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
Q

What would typically fit with MS lesion?

A

A lesion that has a venule in its centre

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26
Q

What is SWI good to look for?

A

Signal change beyond the vessel?

[Intralesional Susceptibility Signal]

27
Q

ISS at 3T

A

Non-enhancing: 48-50% had a rim (magnetic susceptibility)

Enhancing: 58-60% had a rim

28
Q

SWI in Focal Lesions

A

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
Q

What are the features for Spinal cord lesions?

A

Typical:

  • unifocal
  • multifocal

Atypical

  • tumefactive
  • diffuse
30
Q

What are the pathology for NMO disease?

A
  1. Inflammation
  2. Astrocytopathy
  3. Myelin relatively preserved, damage secondary
31
Q

What are the diagnosis of NMO?

A
  1. Optic neuritis: initial event in the young
  2. Acute myelitis: initial event in the older
  3. AQP4 AB: but negative 20-30%
32
Q

What is Aquaporin 4 good for?

A

channeling water

regulates water going in and out

33
Q

What is Aquaporin 4?

A
  1. Most abundant CNS water channel

2. Concentrated in astrocytic foot processes

34
Q

What are the features of AQP4 Channelopathy?

A
  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
Q

What are the features of Myelin Oligodendrocyte Glycoprotein (MOG)-AB disease?

A
  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
Q

Where is MOG-igG-seropositive more frequent in?

A

Paediatric patients

37
Q

What is a large proportion found in the NMOSD?

A

AQP4-Ab disease

38
Q

What are the features of NMO?

A

SC Lesions:

  1. Extent > 3 segments
  2. Central grey matter
  3. Swelling
  4. Partial enhancement
  5. Atrophy & Cavity formation
39
Q

What are specific brain lesions of NMO?

A
  1. Medullary periaqueductal grey

2. Bilat hypothalamus

40
Q

What are the brain lesions features of NMO?

A
  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
Q

What is typically found in cloud-like enhancement?

A

NMO

42
Q

What is not found in cortical lesions?

A

NMO

43
Q

What differentiates MS from NMOSD?

A

Multiple lesions at the cut-off of 54%

If more than half of the lesions have a central vein [MS]

44
Q

What are Atypical lesions

A
  1. Finger-like extensions
  2. Quite broad
  3. Cloud-like enhancement
45
Q

NMO: 2015 diagnostic criteria

A
  1. At least one core clinical characteristic

2. A positive test for aquaporin

46
Q

Area postrema

A

Get hiccups

47
Q

MR requirements (AQP4-IgG -ve/unknown)

A

Acute optic neuritis

> 1/2 ON length or optic chiasm

48
Q

MR requirements (AQP4-IgG -ve/unknown)

A

Acute myelitis

Intramedullary lesions:
>3 contiguous segments (LETM)
>3 contiguous segments SC atrophy (history of myelitis)

49
Q

MR requirements (AQP4-IgG -ve/unknown)

A

Acute brainstem syndrome

Periependymal brainstem lesions

50
Q

MOG-ab similar to AQP4-ab additionally:

A
  1. conus involvement (isolated sphincter and erectile dysfunction)
  2. Fluffy lesions (adults, or ADEM attacks childhood)
  3. Bilateral cerebellar peduncle
51
Q

DD from MS

A
  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
Q

What are the best classifiers between MOG and MS?

A

MS:

  • Ovoid lesions PV (body of lateral ventricles)
  • Dawson’s fingers
  • T1 hypointense lesions

MOG:

  • Fluffy lesions
  • 3< lesions (MOG AB)
53
Q

WML

A

Incidental focal WML > MS
prevalence (20-40 years):
1. MS <0.1%
2. Incidental WML 5-10%

54
Q

Small Vessel Disease

A

Pathology not directly visualised

55
Q

What are MR of Small Vessel Disease?

A
  1. White matter hyperintensities
  2. Recent subcortical infarcts (Lacunes)
  3. Microhaemorrhages
  4. Perivascular spaces
  5. Atrophy
56
Q

What is the definition of Small Vessel Disease?

A
  1. Sporadic, intrinsic process, affecting:
    - Small arteries & arterioles
    - Capillaries
    - Small veins

Clinically: often silent, developping over years before manifesting clinically

57
Q

Pontine Lesions

A
  1. Symmetric
  2. Parallel
  3. Affect certain structures - Medial Lemniscus
58
Q

What is CADASIL?

A

Hereditary small vessel disease

59
Q

What are Lacunes?

A

Atypical for MS

60
Q

What are the evolution of WMH & Lacunes?

A
  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
Q

Microbleeds

A
  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
Q

What does Microbleeds cause?

A
  1. SVD (leakage)
  2. Cerebral amyloid angiopathy (CAA)
  3. AB amyloid deposition in vessel wall
  4. Cortical & leptomeningeal arteries, arterioles (veins)
63
Q

Assistance in DD

A

Spinal MR:

  1. No incidental findings
  2. Differential diagnosis of intramedullary lesions
    - MS: Circumscribed/diffuse changes
    - SVD: No lesions
    - CADASIL: No lesions