demyelinating path

Question 1

MS is not only the MC demyelinating disorder but it is also…

Answer 1

MC chronic CNS disease of young adults in the US

Question 2

Where do MS lesions occur?

Answer 2

periventricular white matter, optic nerves and chiasm, brainstem

Question 3

What stain shows demyelination of MS?

Answer 3

Luxol-fast blue PAS stains myelin

Question 4

What are CSF findings of MS?

Answer 4

• moderate mononuclear pleocytosis
• mildly elevated total CSF protein
• IgG levels are increased and oligoclonal bands are usually found on electrophoreis
• MBP detected

Question 5

What are we looking at?

Answer 5

oligoclonal bands present on the lower part of electrophoresis, like in MS

Question 6

severe idiopathic inflammatory demyelinating disorder of spinal cord and optic nerves/chiasm

Answer 6

neuromyelitis optica

Question 7

Neuromyelitis Optica Spectrum Disorder has ssx like..

Answer 7

• optic neuritis
• transvere myelitis
• intractable vomiting
• pain
• hiccups
• cranial nerve abnormalities

Question 8

What is the primary pathogenesis of neuromyelitis optica?

Answer 8

presence of IgG abs against aquaporin-4 (AQP-4)

Question 9

What is aquaporin-4?

Answer 9

major water channel of astrocytes along the BBB

Question 10

post-infectious, post-vaccination, or allergic encephalomyelitis most commonly affecting children

Answer 10

Acute demyelinating encephalomyelitis (ADEM)

Question 11

What is the pathology behind ADEM?

Answer 11

not infection, but damage due to the T cell immune response triggered by the preceding infection or vaccination

Question 12

We see this in what disease? What is in the image?

Answer 12

ADEM

perivascular lymphocytes - not damaging blood vessels

Question 13

On MRI, what will be a difference between MS and ADEM?

Answer 13

MS will have lesions at different stages while ADEM has lesions at the same stage

Question 14

Weston Hurst disease is aka

Answer 14

Acute necrotizing hemorrhagic encephalomyelitis

Question 15

Similar to ADEM, but Weston Hurst has what unique qualities?

Answer 15

damage is more severe, includes blood vessels

disseminated necrosis of white and gray matter with acute hemorrhage

fibrin deposition and abundant neutrophils

Question 16

JC polyoma virus can cause…

Answer 16

Progressive Multifocal Leukoencephalopathy (PML)

Question 17

Where are the lesions of PML found on MRI?

Answer 17

subcortical white matter

Question 18

What are we looking at?

Answer 18

PML, early lesions on left, later lesions on right

Question 19

In PML, there is no …. or disease of…

Answer 19

peripheral nerve demyelination or disease of any other organ system

Question 20

What are we looking at?

Answer 20

central pontine myelinolysis

myelin loss without evidence of inflammation

neurons and axons are well preserved

all lesions appear at the same stage of myelin loss and reaction

Question 21

When does central pontine myelinolysis usually occur?

Answer 21

MC arises 2-6 days after rapid correction of hyponatremia

Question 22

What is the clinical presentation of central pontine myelinolysis?

Answer 22

locked in syndrome