Demyelination/Demyelinating Disease Flashcards

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1
Q

Demyelination or dysmyelination?

Acquired

A

Demyelination

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2
Q

Demyelination or dysmyelination?

Abnormal myelin

A

Dsymyelination

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3
Q

Demyelination or dysmyelination?

Myelin loss w/ relative preservation of axons

A

Demyelination

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4
Q

Demyelination or dysmyelination?

Remyelination frequently occurs

A

Demyelination

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5
Q

Demyelination or dysmyelination?

Genetic

A

Dysmyelination

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6
Q

Demyelination or dysmyelination?

Axons can undergo degeneration

A

Dysmyelination

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7
Q

Most common demyelinating disorder

A

Multiple Sclerosis (MS)

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8
Q
Relapsing-remitting phase of MS characterized by all of the follow EXCEPT:
A. Frequent inflammation
B. Infrequent relapses 
C. Remyelination
D. Complete recover from disability
A

B. Frequent relapses

Infrequent relapses characteristic of secondary-progressive phase

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9
Q

Multiple Sclerosis is caused mostly by ___

A

autoimmune destruction by CD4 T cells (TH1, TH17)

Also CD8 T cells, macrophages, B cells

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10
Q

Luxol Fast Blue stains for ___

A

myelin

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11
Q

Bielschowsky stains for ___

A

axons

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12
Q

T/F: MS on imaging present and non-enhancing areas in white matter

A

False. Enhancing

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13
Q

MS presents grossly as (2)

A

Plaques in any white matter tracts in cerebrum, brainstem, cerebellum or spinal cord
Enlargement of ventricles via ex vacuo hydrocephalus

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14
Q

Name the disease:

Inflammatory demyelinating disease centered on optic nerve and spinal cord

A

Neuromyelitis Optica (NMO)

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15
Q

Name the disease:

Antibody mediated by anti-aquaporin-4 antibodies

A

Neuromyelitis Optica

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16
Q

Name the disease:

Postinfection autoimmune reaction to myelin

A

Acute Disseminated Encephalomyelitis (ADEM)

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17
Q

T/F: Acute Disseminated Encephalomyelitis (ADEM) presents with plaques around arteries

A

False. Plaques are veins ONLY

18
Q

Which of the following about Acute Disseminated Encephalomyelitis (ADEM) is FALSE?
A. Large sharply demarcated plaques
B. Perivenous inflammation w/ sleeves of demyelination
C. Rapidly progressive, fatal in 20%
D. Nonspecific symptoms including headache, lethargy, coma.
E. Symptoms present 1-2 weeks after infection

A

A. NO large sharply demarcated plaques

19
Q

Name the disease:

Metabolic demyelination where axons are still present

A

Central Pontine Myelinolysis

20
Q

Presentation of Central Pontine Myelinolysis (6)

A
Rapid onset of confusion
Limb weakness
Conjugate gaze palsies
Dysarthria
Dysphagia
Hypotension
21
Q

Name the disease:

Complication of rapidly corrected hyponatremia, low magnesium

A

Central Pontine Myelinolysis

22
Q

Name the disease:

Infectious demyelinating disease

A

Progressive Multifocal Leukoencephalopathy

23
Q

Which of the following about is NOT true about Progressive Multifocal Leukoencephalopathy?
A. Fuzzy, indistinct borders
B. Biral inclusions in oligodendroglia nuclei
C. Perivascular inflammatory infiltrate
D. Atypical, bizarre astrocytes
E. Soft, irregular white matter, granular

A

C. Not enough immune cells in immunocompromised

24
Q

Which of the following about Leukodystrophy is TRUE?
A. Genetic disorder affecting gray matter
B. Autosomal dominant
C. Most often in adults
D. Inability to create or maintain myelin

A

D

A. Affecting white matter
B. AR, some X-linked
C. Children

25
Q

T/F: Leukodystrophy progresses in frontal to occipital manner.

A

False: Occipital to frontal manner

Sparing U fibers

26
Q

Leukodystrophy presentation: behavior changes, psychosis, spasticity in what age range?

A

Adulthood

27
Q

Leukodystrophy presentation: motor disabilities, macrocephaly, seizures or spasticity, developmental failure in what age range?

A

Infancy

28
Q

Leukodystrophy presentation: ataxia, vision changes, behavioral/educational issues in what age range?

A

Childhood

29
Q
Which of the following is NOT a general principle of toxic and metabolic disease? 
A. Bilateral symmetry
B. Systemic effects beyond NS
C. Rare cause of neurologic illness
D. Selective neuroanatomic vulnerability
A

C. Common cause of neurologic illness

30
Q

Wernicke’s encephalopathy and Korsakoff’s syndrome is caused by ____ deficiency

A

Thiamine (Vit. B1)

31
Q
Which of the follow is NOT a presentation of Wernicke's encephalopathy?
A. Abrupt onset of confusion
B. Gaze palsy
C. Ataxia 
D. Irreversible memory disturbances
E. Apathy
F. Clouding of consciousness
A

D. This is a feature of Korsakoff’s syndrome

32
Q

Korsakoff’s syndrome pathology located in ___

A

thalamus

33
Q

Wernicke’s encephalopathy pathology located in ____

A

mamillary bodies, periaqueductal grey, pons, medulla –> Foci of hemorrhage + necrosis

34
Q

Name the disease:
Subacute combined degeneration of spinal cord
Associated w/ pernicious anemia
Causes vacuolar demyelination of long tracts of spinal cord

A

Vitamin B12 Deficiency

Symptoms: Ataxia, LE numbness/tingling

35
Q

CO2 poisoning has a predilection for _____ (2)

A

Globus pallidus

Pars reticulata of substantia nigra

36
Q

In MS, demyelination areas localized around (2)

A

Ventricle (paracortical)

Cortical (juxtacortical)

37
Q

Symptoms of MS occur from ____

A

any of the white matter tracts in the brain and spinal cord

38
Q

T/F: In MS, recovery results from restoration due to remyelination.

A

False. Recovery doesn’t require remyelination. It’s inefficient in CNS.
Redistribution of Na channels mainly contributes to recovery.

39
Q

T/F: MS is diagnosed by CSF analysis

A

False. No single diagnostic test for MS.
• Clinical history
• Blood studies to r/o other possibilities
• CSF analysis consistent w/ MS
• MRI consistent w/ MS and r/o other causes

40
Q

T/F: Progressive MS can be treated with 10 drugs effective toward modulating immune system.

A

False. No drugs treat progressive MS

the 10 drugs are effective for relapsing/remitting MS.