglia: - caudate  nucleus and putamen Cortical atrophy occurs later
33
How does Huntington's disease appear microscopically in the brain?
- Atrophy of striatal  neurones the basal ganglia | - astrocytic gliosis
34
What condition is responsible for Fronto-Temporal dementia?
Pick's disease
35
When does Pick's disease usually present?
commencing in middle life (usually between 50 and 60 years)
36
What symptoms do patients with Pick's disease get (relating to damage of frontal/temporal lobes)?
* Personality and behavioural change •Speech and communication problems •Changes in eating habits * Reduced attention span
37
Pick's disease is rapidly progressive. How many years do patient's normally live with this disease?
2-10 years | av. = 7
38
How does Pick's disease appear macroscopically?
Extreme atrophy of cerebral  cortex in frontal and later in  temporal lobes •Brain weight <1Kg
39
How does pick's disease appear microscopically and what are the hallmark's of this disease?
•Neuronal loss and gliosis Histological hallmarks: •Pick’s cells (swollen neurons) •Intracytoplasmic filamentous inclusions (Pick’s bodies)
40
What groups of patient's are most likely to get multi-infarct dementia?
Men > women >60 = common **BUT also seen in middle‐aged hypertensives**
41
What clues can help to distinguish multi-infarct dementia from alzheimer's disease
* Abrupt onset * Stepwise progression * History of hypertension or stroke * Evidence of stroke on CT or MRI

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