Demyelination/Demyelinating Disease Flashcards
Demyelination or dysmyelination?
Acquired
Demyelination
Demyelination or dysmyelination?
Abnormal myelin
Dsymyelination
Demyelination or dysmyelination?
Myelin loss w/ relative preservation of axons
Demyelination
Demyelination or dysmyelination?
Remyelination frequently occurs
Demyelination
Demyelination or dysmyelination?
Genetic
Dysmyelination
Demyelination or dysmyelination?
Axons can undergo degeneration
Dysmyelination
Most common demyelinating disorder
Multiple Sclerosis (MS)
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
Multiple Sclerosis is caused mostly by ___
autoimmune destruction by CD4 T cells (TH1, TH17)
Also CD8 T cells, macrophages, B cells
Luxol Fast Blue stains for ___
myelin
Bielschowsky stains for ___
axons
T/F: MS on imaging present and non-enhancing areas in white matter
False. Enhancing
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
Name the disease:
Inflammatory demyelinating disease centered on optic nerve and spinal cord
Neuromyelitis Optica (NMO)
Name the disease:
Antibody mediated by anti-aquaporin-4 antibodies
Neuromyelitis Optica
Name the disease:
Postinfection autoimmune reaction to myelin
Acute Disseminated Encephalomyelitis (ADEM)
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
D
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.
