Multiple Sclerosis Pathology

What is MS?

Autoimmune inflammatory demyelinating disease of the CNS, causing progressive neurodegeneration

There are various sub-types of MS; list 5 of these.

1) Acute MS (Marburg's Disease)
2) Relapsing-remitting
3) Progressive relapsing
4) Secondary progressive (no replases)
5) Primary progressive (later onset)
6) Benign (mild w/ no conversion)

Which of these is the most common and what its average duration?

Relapsing-remitting, accounts for roughly 80%. Duration roughly 10 years. 80% in turn of these will go on to secondary progression

What is the essential lesion in MS?

A demyelinated plaque; identified at any site where myelin sheaths are present

Why are symptoms and the disease course so varied between individuals with MS?

The amount and location of damage to the nervous system is different in each person with MS; particular symptoms may be caused by damage in different parts of a system

True or false: extensive lesions in MS often follow the lateral ventricles

True

True or false: lesions are uncommon at the substantia nigra

False; they are common here, and also at many levels of the pyramidal tracts, leading to severe motor symptoms. Also common in pons.

True or false: lesions in MS may occur at any point along the spinal cord axis

True

Why might spinal cord lesions often appear shrunken?

Due to loss of axons as well as myelin sheaths

True or false: lesions in the spinal cord affect only the white matter and follow a predictable pattern

False; they can affect both grey and white matter, and do not follow a pattern.

True or false: perivascular immune cell infiltration is observed in MS

True - CD4/8+ T-cells and CD20 B-cells are seen.

True or false: the demyelination in MS is largely B-cell mediated

False; it is largely macrophage-mediated

How can actively demyelinating lesions be identified?

By the presence of macrophages containing myelin debris

True or false: inflammatory mediators release free radicals and cytotoxic mediators that create a reversible block of electrical signals as well as demyelination

True; this results in transient symptoms. Immunomodulatory therapies aim to stop these effects

List 3 mechanisms of inflammatory neuronal dysfunction observed in MS

1) Release of free radicals by peripheral immune cells/activated microglia
2) Glutamate released by microglia; excitotoxicity
3) Cytotoxic cytokine release by immune cells/microglia (TNF, IL-1B, IFN-gamma)
4) Mitochondrial dysfunction

What is the difference between the borders of chronic silent lesions and active lesions?

Chronic silent lesions have well-defined borders; those of active lesions are more ragged

True or false: demyelinated lesions are characterised by a lack of oligodendrocytes but relative preservation of axons

True

True or false: inflammation leads to more long-term symptoms than demyelination

False; rather it causes the transient reversible symptoms, with demyelination causing long-term symptoms

True or false; MRI lesion load and rate of appearance of new lesions correlates well with clinical progression

False; it correlates poorly

Disease progression correlates well with atrophy of what?

Grey matter atrophy

True or false: grey matter demyelination is extensive and often covers a greater area than white matter demyelination

True

True or false: cortical demyelination is associated with meningeal inflammation

True

What structures are found in the meninges of MS cases with more active and shorter progression?

Tertiary lymphoid-like structures