Term
What is the Classic neuropathological presentation of Multiple Sclerosis?
What is meant that some MS patients are relapse-remitting? |
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Definition
Demyelinating Plaques: Multiple, peri-ventricular, firm tissue lesions
The result of gliosis
(diagnose MS based on this and clinical episodes)
relapsing-remitting = bouts of demyelination followed by partial to full repair |
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Term
Describe the neuropath presentation of 'Dawson's Finger'
What is the line of Gennari? |
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Definition
Dawson's Finger = MS lesions around the ventricular-based brain veins; they are the result of A. inflammation or B. BP-related mechanical damage;
Result: 'fingers' spread along the large periventricular collecting veins
Line of Gennari = only area of myelination within the calcarine fissure of the visual cortex |
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Term
MS Epidemiology
Male/Female ratio?
Race ratio?
Age of onset?
Geographic location?
Associated diseases? (nonspecific) |
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Definition
1.8x more females affected
2x as many whites affected as blacks
Age: 20-50
Much higher incidence along a particular latitude for some reason; Migration effect:
If you leave a high prevalence area before age 15, you dont bring the risk with you; same deal, if you move to a high prevalence area before age 15, you will possess the increased risk of your new home
Other autoimmunity diseases associated |
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Term
| Histopath of demyelination |
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Definition
| W/ stain for myelin, you will see due to the lack of blue that myelin is lost, instead see pink-stained histiocytes digesting the myelin; neurons clearly remain intact |
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Term
Myelitis = ?
Devic's Disease: aka?
Give 3 symptoms |
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Definition
myelitis = disease of the spinal cord
Devic's, aka Neuromyelitis Optica, involves:
- Staggering
- Trouble controlling sphincters
- Eye problems
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Term
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Definition
| Shadow Plaque: This is often seen in acute MS and is characterized by incomplete demyelination. Some studies suggest that this type of plaque may actually be an area of remyelination. |
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Term
Axonal Transection in MS Lesions
What is it, how do you diagnose it, and how does this affect the patient prognosis? |
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Definition
Generally, MS is characterized solely by myelin loss w/ axonal preservation, but some patients have some axon involvement
If patient has an episode and show no improvement/recovery after a few months, axon transection is most likely invovled
Recovery is generally worse in these patients |
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Term
Adrenoleukodystrophy
What's wrong in these patients?
Age of onset?
Inheritance? |
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Definition
Demyelinating disease: Patients cannot digest very long chain fatty acids, and have no source to produce myelin
Onset at age 7-10
X-linked, much more prevalent in boys |
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Term
Acute demyelination - what may this be misdiagnosed as on a brain scan (and what is actually shown)?
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Definition
Looks like a tumor, but its actually an area of rapid demyelination filled w/ histiocytes
Reactive astrocytes also seen, spider like appearance |
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Term
| Balo's concentric sclerosis |
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Definition
| Basically MS, but the tissue is demyelinated in concentric layers; it could either be selective demyelination, or more generalized demyelination w/ specific regeneration |
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Term
Acute Disseminated Encephalitis
Can occur following what 2 events?
3 physiologic symptoms?
Prognosis? |
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Definition
Seen following A. viral infections or B. post-vaccination for rabies
Symptoms:
Cerebral Edema
Peri-venous loss of myelin
perivascular inflammatory infiltrate
Patients either die or survive w/ significant neuro defecits |
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Term
Acute Necrotizing Hemorrhagic Leukoencephalitis
It pops up following what previous clinical event?
3 symptoms
Course/Prognosis? |
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Definition
follows a non-specific respiratory infection
symptoms:
Cerebral edema
Petechiae (multiple hemorrhagic lesions) in white matter
Fibrinoid degeneration of small vessels w/ perivascular hemmorhaging
Rapid progression, often fatal |
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Term
Cerebral Pontine Myelinolisis
What is it, and is assoc w/ what clinical/physiologic event?
How do you avoid this? |
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Definition
= loss of myelin in pons
This happens when someone's Na is low and you infuse them w/ Na and BP dramatically increases (rapid sodium correction)
If someone is hypOnatremic, you can correct it, but do it slowly! |
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Term
Progressive Multifocal Leukoencephalopathy (PML)
Explain what this name tells u about the disease.
Cause?
Gross path appearance? |
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Definition
It is a disease featuring multiple scattered lesions affecting white matter w/ neuro symptoms that worsen over time (gray matter usually spared)
Caused by opportunistic viral inf (usually seen in HIV patients)
Lesions are highly granular, shiny |
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Term
Example of a virus that can cause PML; what other affect on the NS does this virus cause?
Where are PML inclusions located in the CNS, and what is a special functional affect of this? |
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Definition
JC virus
also causes oncogenic transformation of astrocytes
PML inclusions found in oligodendrocytes, many axons are affected by only 1 oligodendrocyte becoming infected |
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Term
| Marchiava-Bignami Disease |
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Definition
Myelin loss, esp at corpus callosum
Get this from drinking cheap/rapidly produced itialian wine, metabolic basis |
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