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Pathoma LAH > Neuro: 17.6: Demyelinating Disorders > Flashcards

Neuro: 17.6: Demyelinating Disorders Flashcards

1
Q

What causes central pontine myelinolysis?

A
  • rapid IV correction of hyponatremia
  • *** “From low to high, your pons will die”
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2
Q

Which cell does the myelination in the PNS?

A

Schwann cells

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3
Q

What causes bowel/bladder/sexual dysfunction in MS?

A

involvement of the autonomic nervous system (ANS)

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4
Q

What does “panencephalitis” mean?

A
  • pan = entire brain (both gray and white matter)
  • encephalitis = inflammation of the brain
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5
Q

What is the outcome of subacute sclerosing panencephalitis (SSPE)?

A

progressive debilitating encephalitis –> death

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6
Q

Name 2 tests to diagnose multiple sclerosis (MS).

A
  1. MRI
  2. LP
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7
Q

This is a pair of tracts that allows crosstalk btw CN3 and CN6 nuclei; it coordinates horizontal eye mvmts.

A

the medial longitudinal fasciculus (MLF)

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8
Q

What are the clinical features of multiple sclerosis?

A
  • blurred/nystagmus/loss of vision in 1 eye
  • vertigo
  • scanning speech
  • internuclear ophthalmoplegia (INO)
  • hemiparesis or unilateral loss of sensation
  • lower extremity loss of sensation or weakness
  • bowel/bladder/sexual dysfunction
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9
Q

What is glatiramer?

A

a treatment for MS

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10
Q

What is the prognosis for adrenoleukodystrophy?

A
  • leads to long-term coma/death
  • adrenal gland crisis
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11
Q

What is leukodystrophy?

A
  • inherited mutations in enzymes necessary for the production or maintenance of myelin
  • white matter dysfunction w/I the brain
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12
Q

What happens in metachromatic leukodystrophy?

A

myelin cannot be degraded –> myelin and sulfatides accumulate in lysosomes

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13
Q

This drug is a glycoprotein normally expressed by virus-infected cells –> antiviral, antitumoral properties.

A

interferon-beta

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14
Q

What are the SEs of interferon-beta?

A
  • flu-like symptoms
  • depression
  • neutropenia
  • myopathy
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15
Q

What happens in Type I hypersensitivity reactions?

A
  • free antigen cross-links IgE on presensitized mast cells and basophils –> vasoactive amine release to postcapillary venules
  • anaphylactic and atopic
  • usually an immediate reaction
  • *** “First and Fast”
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16
Q

In adrenoleukodystrophy, fatty acids cannot be degraded. Where do they then accumulate?

A
  • adrenal glands
  • white matter
  • testes
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17
Q

What causes multiple sclerosis?

A

autoimmune destruction of CNS myelin AND oligodendrocytes

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18
Q

How does central pontine myelinolysis present?

A

acute paralysis- “locked-in syndrome”

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19
Q

Dx?

  • viral inclusions within both the neurons (gray matter) and oligodendrocytes (white matter)
A

subacute sclerosing panencephalitis (SSPE)

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20
Q

How does metachromatic leukodystrophy present?

A
  • ataxia
  • dementia
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21
Q

What is another name for “locked-in syndrome?”

A

Osmotic demyelination syndrome

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22
Q

The CN3 (oculomotor) nucleus of the right eye is connected to the ______ of the left eye.

A

CN6 (abducens) nucleus

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23
Q

What causes Krabbe disease?

A

a deficiency of galactocerebroside beta-galactosidase –> destruction of myelin sheath

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24
Q

Are axons myelinated in the CNS or PNS?

A

both

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25
Q

Name 3 diseases in which the oligodendrocytes are injured.

A
  1. MS
  2. progressive multifocal leukoencephalopathy (PML)
  3. leukodystrophies
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26
Q

What causes blurred vision in MS?

A

involvement of the optic nerve

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27
Q

What causes scanning speech in MS?

A

involvement of the brainstem

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28
Q

What kind of hypersensitivity reaction is MS?

A

type IV = delayed, T-cell-mediated

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29
Q

“Locked-in syndrome” can occur with ____ or ____.

A
  • central pontine myelinolysis
  • a basilar artery stroke
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30
Q

Which is the most common leukodystrophy?

A

metachromatic leukodystrophy

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31
Q

What causes lower extremity loss of sensation or weakness in MS?

A

involvement of the spinal cord

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32
Q

When does subacute sclerosing panencephalitis (SSPE) present?

A

years after a measles infection (which usu. occurs in infancy)

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33
Q

What is another name for Charcot-Marie-Tooth disease?

A

hereditary motor and sensory neuropathy (HMSN)

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34
Q

What is another name for Osmotic demyelination syndrome?

A

“locked-in syndrome”

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35
Q

What MRI findings are diagnostic of multiple sclerosis (MS)?

A
  • multiple white matter lesions separated in space and time
  • periventricular plaques
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36
Q

Which nerve innervates the lateral rectus muscle?

A

CN 6- abducens

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37
Q

How does Charcot-Marie-Tooth disease present?

A
  • foot deformities (pes cavus)
  • lower extremity weakness
  • sensory deficits
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38
Q

How is adrenoleukodystrophy transmitted?

A

X-linked

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39
Q

Each Schwann cell can myelinate _____ axons.

A

1

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40
Q

What is the Charcot triad of MS?

A
  1. Scanning speech
  2. Intention tremor/Incontenence/Internuclear ophthalmoplegia (INO)
  3. Nystagmus
  • *** “Triad is a SIN”
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41
Q

What causes myelination disorders?

A

destruction of the myelin or oligodendrocytes

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42
Q

What causes hemiparesis or unilateral loss of sensation in MS?

A

involvement of the cerebral white matter

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43
Q

What causes Marcus Gunn pupils?

A

optic nerve damage or severe retinal injury–> an afferent pupillary defect

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44
Q

How is metachromatic leukodystrophy transmitted?

A

it’s an auto-recessive lysosomal storage disease

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45
Q

What is the most common chronic CNS disease of young adults?

A

multiple sclerosis

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46
Q

Which nerve innervates the medial rectus muscle?

A

CN3 - oculomotor

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47
Q

This is decreased bilateral pupillary constriction when light is shone in the affected eye (relative to the unaffected eye).

A

Marcus Gunn pupils

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48
Q

How is Krabbe disease transmitted?

A

auto. recessive

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49
Q

HLA-DR2 is associated with which diseases?

A
  • MS
  • hay fever
  • SLE
  • Goodpasture
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50
Q

What gross findings can be seen on a brain specimen from an MS pt?

A

patches of gray within the white matter (areas of demyelination)

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51
Q

The CN3 (oculomotor) nucleus of the right eye is connected to the CN6 (abducens) nucleus of the left eye with the _____ in between.

A

medial longitudinal fasciculus (MLF)

52
Q

What are Schwann cells derived from?

A

neural crest cells

53
Q

Dx?

  • a JC virus infection of oligodendrocytes that is reactivated due to immune suppression –> demyelination of the CNS
A

progressive multifocal leukoencephalopathy

54
Q

What is the tx for MS, both acutely and long-term?

A
  • acute = high-dose steroids
  • long-term = interferon-beta, natalizumab, glatiramer
55
Q

Dx?

  • blurred/nystagmus/loss of vision in 1 eye
  • vertigo
  • scanning speech
  • internuclear ophthalmoplegia (INO)
  • hemiparesis or unilateral loss of sensation
  • lower extremity loss of sensation or weakness
  • bowel/bladder/sexual dysfunction
A

multiple sclerosis

56
Q

Name the hypersensitivity reaction:

  • cytotoxic/antibody mediated
  • IgM, IgG bind to fixed antigen on cell –> destruction via MAC
A

Type II hypersensitivity reaction

57
Q

Who typically gets adrenoleukodystrophy? Why?

A
  • usually males
  • it’s X-linked
58
Q

What is Charcot-Marie-Tooth disease?

A

an auto dominant, progressive nerve disorder related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath

59
Q

How does progressive multifocal leukoencephalopathy present?

A

rapidly progressing neuro. signs –> death

60
Q

What causes Charcot-Marie-Tooth disease?

A
  • auto dominant inheritance
  • defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath
61
Q

What accumulates in adrenoleukodystrophy?

A

long-chain fatty acids

62
Q

What causes progressive multifocal leukoencephalopathy?

A

a JC virus infection of oligodendrocytes that is reactivated due to immune suppression –> demyelination of the CNS

63
Q

Marcus Gunn pupils are associated with _____.

A

MS

64
Q

In myelination disorders, what damage occurs to the axon?

A

myelin is damaged but the axons themselves are preserved

65
Q

What accumulates in Krabbe disease?

A
  • galactocerebroside
  • psychosine
66
Q

What causes adrenoleukodystrophy?

A

impaired addition of coenzyme A to long-chain fatty acids

67
Q

Each oligodendrocyte can myelinate _____ axons.

A

up to 30

68
Q

What does CN3 (oculomotor) innervate?

A

the medial rectus muscle of the eye

69
Q

What happens in Type IV hypersensitivity reactions?

A
  • sensitized T cells encounter antigen –> release cytokines –> activate macs
  • *** delayed reaction
70
Q

In metachromatic leukodystrophy, myelin and sulfatides cannot be degraded. Where do they then accumulate?

A

in lysosomes

71
Q

Dx?

  • an auto dominant, progressive nerve disorder related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath
A

Charcot-Marie-Tooth disease

72
Q

Name 2 drugs that increase the risk of progressive multifocal leukoencephalopathy.

A
  1. natalizumab
  2. rituximab
73
Q

What is natalizumab?

A
  • a tx for MS and Crohns
  • an antibody to alpha-4-integrin
74
Q

What are Marcus Gunn pupils?

A

when there is decreased bilateral pupillary constriction when light is shone in the affected eye (relative to the unaffected eye)

75
Q

Give an example of the findings in internuclear ophthalmoplegia (INO). What causes this?

A
  • Ex: as the pt to look to the Left: left eye looks left right eye doesn’t move left
  • cause: damage to the medial longitudinal fasciculus (MLF) connecting CN6 of L eye to CN3 of R eye
76
Q

What will happen if the cerebral white matter is involved in an MS pt?

A
  • hemiparesis
  • unilateral loss of sensation
77
Q

How does Krabbe disease present?

A
  • peripheral neuropathy
  • developmental delay
  • optic atrophy
  • globoid cells
78
Q

What causes internuclear ophthalmoplegia (INO) in MS?

A

involvement of the medial longitudinal fasciculus (MLF)

79
Q

What is another name for hereditary motor and sensory neuropathy (HMSN)?

A

Charcot-Marie-Tooth disease

80
Q

How does interferon-beta work?

A

it is a glycoprotein normally expressed by virus-infected cells –> antiviral, antitumoral properties

81
Q

Based on these LP findings, what is the diagnosis?

  • increased lymphocytes
  • increased Ig with oligoclonal IgG bands
  • myelin basic protein
A

multiple sclerosis (MS)

82
Q

What is the prognosis for progressive multifocal leukoencephalopathy?

A

death

83
Q

What is the medial longitudinal fasciculus (MLF)?

A
  • a pair of tracts that allows crosstalk btw CN3 and CN6 nuclei
  • coordinates horizontal eye mvmts
84
Q

What will happen if the medial longitudinal fasciculus (MLF) is involved in an MS pt?

A

internuclear ophthalmoplegia (INO)

85
Q

How does natalizumab work?

A

it’s an antibody to alpha-4-integrin

86
Q

This is a tx for MS and Crohns; an antibody to alpha-4-integrin.

A

natalizumab

87
Q

What happens in Type III hypersensitivity reactions?

A
  • immune-complex mediated
  • antigen-antibody (IgG) complexes activate complement –> attracts neutrophils –> release lysosomal enzymes
  • *** “Type 3 is 3 things stuck together: antigen-antibody-complement”
88
Q

What causes metachromatic leukodystrophy?

A

it’s an auto recessive deficiency of arylsulfatase (this is a lysosomal storage disease)

89
Q

What is an oligodendrocyte?

A

a cell in the CNS that does the myelination

90
Q

What happens in Type II hypersensitivity reactions?

A
  • cytotoxic/antibody mediated
  • IgM, IgG bind to fixed antigen on cell –> destruction via MAC
  • *** “Type 2 is cy-2-toxic”
91
Q

What LP findings are diagnostic of multiple sclerosis (MS)?

A
  • increased lymphocytes
  • increased Ig with oligoclonal IgG bands
  • myelin basic protein
92
Q

What genetic marker is assoc. with multiple sclerosis?

A

HLA-DR2

93
Q

What does a deficiency of arylsulfatase cause?

A

metachromatic leukodystrophy

94
Q

An optic nerve damage or severe retinal injury–> an afferent pupillary defect called ______.

A

Marcus Gunn pupils

95
Q

Dx?

  • focal demyelination of the pons due to rapid IV correction of hyponatremia
A

central pontine myelinolysis

96
Q

What does CN6 (abducens) innervate?

A

the lateral rectus muscle of the eye

97
Q

What happens in Krabbe disease?

A

galactocerebroside cannot be degraded –> accumulates in macrophages

98
Q

What happens when hypernatremia is corrected too quickly?

A
  • cerebral edema/herniation
  • *** “From high to low, your brain will blow”
99
Q

In general, these are inherited mutations in enzymes necessary for the production or maintenance of myelin (white matter dysfunction w/I the brain).

A

leukodystrophies

100
Q

What will happen if the autonomic nervous system (ANS) is involved in an MS pt?

A

bowel/bladder/sexual dysfunction

101
Q

Dx?

  • a deficiency of galactocerebroside-beta-galactosidase –> destruction of myelin sheath
A

Krabbe disease

102
Q

Dx?

  • paralysis of ALL voluntary movements EXCEPT for the eyes
A

“locked-in syndrome”

103
Q

What causes vertigo in MS?

A

involvement of the brainstem

104
Q

What will happen if the brainstem is involved in an MS pt?

A
  • vertigo
  • scanning speech
105
Q

What will happen if the optic nerve is involved in an MS pt?

A

blurred/loss of vision in 1 eye

106
Q

Dx?

  • autoimmune destruction of CNS myelin and oligodendrocytes
A

multiple sclerosis

107
Q

What accumulates in metachromatic leukodystrophy?

A
  • myelin
  • sulfatides
108
Q

What destroys Schwann cells?

A

Guillan-Barre syndrome (campylobacter)

109
Q

Dx?

  • impaired addition of coenzyme A to long-chain fatty acids
A

adrenoleukodystrophy

110
Q

Rapid IV correction of ______ can cause central pontine myelinolysis.

A
  • hyponatremia
  • *** “From low to high, your pons will die”
111
Q

What is “locked-in syndrome?”

A

paralysis of ALL voluntary movements EXCEPT for the eyes

112
Q

What is scanning speech similar to?

A

drunk speech

113
Q

What will happen if the spinal cord is involved in an MS pt?

A

lower extremity loss of sensation or weakness

114
Q

Who gets central pontine myelinolysis?

A

severely malnourished pts (alcoholics, liver disease, etc)

115
Q

Name the hypersensitivity reaction:

  • sensitized T cells encounter antigen –> release cytokines –> activate macs
  • *** delayed reaction
A

Type IV hypersensitivity reaction

116
Q

What color is the white matter within the brain? Why?

A
  • white
  • it’s heavily myelinated
117
Q

What causes subacute sclerosing panencephalitis?

A

persistent brain infection by measles virus

118
Q

Name the hypersensitivity reaction:

  • free antigen cross-links IgE on presensitized mast cells and basophils –> vasoactive amine release to postcapillary venules
  • anaphylactic and atopic
  • usually an immediate reaction
A

Type I hypersensitivity reaction

119
Q

What characterize subacute sclerosing panencephalitis (SSPE)?

A

viral inclusions within both the neurons (gray matter) and oligodendrocytes (white matter)

120
Q

Where does multiple sclerosis most commonly occur in the world?

A

regions far from the equator

121
Q

Name the hypersensitivity reaction:

  • immune-complex mediated
  • antigen-antibody (IgG) complexes activate complement –> attracts neutrophils –> release lysosomal enzymes
A

Type III hypersensitivity reaction

122
Q

Which cell does the myelination in the CNS?

A

oligodendrocytes

123
Q

What is central pontine myelinolysis?

A

focal demyelination of the pons

124
Q

Why is myelin basic protein found in the CSF in MS?

A

bc myelin is being degraded

125
Q

What color is the gray matter in the brain? Why? Where is it located?

A
  • gray
  • no myelin
  • at the periphery
126
Q

In Krabbe disease, galactocerebroside cannot be degraded. Where does it then accumulate?

A

in macrophages

127
Q

What does a persistent brain infection by measles virus cause?

A

subacute sclerosing panencephalitis (SSPE)

Pathoma LAH (77 decks)

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