NP4F

Question 1

Inflammatory _________________is a key piece of evidence supporting autoimmunity

Answer 1

primary demyelination

Question 2

All ___________________ diseases are ______________, but not all ________________ disease is ___________

Answer 2

autoimmune, immune mediated
immune mediated, autoimmune

Question 3

is simply the immune system’s response to (what is perceived as) harmful stimuli

Answer 3

inflammation

Question 4

is simply disease driven by the inflammatory response, and if that inflammation is actually caused by the disease

Answer 4

immune-mediated

Question 5

is a subset of immune-mediated disease where we know exactly the antigenic target that is driving the process, and so these generally are the inflammatory demyelinations

Answer 5

autoimmune disease

Question 6

Microbial agents may also express proteins that are similar to those of the host

Answer 6

molecular mimicry

Question 7

An ____________ against the viral/bacterial antigens may thus destroy infected as well as uninfected cells. Once the infectious agent is eliminated, the host antigen may provide sufficient stimulation to sustain the inflammation

Answer 7

immune response

Question 8

Caprine Arthritis Encephalitis virus :
Sustained activation of microglia (the result of viral persistent infection) drives an autoimmune reaction against myelin. The result is a highly inflammatory primary demyelination in both the __________ and _______.

Answer 8

brain and spinal cord

Question 9

What if there is no microorganism associated with the lesions?

Answer 9

• the trigger (the event precipitating the autoimmune reaction) is hard to track down
• A previous infection (or infections) causing nervous system inflammation is often proposed

Question 10

Autoimmunity can affect the peripheral nervous system as well, giving rise to an inflammatory demyelinating polyneuropathy. Classic example is:

Answer 10

cauda equine neuritis

Question 11

Where inflammatory responses are localized to nerve roots of the cauda equina

Answer 11

equine cauda equine neuritis

Question 12

This disease illustrates features in common with many inflammatory demyelinating polyneuropathies

Answer 12

cauda equine neuritis (inflammatory demyelinating polyneuropathy)

Question 13

cauda equine neuritis:
There is no infectious agent directly associated with the active disease, although _______________ are thought to trigger an immune response with a strong autoimmune component, hence the term “post infectious allergic neuritis”

Answer 13

prior infections

Question 14

Here, the antibody is directed against acetylcholine ion channel receptors in the neuromuscular junction, causing periodic weakness that is associated with exercise. There are no cellular infiltrates at the neuromuscular junction, only the antibody. We don’t know what initiates the production of these antibodies. A presumptive diagnosis can be based upon clinical response to acetylcholinesterase inhibitors.

Answer 14

myasthenia gravis

Question 15

The acquired form of _____________ is another example of an autoimmune reaction in the peripheral nervous system

Answer 15

myasthenia gravis

Question 16

This is a clinical term used to describe an immune-mediated process where a cause (e.g., infectious agent) is not identified.

Answer 16

Meningoencephalitis of unknown etiology (MUE)

Question 17

Inflammation is localized to the meninges and WM of the forebrain, with large perivascular cuffs of monocytes and lymphocytes. There is no necrosis or demyelination in the inflamed tissues. The problem is edema.

Answer 17

Granulomatous meningoencephalitis (GME)

Question 18

Necrosis is the distinguishing characteristic of these disorders. These two “entities” overlap, with NME primary affecting GM and NLE affecting white matter.

Answer 18

Necrotizing meningoencephalitis (NME) and necrotizing leukoencephalitis (NLE)

Question 19

primary affecting grey matter

Answer 19

Necrotizing meningoencephalitis (NME)

Question 20

affecting white matter

Answer 20

necrotizing leukoencephalitis (NLE)

Question 21

Infectious agents are not directly associated with the lesions, although prior exposure to an infectious agent may be key to the pathogenesis

Answer 21

chronic inflammatory polyneuropathy