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Neurology > Demyelinating Disorders > Flashcards

Demyelinating Disorders Flashcards

1
Q

What are components of myelin in the CNS that can be targeted by the immune system?

A
  • Myelin Basic Protein - cytoplasmic side

- Proteolipid Protein - intramembranous

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2
Q

What is key in determining a diagnosis of Multiple Sclerosis?

A

Lesions in Time and Space

  • different areas
  • different ages
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3
Q

What are the demographics of patients who are diagnosed with MS?

A
  • Temperate Climates
  • Female
  • 1st degree relatives who were diagnosed
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4
Q

What does the gross pathology look like in MS and where?

A
  • Periventricular White Matter degradation - loss of white
  • Optic Nerve and Chiasm
  • Cerebellum
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5
Q

What is the difference between an active plaque and and inactive plaque?

A

Active - macrophages with myelin debris and immune cells
Inactive - minimal macrophages and other immune cells
— Both have Axon preservations —

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6
Q

Where are MS lesions almost always found?

A

– Periventricular

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7
Q

Who does Acute Disseminated Encephalomyelitis generally affect the most?

A
  • Children
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8
Q

What might be the most likely diagnosis if perivenous demylination is found?

A
  • Acute Disseminated Encephalomyelitis
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9
Q

What is a characteristic finding of ADEM on MRI?

A
  • Generalized lesions throughout the white matter wide-spread “dissemination” - perivenular regions
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10
Q

What disorder is found to have antibodies to Aquaporin (AQP4)?

A

Neuromyelitis Optica

- attack the foot process of the astrocytes

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11
Q

When might you most common find ADEM manifest in a child?

A

Following a viral infection or vaccination

– due to cross reactive antibodies –

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12
Q

What are findings consistent with Neuromyelitis Optica?

A
  • Neuritis of the optic nerve/chiasm (necrosis/inflammation)

- Longitudinal Spinal Cord Demyelinating Lesions

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13
Q

If a chronic alcoholic presents to the ED with severe hyponatremia and is rapidly infused with saline, what is he at risk for?

A

Central Pontine Myelinolysis

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14
Q

What are the clinical manifestations of central pontine myelinolysis?

A

Damage to the pons

  • weakness in all extremities
  • dysfunctional cranial nerves
  • “locked in syndrome”
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15
Q

What cells are thought to be most responsible for Multiple sclerosis?

A
  • CD4 T-cells autoreactive to self oligodendrocytes
    • Myelin Basic Protein (MBP)
    • Myelin Oligo Glycoprotein (MOG)
    • Proteolipid Protein (PLP)
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16
Q

What are general characteristics that occur during the etiology of MS?

A
  • Activation of CD4 cells
  • Breakdown of BBB, allowing more immune infiltrates
  • Demyelination at areas of infiltrate
17
Q

What are common symptoms that occur at onset of MS, compared to symptoms during disease course?

A
  • Sensory Distruptions
  • Weakness
  • Visual Loss
  • -Weakness
  • -Sensory
  • -Ataxia
  • -Bladder Dysfunction
18
Q

What are the different modalities the disease course can present?

A
  • Relapsing-Remitting – acute episodes, returns to baseline or gets worse after each attack (Most patients)
  • Secondary Progressive – acute attacks with worsening baseline between and after each attack (From Relap-Remit)
  • Primary Progressive – constant progressive without attacks
  • Progressive-relapsing – constant progressing with attacks
19
Q

Can you diagnose a patient with MS if they have focal clinical finding with single lesion identified on MRI?

A

– NO. Must be “dissemination in time and space”

They have to have another episode

20
Q

What are clinical criteria used for diagnosing MS?

A
  • Need >1 lesion on MRI and more than one attack, or suspicious history of previous attack
    “Time and Space Disseminated”
21
Q

How is Clinically Isolated Syndrome different from MS?

A

This is considered the FIRST clinical demyelinating event, predictive of development of MS

    • if there is already one lesion = 88% chance of MS development
    • if there are no lesions = 19% chance
22
Q

What are the current treatments to treat MS?

A

For reducing symptoms and disability

- Interferon B – delays progression

23
Q

If a patient develops acute vision loss and pain with movement of the eyes, what is the most concerning diagnosis?

A

– Demyelinating Optic Neuritis

24
Q

What are the classic signs of Demyelinating Optic Neuritis?

A
  • -Female 30s
  • -Decreases acuity, color, and contrast vision
  • -Pain with eye movements
25
Q

For Demyelinating Optic Neuritis, do steriod change the long term prognosis?

A

Oral Prednisone – INCREASES RISK of Recurrance

  • -IV Steroids – increase rate of recovery from the episode without increased rate of recurrance
    • Most people fully recover without lasting deficits
26
Q

What are benefits of giving IV Steroids to Demyelinating Optic Neuritis during an acute episode?

A
  • -Decreases amount of time to recover

- -Short-term protective effect against MS

27
Q

What are the risks associated with DON and MS?

A
  • if DON does not have any MRI lesions = 25% Risk of MS

- if DON has at least 1 lesion = 72% Risk of MS

28
Q

What is a common presentation for Acute Disseminated Encephalomyelitis?

A

– Multiple Neurologic deficits
– Children who are very ill, possibly coma
However, Prognosis is very good with steroid treatment

29
Q

What is a unique characteristic of ADEM on imaging?

A
  • -Multiple/extensive lesions in both hemispheres including grey matter involvement
    • Histo –> Perivenous Demyelination/Inflammation
30
Q

If a patient is experiencing electrical sensations in the arms due to neck movement and/or intermittent brief attacks of painful limb movement triggered by limb movement, what might be the cause?

A

Transverse Myelitis

  • -Lhermitte Sign – electric sensations
  • -Paroxysmal Tonic Spasms – extremities pain
31
Q

What is the difference between complete and incomplete transverse myelitis?

A
    • complete – most severe, more suggestive of infectious disorder
    • incomplete – most suggestive of progressing to MS
  • *Treat with high dose IV steroids if not infectious**
32
Q

If a patient develops marked vision loss and an MRI finds scattered areas of demyelination in the optic nerves and longitudinal spinal lesion (3+ segments). What might be most likely diagnosis?

A

Neuromyelitis Optica

33
Q

What might be a characteristic finding in Neuromyelitis Optica?

A

–Optic disc may appear swollen
– severe loss of vision, sometimes bilateral
++Aquaporin-4 IgG Antibody to Astrocyte foot processes

34
Q

What is the difference between Neuromyelitis Optica and Demyelinating Opticneuritis?

A

Differences –
DON – Pain with eye movement, inflammation of optic nerve – not necessarily lesion there, IV steroids help, associated w/ MS

NMO – swollen optic disc, scattered dymyelination of optic nerves/SPINAL CORD involvement + Aquaporin Antibodies

Neurology (11 decks)

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