Differentiating diagnosis of WML Flashcards

1
Q

What are the 3 areas of differentiating diagnosis of WML?

A
  1. Multiple Sclerosis
  2. Inflammation
  3. Small vessel disease
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2
Q

What is the problem for differentiating diagnosis of MS?

A

No single clinical feature or test is sufficient [clinical or Imaging]

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3
Q

What are the diagnostic criteria for MS?

A
  1. Dissemination in space
  2. Dissemination in time
  3. Reasonable exclusion of alternatives
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4
Q

What are the diagnostic criteria of MS based on?

A

Based on the evolution of the disease of the patients

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5
Q

What does dissemination in space mean?

A

Not always in the same area in the brain

- more than one area is affected

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6
Q

What does dissemination in time mean?

A

The symptoms should not be happening at the same time

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7
Q

Misdiagnosis and Differential Diagnosis

A
  1. Potential differential diagnosis is broad

2. Misdiagnosis is an issue

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8
Q

Why are misdiagnosis an issue?

A
  1. Often common conditions with nonspecific symptoms, signs, MRI findings
  2. NMOSD
  3. Can have harmful consequences
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9
Q

What does NMOSD stand for?

A

Neuromyelitis Optica Spectrum Disorder

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10
Q

Why can misdiagnosis have such harmful consequences?

A

If you misdiagnosis patient, but treat them - expose him to a treatment that is not useful and will not improve and make him worse

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11
Q

Why is over reliance of MRI for neurologist a problem?

A
  1. something is written about WM lesions and that is taken as evidence for MS where the clinical evidence may not be there
  2. As soon as lesions are identified - 4/5 points are made: Vascular, MS, Vasculitis
  3. There are a number of drugs that do not cure but can stop the disease for a certain time
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12
Q

When do we want to treat MS?

A

ASAP
you do not want to treat late where cells have died
[pressure not to miss a window]

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13
Q

What are the MR MS pattern?

A
  1. Location: immediately adjacent PV, within the cortical, below Sub-cortical, Optic nerve, Corpus Callosum and Spinal Cord
  2. Shape: Oval, Dawson’s Finger
  3. Enhancement: First 3-6 weeks
  4. SWI: CVS, Iron deposition
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14
Q

Where are the lesions of MS found?

A

Around the vein (venule)

lesions develop around the vein with vein at its centre

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15
Q

Where are veins located?

A

Perpendicular to the ventricles

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16
Q

What is found adjacent to the cortex?

A

U fibres [ white matter tract that connect one gyri with the other gryi]

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17
Q

What does enhancing and non-enhancing lesions show?

A

They have different age

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18
Q

Where is lesion found in the posterior fossa?

A

Along the internal course [intermedullary course]

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19
Q

What are the 2 cranial nerves of facial colliculus?

A

5th and 6th

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20
Q

What happens in silent lesions?

A

lesions that are not manifesting themselves clinically

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21
Q

What are examples of cortical lesions?

A
  1. Focal demyelinated plaques in the white matter
  2. Cortical demyelination
  3. Demyelinated lesions in the deep grey matter
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22
Q

What imaging modalities are used for Curvilinear lesions?

A
  1. PSIR

2. DIR

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23
Q

What are Central Vein Sign (CVS)?

A

These lesions have vessel in their centre

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24
Q

What Imaging modality is good for CVS?

A

FLAIR

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25
What would typically fit with MS lesion?
A lesion that has a venule in its centre
26
What is SWI good to look for?
Signal change beyond the vessel? | [Intralesional Susceptibility Signal]
27
ISS at 3T
Non-enhancing: 48-50% had a rim (magnetic susceptibility) | Enhancing: 58-60% had a rim
28
SWI in Focal Lesions
Magnetic susceptibility increases rapidly as it changes from enhanced to non-enhanced High susceptibility values during first 2-4 years Then gradually decreases (susceptibility similar to NAWM)
29
What are the features for Spinal cord lesions?
Typical: - unifocal - multifocal Atypical - tumefactive - diffuse
30
What are the pathology for NMO disease?
1. Inflammation 2. Astrocytopathy 3. Myelin relatively preserved, damage secondary
31
What are the diagnosis of NMO?
1. Optic neuritis: initial event in the young 2. Acute myelitis: initial event in the older 3. AQP4 AB: but negative 20-30%
32
What is Aquaporin 4 good for?
channeling water | regulates water going in and out
33
What is Aquaporin 4?
1. Most abundant CNS water channel | 2. Concentrated in astrocytic foot processes
34
What are the features of AQP4 Channelopathy?
1. Female > 80% 2. Non-Caucasian predominance 3. Relapsing if untreated 4. Severe disability and high mortality 5. Associated with other auto-immunity 6. Onset with both ON + TM uncommon
35
What are the features of Myelin Oligodendrocyte Glycoprotein (MOG)-AB disease?
1. Female: Male equal 2. No non-caucasian predomunance 3. ~50% monophasic 4. Better outcome than AQP4-Ab disease 5. Not associated with other auto-immunity 6. Onset with both ON + TM common 7. Overlap with ADEM (monophasic & relapsin)
36
Where is MOG-igG-seropositive more frequent in?
Paediatric patients
37
What is a large proportion found in the NMOSD?
AQP4-Ab disease
38
What are the features of NMO?
SC Lesions: 1. Extent > 3 segments 2. Central grey matter 3. Swelling 4. Partial enhancement 5. Atrophy & Cavity formation
39
What are specific brain lesions of NMO?
1. Medullary periaqueductal grey | 2. Bilat hypothalamus
40
What are the brain lesions features of NMO?
1. Present 60% 2. Location: PV (increase in AQP4 expression) 3. Extensive 4. Multiple, patchy, enhancing (cloud-like enhancement) in 9-% of pat with CE 5. CC: splenium, diffuse, oedematous, heterogenous
41
What is typically found in cloud-like enhancement?
NMO
42
What is not found in cortical lesions?
NMO
43
What differentiates MS from NMOSD?
Multiple lesions at the cut-off of 54% | If more than half of the lesions have a central vein [MS]
44
What are Atypical lesions
1. Finger-like extensions 2. Quite broad 3. Cloud-like enhancement
45
NMO: 2015 diagnostic criteria
1. At least one core clinical characteristic | 2. A positive test for aquaporin
46
Area postrema
Get hiccups
47
MR requirements (AQP4-IgG -ve/unknown)
Acute optic neuritis | > 1/2 ON length or optic chiasm
48
MR requirements (AQP4-IgG -ve/unknown)
Acute myelitis Intramedullary lesions: >3 contiguous segments (LETM) >3 contiguous segments SC atrophy (history of myelitis)
49
MR requirements (AQP4-IgG -ve/unknown)
Acute brainstem syndrome Periependymal brainstem lesions
50
MOG-ab similar to AQP4-ab additionally:
1. conus involvement (isolated sphincter and erectile dysfunction) 2. Fluffy lesions (adults, or ADEM attacks childhood) 3. Bilateral cerebellar peduncle
51
DD from MS
1. > 1 lesion adjacent to body of lat ventricle and in inferior temporal lobe 2. S-shaped U-fibre lesion 3. Dawson's finger-type lesion
52
What are the best classifiers between MOG and MS?
MS: - Ovoid lesions PV (body of lateral ventricles) - Dawson's fingers - T1 hypointense lesions MOG: - Fluffy lesions - 3< lesions (MOG AB)
53
WML
Incidental focal WML > MS prevalence (20-40 years): 1. MS <0.1% 2. Incidental WML 5-10%
54
Small Vessel Disease
Pathology not directly visualised
55
What are MR of Small Vessel Disease?
1. White matter hyperintensities 2. Recent subcortical infarcts (Lacunes) 3. Microhaemorrhages 4. Perivascular spaces 5. Atrophy
56
What is the definition of Small Vessel Disease?
1. Sporadic, intrinsic process, affecting: - Small arteries & arterioles - Capillaries - Small veins Clinically: often silent, developping over years before manifesting clinically
57
Pontine Lesions
1. Symmetric 2. Parallel 3. Affect certain structures - Medial Lemniscus
58
What is CADASIL?
Hereditary small vessel disease
59
What are Lacunes?
Atypical for MS
60
What are the evolution of WMH & Lacunes?
1. WMH appear in vascular end zones 2. Progress proximally along perforating arteries 3. Lacunes appear at WMH edge 4. WMH expand around Lacune
61
Microbleeds
1. Small areas of signal void 2. 2-5mm in diameter, but up to 10mm 3. Associated blooming on T2*-w 4. Perivascular collections of hemosiderin-laden macrophages
62
What does Microbleeds cause?
1. SVD (leakage) 2. Cerebral amyloid angiopathy (CAA) 3. AB amyloid deposition in vessel wall 4. Cortical & leptomeningeal arteries, arterioles (veins)
63
Assistance in DD
Spinal MR: 1. No incidental findings 2. Differential diagnosis of intramedullary lesions - MS: Circumscribed/diffuse changes - SVD: No lesions - CADASIL: No lesions