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Pathology II Exam 3 > 7 degenerative and demylinating Diseases > Flashcards

7 degenerative and demylinating Diseases Flashcards

1
Q

Is demyelination or dysmyelination acquired? What does this mean about the original myelin?

A
  1. demyelination

2. originally normal myelin as opposed to abnormal myelin in dysmyelination

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2
Q

Are axons usually preserved in in dysmyelination?

A

No- they usually undergo degeneration. They are preserved in demyelination

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3
Q

Can remyelination occur in demyelination?

A

Yes

In dysmelination- myelin sheaths may not form, those that do are abnormal and often undergo degradation

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4
Q

T-F- diphtheria causes toxic demyelination of the CNS?

A

False- PNS

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5
Q

What is defined as an acquired autoimmune demyelinating disease typified by well defined episodes of neurological deficits separated by time and by space?

A

Multiple sclerosis

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6
Q

What does separated by time and by space mean?

A

multifocal with lesions of different ages and in different site.

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7
Q

T-F- multiple sclerosis is the 2nd most common demyelinating disease of the CNS?

A

False- most

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8
Q

In MS- more commonly progressive or relapsing and remitting ?

A

Relapsing and remitting in 80%

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9
Q

There are 4 types of MS- classic, acute, neuromyelitis optics, schilder’s– which one has spinal cord and optic nerve involvement? What is the defect in?

A
  1. Neuromyelitis Optica (Devic’s type)

2. Aquaporin 4

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10
Q

What type of MS- occurs in children, extensive demyelination, acute, can be remitting? does it respond to steroids?

A

Shilder’s disease

Yes

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11
Q

Is MS more common in men or women? age? is there a genetic component?

A
  1. Women 4.1 to 1
  2. Age- 20-40
  3. Yes- [risk 20x if in first degree relatives, Class II MHC, DR2, DR4, DR15 DQ6, Polygenic]
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12
Q

What is the etiology of MS?

A

Immunological- antibodies against components of myelin sheath.
There is a CD4+ Th-1 T-cell mediated attack against a number of oligodendrocyte and white matter antigens.

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13
Q

Normally- are B lymphocytes included in the CNS?

A

B-lymphocytes are excluded from the CNS

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14
Q

What happens to B-lymphoctes in the CSF in MS?

A

T-helper cell induced clonal expansion in the CSF producing IgG immunoglobulins

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15
Q

What is the diagnostic hallmark of MS?

A

oligoclonal banding seen on CSF electrophoresis due to clonal proliferation

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16
Q

The antibody antigen complex formed by antibodies to the myelin proteins is recognized by what?

A

Fc receptos on the surface of macrophages

[this with complement activation results in damage to myelin and phagocytksed by macrophages]

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17
Q

Does lymphocyte mediated injury take place in multiple sclerosis?

A

Yes- C8+ T cells are thought to target oligodendroglia

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18
Q

Besides ingesting myelin- how to macrophages damage myelin?

A

productions of ROS and nitrogen species, proteolytic enzymes, cytokines

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19
Q

Where are MS demyelinating plaques frequently found?

A

periventricular

  • but can be anywhere in the white matter of the brain and at the junction of cortex and white matter, brain stem, spinal cord, optic chiasm, cerebellum
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20
Q

What do macrophages distinctively contain in them in MS?

A

myelin debris

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21
Q

Does and MS active or inactive plaques have long term loss of axons, loss of oligodendroglia and gliosis/cavitation?

A

Inactive

-[active has loss of myelin, preservation of axon, perivascular T cells]

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22
Q

What does the shadow plaque in MS have?

A

reduced myelin and remyelinating axons

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23
Q

What does the luxol fast blue stain?

A

myelin

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24
Q

What does the bielschowsky stain stain?

A

axons

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25
Q

What stain is best for remyelination?

A

luxol fast blue stain

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26
Q

What does the luxol fast blue stain?

A

myelin- Really makes macrophages with myelin bodies really stand out

27
Q

What does the bielschowsky stain stain?

A

axons- a type of silver stain

28
Q

What stain is best for remyelination?

A

luxol fast blue stain

29
Q

Note on the luxol fast blue stain-

A

active plaque is nearly white and the shadow plaque is in between the dark blue and the white color. KINDA LIKE A SHADOW I GUESS

30
Q

What form of MS is acute, rare, rapidly progressive with a poor outcome, severe disability and death?

A

marburg variant

31
Q

What are some of the signs of the marburg variant?

A

confusion, HA, vomiting, gait unsteadiness and hemiparesis

32
Q

What protein is messed up in neuromyelitis optica?

A

Aquaporin 4- part of the integrity system of the BBB

33
Q

What are the 2 primary Poser diagnostic criteria for Schilder’s Disease? review the others

A
  1. acute/subacute with one or more roughly symmetrical bilateral plaques measuring at least 2x3cm involving the centrum semiovale
  2. No other lesions clinically, paraclinical, or imaging
  • no PNS lesions
  • normal adrenal function
  • normal very long chain fatty acids
  • pathology resembles MS
34
Q

What is a mono phasic demyelinating disease that predominantly involves the pontine basis? What is it caused by? how does it rapidly present?

A
  1. Central pontine myelinolysis
  2. complication of rapidly correcting hyponatremia, or low magnesium
  3. confusion, limb weakness, conjugate gaze palsy, dysarthri, dysphagia- FATAL IN WEEKS.
35
Q

Guillain-Barre Syndrome - also known as acute inflammatory demyelinating polyradiculoneuropathy is a rapidly ascending paralysis of spinal nerve roots. What is a key laboratory characteristic of this condition?

A

VERY HIGH CSF PROTEIN

36
Q

Is acute disseminated encephalomyelitis (inflammatory disease) a type II or type IV hypersensitivity case? What is the outcome?

A
  1. Type IV- T-cell mediated hypersensitivity reaction

2. Resolve over weeks in most, steroids + plasmophoresis, 20% die in acute phase

37
Q

Name(review) some viral, bacterial and iatrogenic causes?

A

viral- measles, mumps, varicella, rubella, influenza, imono

bacteria- mycoplasma, campylobacter, strepA (rare)

iatrogenic- gold, levamisole, 5FU

38
Q

What causes progressive multifocal leukoencephalopathy? is it progressive?

A
  1. polyoma virus/ JC virus

2. yes- leading to death

39
Q

What disease is described by the following pathology- multiple foci of demyelination, viral inclusions in oligodendroglia nuclei, atypical/bizarre astrocytes and perivascular inflammatory infiltrate?

A

progressive multifocal leukoencephalopathy

40
Q

What are the different signs between thiamine deficiency types WErnicke’s and Korsakoff’s?

A

Wernicke- gaze palsy, ataxia, apathy, clouded conscious

Korsakoff- retro and anterograde amnesia and confabulation

41
Q

What causes direct toxicity in cerebellar degeneration?

A

thiamine

42
Q

What chemo drug is very prominent in neuro toxicity?

A

methotrexate

43
Q

What toxicity leads to hypoesthesia of hands and feet, ataxia, impairment of hearing, visual constriction, dysarthria, maybe coma and death?

A

mercury- methyl mercury especially through consumption of contaminated fish or skin lightening creams used in pregnant women.

44
Q

What does a gross brain with CO poisoning look like?

A

Tons of little red dots everywhere (hypoxia)- symmetrical necrosis of the globus pallidus
[movement disorders in survivors]

45
Q

Can remyelination occur in demyelination?

A

Yes

In dysmelination- myelin sheaths may not form, those that do are abnormal and often undergo degradation

46
Q

There are 4 types of MS- classic, acute, neuromyelitis optics, schilder’s– which one has spinal cord and optic nerve involvement? What is the defect in?

A
  1. Neuromyelitis Optica (Devic’s type)

2. Aquaporin 4

47
Q

What type of MS- occurs in children, extensive demyelination, acute, can be remitting? does it respond to steroids?

A

Shilder’s disease

Yes

48
Q

Is MS more common in men or women? age? is there a genetic component?

A
  1. Women 4.1 to 1
  2. Age- 20-40
  3. Yes- [risk 20x if in first degree relatives, Class II MHC, DR2, DR4, DR15 DQ6, Polygenic]
49
Q

What is the etiology of MS?

A

Immunological- antibodies against components of myelin sheath.
There is a CD4+ Th-1 T-cell mediated attack against a number of oligodendrocyte and white matter antigens.

50
Q

What is the diagnostic hallmark of MS?

A

oligoclonal banding seen on CSF electrophoresis due to clonal proliferation

51
Q

The antibody antigen complex formed by antibodies to the myelin proteins is recognized by what?

A

Fc receptos on the surface of macrophages

[this with complement activation results in damage to myelin and phagocytksed by macrophages]

52
Q

Besides ingesting myelin- how to macrophages damage myelin?

A

productions of ROS and nitrogen species, proteolytic enzymes, cytokines

53
Q

What stain is best for remyelination?

A

luxol fast blue stain

54
Q

Note on the luxol fast blue stain-

A

active plaque is nearly white and the shadow plaque is in between the dark blue and the white color. KINDA LIKE A SHADOW I GUESS

55
Q

What are some of the signs of the marburg variant?

A

confusion, HA, vomiting, gait unsteadiness and hemiparesis

56
Q

What protein is messed up in neuromyelitis optica?

A

Aquaporin 4- part of the integrity system of the BBB

57
Q

What are the 2 primary Poser diagnostic criteria for Schilder’s Disease? review the others

A
  1. acute/subacute with one or more roughly symmetrical bilateral plaques measuring at least 2x3cm involving the centrum semiovale
  2. No other lesions clinically, paraclinical, or imaging
  • no PNS lesions
  • normal adrenal function
  • normal very long chain fatty acids
  • pathology resembles MS
58
Q

What is a mono phasic demyelinating disease that predominantly involves the pontine basis? What is it caused by? how does it rapidly present?

A
  1. Central pontine myelinolysis
  2. complication of rapidly correcting hyponatremia, or low magnesium
  3. confusion, limb weakness, conjugate gaze palsy, dysarthri, dysphagia- FATAL IN WEEKS.
59
Q

Guillain-Barre Syndrome - also known as acute inflammatory demyelinating polyradiculoneuropathy is a rapidly ascending paralysis of spinal nerve roots. What is a key laboratory characteristic of this condition?

A

VERY HIGH CSF PROTEIN

60
Q

What disease is described by the following pathology- multiple foci of demyelination, viral inclusions in oligodendroglia nuclei, atypical/bizarre astrocytes and perivascular inflammatory infiltrate?

A

progressive multifocal leukoencephalopathy

61
Q

What are the different signs between thiamine deficiency types WErnicke’s and Korsakoff’s?

A

Wernicke- gaze palsy, ataxia, apathy, clouded conscious

Korsakoff- retro and anterograde amnesia and confabulation

62
Q

What causes direct toxicity in cerebellar degeneration?

A

thiamine

63
Q

What chemo drug is very prominent in neuro toxicity?

A

methotrexate

Pathology II Exam 3 (13 decks)

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