MS, SVD - Differential diagnosis

Question 1

What is the diagnostic criteria for MS?

Answer 1

Dissemination in time and space. Reasonable exclusion of alternatives.

Question 2

What disease is a an example of misdiagnosis in MS?

Answer 2

NMOSD.

Question 3

What are some things you should look at before making a clinical diagnosis?

Answer 3

age, signs, symptoms, CSF

Question 4

What will you see on MR for MS?

Answer 4

pattern - PV, cortical, Sub-c, ON, CC, spinal cord
shape - oval, Dawson's finger
enhancement
SWI - hypointensity, MCB, central vein
anything unsuual - lacunes, VR spaces

Question 5

What is the location of MS on MR?

Answer 5

adjacent periventricular, cortical, sub-c, ON, CC, spinal cord

Question 6

What can you see on SWI for MS?

Answer 6

can see vessel or vein. CVS (central vein sign - lesion has venual in centre), iron deposition

Question 7

What locations can we have on posterior fossa for MS?

Answer 7

facial colliculus -  nerve 5 and 6
middle cereberlar peduncle
white matter
5th nerve trigemnial
intramedullary

Question 8

Where are cortical lesions as seen from pathology in RRMS, SPMS, PPMS

Answer 8

focal demyelinated plaques in the WM
cortical demyelination
demyelinated lesions in the deep GM

Question 9

How much enhancing does SWI, intralesional susceptibility signal (ISS) show at 3T for MS? What does it represent?

Answer 9

non-enhancing - 48%
enhancing - 58%
represents iron-rich macrophages/microglia. myelin loss also contributes

Question 10

What does SWI show in focal lesions over time?

Answer 10

As lesion develops, will enhance for 3-6 weeks then will go
Low signal- dark rim will happen quick then slowly increase then slowly decrease after 2-3 years.
Magnetic susceptibility increases rapidly as it changes from enhanced to non-enhanced. High susceptibility values during first 2-4 years. Then gradually decreases.

Question 11

What is typical to see on imaging on the spiral cord for MS? What is atypical?

Answer 11

unifocal/multifocal

tumefactive disease

Question 12

What is the pathology for NMO?

Answer 12

Inflammation
Astrocytopathy - affects astrocides
Myelin relatively preserved - damaged in secondary phase

Question 13

What are diagnosis for older and younger people for NMO?

Answer 13

Optic neuritis - initial event in young
Acute myelities - intial event in the older
measure body against AQP4 AB.

Question 14

What is AQP4 AB? How many people is it negative in? Why is it important?

Answer 14

Negative in 20-30%. most abundant CNS channel. concentrated in astrocytic foot processes. Important to channel water as regulates water going in and out.

Question 15

What people is AQP4 channelopathy seen in? what happens with it?

Answer 15

Female > 80%
non-Caucasian predominace
relapsing if untreated
Severe disability and high mortality
Associated with other auto-immunity
onset with both ON and TM uncommon
10% monophasic
90% relapsing

Question 16

What people are Myelin Oligodendrocyte Glycoprotein (MOG)-AB disease seen in and what can happen?

Answer 16

female-male equal
no non-caucasian predominance
50% monophasic
onset with both ON and TM common
overlap with ADEM (monophasic and relapsing)

Question 17

Where/what are subcortical lesions seen on NMO?

Answer 17

see on more than 3 segments
central GM
swelling
partial enhancement
atrophy and cavity information (volume loss in cavity)

Question 18

Where are specific brain lesions in NMO?

Answer 18

Medullary periaqueductal grey

Bilat hypothalamus

Question 19

What does brain lesions looks like in NMO and where seen?

Answer 19

60% present
located in periventricular (aqp4 positive?)
extensive
multiple, patchy, enhancing (cloud like - dots of different sizes) in 90& of pat with CE
in more diffused way in corpus callosum - splenium, oedematous, heterogenous

Question 20

How much % is cloud like enhancement in NMO and MS?

Answer 20

NMO - 90%

MS - 8%

Question 21

How much % is cortical lesions in NMO/MS?

Answer 21

NMO - 0

MS - 67%

Question 22

What is the diagnostic criteria (2015) for NMO (including for NMOSD with/without AQP4)?

Answer 22

with - positive test and 1 clinical characteristic
without - 2 core clinical characteristics, dissemination in space, negative test
clinical characteristics - optic neuritis, acute myelitis, acute brainstem syndrome etc.

Question 23

What are MR requirments for NMO when AQP4-IgG is negative/unknown - acute optic neuritis?

Answer 23

brain is normal.unspecific

optic nerve > 1/2 length or optic chiasm

Question 24

What are MR requirments for NMO when AQP4-IgG is negative/unknown - acute myelitis?

Answer 24

intramedullary lesions

> 3 contiguous segments SC atrophy

Question 25

What are MR requirments for NMO when AQP4-IgG is negative/unknown - area postrema syndrome?

Answer 25

dorsal medullar/ area postrema lesions

Question 26

What are MR requirments for NMO when AQP4-IgG is negative/unknown - acute brainstem syndrome?

Answer 26

periependymal brainstem lesions

Question 27

What is similar in AQP4-ab and MOG-ab

Answer 27

``` conus involvement(isolalated sphincter and erectile dysfunction) fluffy lesions (adults or ADEM attacks childhood) bilateral cerebellar peduncle ```

Question 28

What is differential diagnosis for MS?

Answer 28

>1 ovoid lesions PV adjacent to body of lateral ventricle and in inferior temporal lobe S-shaped u-fibre lesions T1 hypointense lesions Dawson's finger-type lesion

Question 29

What is DD for MOG?

Answer 29

Fluffy lesions | 3< lesions (MOG AB)

Question 30

What's more common than MS?

Answer 30

Incidental focal WML - not related to any disease 5-10% in 20-40 years

Question 31

What is seen on MR for SVD?

Answer 31

``` wm hyperintensities recent subcortical infarcts lacunes microhaemorrhages enlargement of perivascular spaces atrophy - volume loss ```

Question 32

What is seen in MR or white matter hyperintensities of presumed vascular orign - SVD

Answer 32

signal abnormality of variable size in WM T2/FLAIR hyperintensity, without cavitation lesions in subcortical GM or brainstem not included

Question 33

what is affected in SVD?

Answer 33

small arteries and arterioles capillaries small veins often silent, develops over years before manifesting clincally

Question 34

what is the appearance for WM hyperintensities on PV and SC?

Answer 34

heterogeneous PV - caps, pencil-thin lining, halo SC - punctuate, early/confluent

Question 35

If U-fibres are spared in MS, what might the disease be?

Answer 35

between cortex and white matter there is a black band always white matter in between so suspicious of ms typical to involve u fibres if spares u fibres might be small vessel disease

Question 36

if there are pontine lesions, is it MS

Answer 36

in medial lemiscus symmetrical not MS

Question 37

if temporal lobe is spared, is it MS

Answer 37

no | maybe cadasil if not around anterior temporal lobe

Question 38

does CADASIL(Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, usually called CADASIL, is an inherited condition that causes stroke and other impairments. This condition affects blood flow in small blood vessels, particularly cerebral vessels within the brain) involve ant temporal pole

Answer 38

no | could be SVD

Question 39

explain the evolution of wmh and lacunes

Answer 39

WMH appear in vascular end zones progress proximally along perforating arteries lacunes appear at WMH edge WMH expand around lacune

Question 40

where can you see CADASIL

Answer 40

subcortical lacunar lesions

Question 41

what do you see on perivascular spaces, imaging shape, size

Answer 41

fluid-filled spaces, follow typical course of a vessel, in GM/WM, linear when imaged parallel round or avoid, when imaged perpendicular to the course of the vessel < 3 mm

Question 42

what are size of microbleeds seen on what imaging

Answer 42

small areas of signal void 2-5 mm in diameter but up to 10mm associated blooming on T2*

Question 43

causes of microbleeds?

Answer 43

SVD (leakage) cerebral amyloid angiopathy (CAA) AB amyloid deposition in vessel wall cortical and leptomeningeal arteries, arterioles

Question 44

What is distribution of SVD and CAA

Answer 44

svd - lobar and deep | caa - lobar

Question 45

what else is seen on spinal MR with DD of intramedullary lesions on MS/SVD/CADASIL?

Answer 45

MS - diffuse changes | svd/cadasil - no lesions

Question 46

are microbleeds on t2* in ms/svd/cadasil

Answer 46

absent in MS | Present in svd/cadasil

Question 47

what % is central vein sign in ms and migraine

Answer 47

ms - 84% | migraine - 22%

Question 48

What is DD of MS

Answer 48

subcortical, periventriular, PF, curvilinear, central vein sign, ISS, spinal cord

Question 49

what is DD of NMOSD

Answer 49

optic nerve, spinal cord, fluffy, cloud, conus (anti mog), no CVS, no ISS

Question 50

what is DD of SVD

Answer 50

``` no subcortical no spinal cord pons PVS lacunes MCB no CVS no ISS ```