86 Pathology: Demyelinating Diseases + Prions Flashcards
1
Q
Overview
- What is the purpose of myelin within the CNS?
A
2
Q
Overview
- What is the function of an oligodendrocyte?
A
3
Q
Overview
- What kind of disorders are almost all diseases of myelin?
- Why?
A
4
Q
Disease of Myelin
- What are the 2 broad groups of CNS disease that involve myelin?
- Which one is mostly from acquired diseases and which one is mostly from mutations?
A
- Demyelinating Diseases of the CNS
- Acquired
- Improper formation/turnover kinetics
- Genetic
5
Q
MS
- Overview
- What kind of disorder is this labeled as?
- How common is it?
- Is it becoming more or less common?
A
6
Q
MS
- Overview
- When does this present in life?
- Who is affected by it the most?
A
7
Q
MS
- Pathogenesis
- What structure is lesioned in this disease?
- Why?
- What 2 general things are thought to cause this?
- What gene is associated with MS?
- What structure is lesioned in this disease?
A
8
Q
MS
- Pathogenesis
- Who much does a first-degree relative increase your chance for MS?
- What HLA ALLELE is associated with a 3 fold increase in risk?
- Mutations in what interleukin genes are also associated with increased risk?
- What HLA ALLELE is associated with a 3 fold increase in risk?
- Who much does a first-degree relative increase your chance for MS?
A
9
Q
MS
- Pathogenesis
- What kind of cells initiate an attack against myelin?
- Which cells activate macrophages, and how?
- Which cells recruit leukocytes?
- What cells actually cause the demyelination?
- What kind of cells initiate an attack against myelin?
A
10
Q
MS
- Morphology
- Is this multifocal or unifocal disease?
- What do the lesions look like grossly?
- Where are these common? (6)
A
11
Q
MS
- Morphology
- What do the edges of the lesions look like microscopically?
A
12
Q
MS
- Histology
- What cells are contained with active plaques?
- What evidence shows myelin breakdown?
- What other cells are present?
- Are axons present?
- What cells are contained with active plaques?
A
13
Q
MS
- Histology
- What happens to the inflammation when a plaque is inactive?
- What cells are present?
- What happens to the inflammation when a plaque is inactive?
A
14
Q
MS
- Clinical Features: Types
- What is CIS?
- What is RRMS?
- What is SPMS?
- What is PPMS?
- Which of the above is the most common amongst patients?
A
15
Q
MS
- Clinical Features
- Do patients usually recover fully from relapses?
- What are the unilateral visual impairments usually due to?
- What happens when the medial longitudinal fasciculus os interrupted?
- Where would a lesion be located if a patient lost voluntary bladder control?
A
16
Q
MS
- Clinical Features
- What does the CSF of patients show?
- What is shown when the above protein is future examined?
- What does the CSF of patients show?
A
17
Q
MS
- Clinical Features
- What is seen when using an MRI to view an MS patients brain?
- What does this show about the clinical manifestations of some plaques?
A
18
Q
MS
- Clinical Features
- How do the cognitive features differ than other features of MS?
- What is the goal of most current treatments?
A
19
Q
Demyelinating Diseases: Other
- When can immune-mediated demyelinating occur, aside from MS?
- Why is this thought to occur?
A
20
Q
Demyelinating Diseases: Other
- What are the 3 patterns of post-infectious autoimmune reactions to myelin?
- For the not as devasting pattern:
- When do symptoms occur?
- How fast do symptoms progress?
- How many people die?
- For the more devasting one:
- What populations are typically affected?
- For the not as devasting pattern:
A
21
Q
- What is neuromyelitis optica?
- What is central pontine myelinolysis?
- What gets this usually?
- What does it present as?
A
22
Q
Demyelinating Diseases: Other
- Progressive multifocal leukoencephalopathy
- What virus causes this?
- What does it infect?
- When does initially infection usually occur?
- Why does it manifest later in life?
- What is characteristic about the neurological symptoms and signs of this disease?
- What virus causes this?
A
23
Q
Demyelinating Diseases: Other
- Progressive multifocal leukoencephalopathy
- How do the lesions differ than lesions seen in MS?
A
24
Q
Demyelinating Diseases: Other
- Progressive multifocal leukoencephalopathy
- What kind of cell at the center of each lesion?
- What happens to oligodendrocytes at the edges of lesions?
A
25
Demyelinating Diseases: Other
* Subacute Sclerosing Panencephalitis
* What virus causes this?
* Does it progress quickly or slowly?
* What kind of tissue does it infect?
* When are patients usually infected?
* When do neurological signs develop?
* Why is this becoming more common?
26
Leukodystrophies
* How do PTs get this?
* What type of genes are mutated?
* What kind in inheritence pattern do most of these have?
27
Leukodystrophies
* How do these differ in terms of clinical presentation and age of onset, compared to demyelinating diseases?
* What do most of these look like on imaging studies?
28
Leukodystrophies
* What happens to the color and volume of white matter?
* Even though some patches pay present with pathology initially, what will always happen?
* What happens to the brain as these diseases progress?
29
Prion Diseases
* What disease is associated with Papua New Guinea?
* What disease occurs in sheep?
* What is the real name of mad cow disease?
* What disease occurs in deer and elk?
30
Prion Disease
* What is a normal functioning PrP usually rich in?
* What changes to make the PrP resistant to proteolysis?
31
Prion Disease
* What is notable about the stability/longevity of prions?
32
Prion Disease
* Creutzfeldy-Jakob Disease
* What movement disorder usually occurs with dementia?
* What subtle changes occur initially?
* How long does it take until PTs usually die?
* How common is this?
* What age are most individuals who get this?
* What would make these patients get CJD at an earlier age?
33
Prion Disease
* Creutzfeldy-Jakob Disease
* What is the pathognomonic finding in CJD?
* What structures from within the neuropil and sometimes within the perikaryon of neurons?
