CNS Demyelinating Disorders Flashcards
1
Q
Autoimmune CNS demyelinating disorder with symptoms/lesions separated by time and space
A
Multiple sclerosis
2
Q
Multiple sclerosis is an autoimmune CNS demyelinating disorder with symptoms/lesions separated by this
A
Time (episodes of symptoms with intervals or remission) and space (distinct lesions with intervening unaffected areas)
3
Q
Is onset of multiple sclerosis abrupt or insidious?
A
Can be either
MS is a very variable disorder
4
Q
Are males or females more likely to have multiple sclerosis?
A
Females (3:1)
5
Q
What age does multiple sclerosis present?
A
Young adulthood, 20-40’s
6
Q
This ethnicity is most likely to have multiple sclerosis
A
Caucasians
7
Q
This autoimmune condition is most common in temperature areas (Northern NA and Europe, Southern Australia and NZ)
A
Multiple sclerosis
8
Q
Is living in low or high latitudes a risk factor for multiple sclerosis?
A
High latitudes
9
Q
Living in high latitudes is a risk factor for this autoimmune condition
A
Multiple sclerosis
10
Q
This genetic/antigen is a risk factor for multiple sclerosis
A
HLA-DR2
11
Q
HLA-DR2 is a risk factor for this autoimmune condition
A
Multiple sclerosis
12
Q
Deficiency of this is a risk factor for multiple sclerosis
A
Vitamin D
13
Q
Vitamin D deficiency is a risk factor for this autoimmune disorder
A
Multiple sclerosis
14
Q
These viral infections are possibly risk factors for multiple sclerosis
A
EBV or HHV-6
15
Q
An acute event of multiple sclerosis is due to autoimmunity against this
A
Oligodendrocyte antigens
(T cell driven process)
16
Q
This condition involves autoimmunity against oligodendrocyte antigens
T-cell driven process
A
Multiple sclerosis
17
Q
Are axons damaged in multiple sclerosis?
A
Are spared initially
Damage occurs when cycle is repeated
18
Q
What component of the CNS is initially damaged/destroyed in multiple sclerosis?
A
Oligodendrocytes and myelin sheath
19
Q
Are lymphocytes seen in pathology of multiple sclerosis?
A
Yes - perivascular lymphocytes
20
Q
Oligodendrocyte loss and preserved axons are seen in the pathology of this condition
A
Multiple sclerosis
21
Q
Late stage of this condition involves axon loss and gliosis that produces plaques
A
Multiple sclerosis
22
Q
Classic picture of this autoimmune condition is “relapse and remitting” deficits
A
Multiple sclerosis
23
Q
Optic neuritis occurs in multiple sclerosis due to damage of this nerve
A
CN II
24
Q
Does unilateral or bilateral internuclear ophthalmoplegia occur in multiple sclerosis?
A
Usually bilateral in MS
25
Internuclear ophthalmoplegia is seen in MS due to damage to this
CN VI / Multiple longitudinal fasciculus
26
Damage to this structure in multiple sclerosis causes head/neck ataxia, phonetic ataxia (scanning speech)
Cerebellum
27
Is flaccid or spastic paresis seen in multiple sclerosis?
Spastic
(due to UMN lesion)
28
This is demyelination and inflammation of optic nerve
Painful eye, especially on moving EOM
Variable vision loss
Optic neuritis
29
This describes when there is a loss of CN II sensory fibers with preserved CN III motor fibers, which prevents pupil constriction with light shone in lesional eye
Marcus-Gunn pupil
30
Marcus-Gunn pupil occurs in optic neuritis, and is due to a loss of these fibers
CN II sensory fibers
(CN III motor fibers are preserved)
31
Is fundoscopy normal in optic neuritis?
Yes
32
Do patients with optic neuritis recover normal vision?
Majority do at 2-3 months
33
What muscle is non-functional in internuclear ophthalmoplegia?
Middle rectus muscle
34
CSF with oligoclonal bands (due to B cell activation) is seen in this condition
Multiple sclerosis
35
These two components are mildly increased in CSF in multiple sclerosis
Protein and IgG
36
This is an autoantigen seen in the CSF of patients with multiple sclerosis
Myelin basic protein
37
CSF IgG index greater than this value indicates intrathecal IgG synthesis (possibly indicating multiple sclerosis)
0.6
38
Gadolinium leaking into parenchyma is seen in early lesion of this condition
Multiple sclerosis
39
Multiple sclerosis lesions favor these areas
Periventricular areas
40
Multifocal plaques, often perpendicular to surface, are seen radiographically in this condition
Multiple sclerosis
41
This is a subacute demyelinating disorder due to Polyoma JC virus reactivation
Progressive Multifocal Leukoencephalopathy
42
Progressive Multifocal Leukoencephalopathy is a subacute demyelinating disorder due to this
Polyoma JC virus reactivation
43
Is Progressive Multifocal Leukoencephalopathy serious?
Subacute; no disease in immunocompetent
44
Immune deficiency allows reactivation of this virus, causing Progressive Multifocal Leukoencephalopathy
Polyoma JC virus
45
Polyoma JC virus remains latent in these cells
Non-CNS cells
46
Progressive Multifocal Leukoencephalopathy infects these cells
Oligodendrocytes
47
Progressive Multifocal Leukoencephalopathy favors this part of cerebral hemispheres
White matter tracts
48
Progressive Multifocal Leukoencephalopathy favor the white matter tracts of cerebral hemispheres, particularly these 3 lobes
Frontal lobe (mentation and personality changes)
Occipital lobe (vision changes)
Parietal lobe (speech disorders)
49
Multifocal areas of demyelination of brain, with optic nerves and spinal column spared, are seen in this condition
Progressive Multifocal Leukoencephalopathy
50
This condition typically begins with disorders of vision, personality, or mentation
Progressive Multifocal Leukoencephalopathy
51
Progression over days to weeks, with focal neurologic defects, dementia, cortical blindness, paresis, dysarthria, and CB ataxia are seen in this condition
Progressive Multifocal Leukoencephalopathy
52
This condition involves rapidly progressive dementia that is usually fatal
Progressive Multifocal Leukoencephalopathy
53
Is there a cure for Progressive Multifocal Leukoencephalopathy?
No
54
This is the gold standard in diagnosis of Progressive Multifocal Leukoencephalopathy
Brain biopsy
55
Multifocal asymmetric white matter lesions are seen on MRI of this condition
Progressive Multifocal Leukoencephalopathy
56
Is there pleocytosis in multiple sclerosis?
Yes
57
Is there pleocytosis in Progressive Multifocal Leukoencephalopathy?
Minimal
58
Nuclear inclusions in oligodendrocytes with astrocytes also infected (with bizarre, enlarged nuclei) are seen morphologically in this condition
Progressive Multifocal Leukoencephalopathy
59
Astrocytes with bizarre, enlarged nuclei are seen morphologically in this condition
Progressive Multifocal Leukoencephalopathy
60
This condition involves rapid pontine demyelination due to correction of hypo-osmolarity
Central pontine myelinolysis
(AKA osmotic demyelinating syndrome)
61
Locked-in state involves sudden quadriplegia, mute but conscious, and vertical eye movements intact (but no other voluntary movements), and is seen in this condition
Central pontine myelinolysis
(AKA osmotic demyelinating syndrome)
62
Chronic EtOH, polydipsia, SIADH, and malnutrition are risk factors for this condition
Central pontine myelinolysis
(AKA osmotic demyelinating syndrome)
63
Sudden quadriplegia and pseudobulbar palsy (paralysis (including EOMs), dysarthria, dysphagia, "locked in state") are seen in this condition
Central pontine myelinolysis
(AKA osmotic demyelinating syndrome)
64
Is there inflammation in Central pontine myelinolysis?
No (macrophages only)
65
Condition that is spinal cord demyelination of dorsal and lateral columns due to vitamin B12 deficiency
Subacute combined degeneration
66
Subacute combined degeneration is spinal cord demyelination of dorsal and lateral columns due to this
Vitamin B12 deficiency
67
Subacute combined degeneration is spinal cord demyelination of dorsal and lateral columns due to deficiency in this
Vitamin B12
68
Luxol Fast Blue stains this blue
Myelin
69
Subacute combined degeneration involves demyelination of this structure
Dorsal and lateral columns of spinal cord
70
Vitamin B12 is used as a cofactor for these 2 enzymes
Methylmalonyl CoA mutase (fatty acid synthesis)
Methionine synthase (myelin basic protein synthesis)
71
Methylmalonyl CoA mutase and Methionine synthase use this as a cofactor
Vitamin B12
72
Is subacute combined degeneration a rapid or gradual demyelination?
Gradual
73
Does Subacute combined degeneration involve symmetric or asymmetric numbness of hands, then feet?
Symmetric
74
Subacute combined degeneration is a symmetric numbness of these
Hands, then feet
75
Eventual progression of this condition leads to spastic weakness of lower extremities
Subacute combined degeneration
76
This demyelinating disorder is often seen with anemia
Subacute combined degeneration
77
Megaloblastic anemia, Gastric achlorhydria and Ileal disease are associated with this demyelinating disorder
Subacute combined degeneration
78
These two compounds are elevated in a B12 deficiency, and are increased in Subacute combined degeneration
Homocysteine and Methylmalonic acid
79
Macrocytosis and Hypersegmented neutrophils are seen in this condition
Subacute combined degeneration
80
Inherited disorders of myelin production, maintenance of turnover
Leukodystrophies
81
Leukodystrophies are inherited disorders of production, maintenace, or turnover of this
Myelin
82
Krabbe disease, Metachromatic leukodystrophy and Adrenoleukodystrophy are in this group of inherited disorders of myelin
Leukodystrophies
83
This condition is caused by an autosomal recessive deficiency of Galactocerebroside beta-galactosidase
Krabbe disease
84
Krabbe disease is a deficiency of this enzyme
Galactocerebroside beta-galactosidase
85
What is the inheritance pattern of Krabbe disease?
Autosomal recessive
86
This condition results in accumulation of galactosphingosine, which is toxic to oligodendrocytes
Krabbe disease
87
Accumulation of galactosphingosine in Krabbe disease is toxic to oligodendrocytes, forming this
Globoid bodies
88
These are aggregates of engorged macrophages seen in Krabbe disease
Globoid bodies
89
Globoid bodies are aggregates of engorged macrophages, seen in this condition
Krabbe disease
90
Accumulation of galactosphingosine in Krabbe disease is toxic to these cells
Oligodendrocytes
91
When is the onset of Krabbe disease?
3-6 months
(is a rapidly progressive UMN disease)
92
In Krabbe disease, death occurs by this age
2 years
93
This condition is caused by an autosomal recessive deficiency of lysosomal Arylsulfatase A
Metachromatic leukodystrophy
94
Metachromatic leukodystrophy is a deficiency of this enzyme
Lysosomal Arylsulfatase A
95
What is the inheritance pattern of Metachromatic leukodystrophy?
Autosomal recessive
96
Metachromatic leukodystrophy results in accumulation of this
Sulfatides
97
Condition that results in accumulation of sulfatides, which is toxic to oligodendrocytes and results in demyelination and gliosis
Metachromatic leukodystrophy
98
When is the onset of Metachromatic leukodystrophy?
12-18 months
99
In Metachromatic leukodystrophy, death occurs by this age
10 years
100
This condition is caused by an X-linked recessive deficiency of ABCD1 gene
Adrenoleukodystrophy
101
Adrenoleukodystrophy is a deficiency of this
ABCD1 gene
102
What is the inheritance pattern of Adrenoleukodystrophy?
X-linked recessive
103
Leukodystrophy that results in defective fatty acid transport
Adrenoleukodystrophy
104
Adrenoleukodystrophy results in defective transport of this
Fatty acid
105
Who is more likely to have Adrenoleukodystrophy, males or females?
Young males
106
Young male with behavior changes and adrenal insufficiency may have this condition
Adrenoleukodystrophy
