Demyelination/Demyelinating Disease Flashcards by Shirley Yancey

Demyelination or dysmyelination?

Acquired

Demyelination

How well did you know this?

Not at all

Perfectly

Demyelination or dysmyelination?

Abnormal myelin

Dsymyelination

How well did you know this?

Not at all

Perfectly

Demyelination or dysmyelination?

Myelin loss w/ relative preservation of axons

Demyelination

How well did you know this?

Not at all

Perfectly

Demyelination or dysmyelination?

Remyelination frequently occurs

Demyelination

How well did you know this?

Not at all

Perfectly

Demyelination or dysmyelination?

Genetic

Dysmyelination

How well did you know this?

Not at all

Perfectly

Demyelination or dysmyelination?

Axons can undergo degeneration

Dysmyelination

How well did you know this?

Not at all

Perfectly

Most common demyelinating disorder

Multiple Sclerosis (MS)

How well did you know this?

Not at all

Perfectly

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

B. Frequent relapses

Infrequent relapses characteristic of secondary-progressive phase

How well did you know this?

Not at all

Perfectly

Multiple Sclerosis is caused mostly by ___

autoimmune destruction by CD4 T cells (TH1, TH17)

Also CD8 T cells, macrophages, B cells

How well did you know this?

Not at all

Perfectly

Luxol Fast Blue stains for ___

myelin

How well did you know this?

Not at all

Perfectly

Bielschowsky stains for ___

axons

How well did you know this?

Not at all

Perfectly

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

False. Enhancing

How well did you know this?

Not at all

Perfectly

MS presents grossly as (2)

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

How well did you know this?

Not at all

Perfectly

Name the disease:

Inflammatory demyelinating disease centered on optic nerve and spinal cord

Neuromyelitis Optica (NMO)

How well did you know this?

Not at all

Perfectly

Name the disease:

Antibody mediated by anti-aquaporin-4 antibodies

Neuromyelitis Optica

How well did you know this?

Not at all

Perfectly

Name the disease:

Postinfection autoimmune reaction to myelin

Acute Disseminated Encephalomyelitis (ADEM)

How well did you know this?

Not at all

Perfectly

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

False. Plaques are veins ONLY

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. NO large sharply demarcated plaques

Name the disease:

Metabolic demyelination where axons are still present

Central Pontine Myelinolysis

Presentation of Central Pontine Myelinolysis (6)

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

Name the disease:

Complication of rapidly corrected hyponatremia, low magnesium

Central Pontine Myelinolysis

Name the disease:

Infectious demyelinating disease

Progressive Multifocal Leukoencephalopathy

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

C. Not enough immune cells in immunocompromised

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

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

False: Occipital to frontal manner | Sparing U fibers

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

Adulthood

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

Infancy

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

Childhood

``` 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 ```

C. Common cause of neurologic illness

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

Thiamine (Vit. B1)

``` 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 ```

D. This is a feature of Korsakoff's syndrome

Korsakoff's syndrome pathology located in ___

thalamus

Wernicke's encephalopathy pathology located in ____

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

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

Vitamin B12 Deficiency Symptoms: Ataxia, LE numbness/tingling

CO2 poisoning has a predilection for _____ (2)

Globus pallidus | Pars reticulata of substantia nigra

In MS, demyelination areas localized around (2)

Ventricle (paracortical) | Cortical (juxtacortical)

Symptoms of MS occur from ____

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

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

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

T/F: MS is diagnosed by CSF analysis

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

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

False. No drugs treat progressive MS | the 10 drugs are effective for relapsing/remitting MS.