new department of radiology, university of washington · 2017. 9. 7. · 4/2/2015 1 white matter,...

4/2/2015

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White Matter, Metabolic,

and Degenerative Disease

UW Radiology Review 2015

Dean Shibata, M.DNeuroradiology

Department of Radiology,

University of Washington

• MS (Multiple Sclerosis)

• ADEM (Acute DisseminatedEncephalomyelitis)

• CPM (Central Pontine Myelinolysis)

• PRES (Posterior Reversible Encephalopathy Syndrome)

• PML (Progressive Multifocal Leukoencephalopathy)

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WM Dz: Classific by Mechanism

• Primary demyelinating disease¯ Multiple Sclerosis

• Secondary demyelinating disease¯ Allergic

¯ Viral

¯ Vascular

¯ Metabolic

¯ Toxic


• Allergic (immunologic)¯ Acute disseminated encephalomyelitis - ADEM

• Viral¯ HIV-associated encephalitis

¯ Prog multifocal leukoencephalopathy - PML

¯ Subacute sclerosing Panencephalitis y – SSPE

• Vascular¯ Binswanger’s disease – (WM sml vessel dz)

¯ Postanoxic encephalopathy

¯ Toxic

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• Toxic¯ Radiation

¯ Marchiafava-Bignami Disease (Corpus Collosum)

¯ Disseminated necrotizing leukoencephalopathy

¯ Drugs (chemoRx, metamphetamine, cocaine)

¯ Toxins (triethyl, tin, lead)

• Metabolic¯ central pontine myelinolysis (osmotic) - CPM

• Traumatic: Diffuse axonal shear injury - DAI


• Dysmyelinating Disease¯ Adrenoleukodystrophy – Males, posterior,

Pons/Medulla, Gad+

¯ Metachromatic Leukodystrophy (MLD) –cerebellum, spares subcortical U fibers and BG

¯ Krabbe’s Disease – Ca++ BG

¯ Alexander Disease – Big head, Anterior

¯ Canavan’s Disease – Big head, NAA, stem/BG

¯ Pelizaeus-Merzbacher Disease (PMD) – tigroid WM

¯ Toxins (solvents, tin, lead)

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Metachromatic leukodystrophy

Cheon J et al. Radiographics 2002;22:461-476©2002 by Radiological Society of North America

. Tigroid WM

(AlsoPelizaeus-Merzbacher)

Metachromatic leukodystrophy


Leopard Skin WM

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Hallervorden-SpatzPantothenate kinase-associated neurodegeneration (PKAN)

©2002 by Radiological Society of North AmericaGuillerman R P Radiology 2000;217:895-896

Canavan’sBarkovich 1995

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Which one is Adrenoleukodystrophy?

1 2

Adrenoleukodystrophy?

1. Post Dz

2. Ant Dz

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ALD Alexander

WM Small Vessel Dz

• Risk factors¯ Hypertension¯ APOE4 ¯ Responds to Rx

• Predicts future clinical strokes

• Correlates with cognitive decline

• Clinical Reporting variable

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Clinical Correlations: Risk for Stroke

WMH grade

Strokes/1000 Person Years (Number at Risk)

MRI Infarct absent MRI Infarct present

<= 1 5.8 (1005) 7.2 (170)

2 9.3 (753) 14.6 (269)

3 17.1 (414) 31.0 (222)

4 28.5 (116) 34.1 (124)

>= 5 26.0 (92) 21.4 (913)

CHS: Kuller LH, et al, Stroke 2004

0

20

40

60

80

100

120

140

160

0 1 2 3 4 5 6 7 8

Nu

mb

er o

f R

epo

rts

White Matter Grade (CHS Standard Scale)

Severity of White Matter Disease in Clinical Reports

Severe

Moderate

Mild

Minimal

None

No Mention

2x

1x (risk for future stroke)

3x

4x4x

4x 4x 4x1x

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1 3

5 7

2 4

6 8

White Matter Dz Standards


0

20

40

60

80

100

120

140

160

0 1 2 3 4 5 6 7 8


Nu

mb

er o

f R

epo

rts

Severe

Moderate

Mild

Minimal

None

No Mention


0

20

40

60

80

100

120

140

160

0 1 2 3 4 5 6 7 8


Nu

mb

er o

f R

epo

rts

Severe

Moderate

Mild

Minimal

None

No Mention

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0%

20%

40%

60%

80%

100%

0 1 2 3 4 5 6 7 8

White Matter Grade

Cli

nic

al R

epo

rt D

escr

ipti

on

Severe

Moderate

Mild

Minimal

None

No Mention


Multiple Sclerosis

• Variants:¯ “Acute” - death in 10 months

¯ Devics = Neuromyelitis optica

¯ Balo’s Disease - Concentric Sclerosis

• 85 % have ovoid perivent lesions (Dawson’s fingers)

• 50 to 90 % w “definite” MS have CC lesions

• 10% of adults have PF lesions

• MS lesions generally lack mass effect

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44 year old numbnesslegs and feet

35 year old R body numbnessR leg weakness

Dawson’s Fingers

Flame shaped periventricular

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21 year old opticneuritis

29 year old troublecontrolling R side body

Target Sign

42 year old scatteredparethesias

41 year old blurredvision

FLAIR Sagittal

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Corpus Collosum

Punched out lesions on T1W

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DDx: Corpus Callosum Lesions

• Tumors: GBM, Lymphoma, Lipoma

• Demyelinating Dz: MS, Marchiafava-Bignami, PML

• Infarct (“rare”, dual arterial supply)

Multiple Sclerosis: Which one is False?

• 1) Most lesions occur at the periventricular white matter and corpus collosum

• 2) Atrophy of the corpus collosum is not seen

• 3) Enhancement of the optic nerves may be seen

• 4) Spinal cord lesions may mimic neoplasm with cord enlargement

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Multiple Sclerosis: which is false?

1. Periventricular white matter and corpus collosum lesions

2. No corpus collosum atrophy

3. Optic nerve enhancement

4. Spinal cord lesions may mimic neoplasm

Tumefactive MS

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Tumefactive MS

PreGad PostGad

24 year old lethargy p

respiratory illness


Which one is Multiple Sclerosis?

1 2

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Which is Multiple Sclerosis?

1. 1

2. 2

24 year old lethargy prespiratory illness


Histoplasmosis MS

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23 year old vagueparethesias

39 year old headaches


1 2


1. 1

2. 2

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23 year old vagueparethesias

39 year old headaches

MS Mets

ADEM: Acute DisseminatedEncephalomyelitis

• “Monophasic” but often variable enhancement

• Relatively little mass effect

• Deep grey (thalami) common

• May have hemorrhage

• Subcortical (ADEM) Vs calloseptal (MS)

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11 year old lethargyhemiparesis

3 year old rapidly ↓mental status

ADEM

ADEM

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5 year old drowsinessand seizure

ADEM

6 year old Bilat armand leg numbness

9 year old 9 days of L hemiparesis

ADEM

3 year old ↓ level ofconciousness

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ADEM: Which one is False?

• 1) It is a viral infection

• 2) It is more common in children than adults

• 3) Optic neuritis can be seen

• 4) Long-term f/u may be need to r/o MS

• 5) Both cerebral and cerebellar WM may be involved

ADEM: Which is False?

1. Viral Infection

2. Children > Adults

3. Optic Neuritis

4. F/U imaging to

R/O MS

5. Cerebral &

Cerebellar WM

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CPM: Central Pontine Myelinolysis

• Transverse pontine fibers most severely involved Vs corticospinal tract

• Extra PM in 50%

• >75% EtOH or hyponatremia correction

• DWI bright early, may enhance

• Variable resolution

• Spastic quadriparesis, pseudobulbar palsy

CPM

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CPM

CPM: Which is NOT in DDX ?

• 1) Pontine Infarct

• 2) Multiple sclerosis

• 3) Lyme Disease

• 4) Brainstem Glioma

• 5) Marchiafava-Bignami Disease

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CPM: Which is not in DDx?

1. Pontine infarct

2. Multiple Sclerosis

3. Lyme Disease

4. Brainstem Glioma

5. Marchiafava-

Bignami Disease

? Diagnosis “Normal”

Dina A. Jacobs et al. Neurology 2003;61:969

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Brain stem disease:

1. CPM

2. Multiple System

Atrophy

3. Wilson’s Disease

4. ADEM

5. Olivary Pontine

Degenerative

Wilsons “Normal”

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Wilson’s Disease

• AKA: Hepatolenticular Degeneration

• Autosomal recessive: Copper accumulation

• Hand weakness, dysarthria, pseudo-Parkinsons

• High T2: Lentiform nuclei and midbrain

• Red nucleus and substania nigra surrounded by high T2 signal

• Midbrain: “Panda sign”

Wilson’s “Gourmet”

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Wilson’s


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Brain stem disease:

1. CPM

2. Multiple System

Atrophy

3. Huntington’s

4. ADEM

5. Progressive

supranuclear palsy

MultisystemAtrophy

“Normal”

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Multisystem Atrophy (MSA)

• Sporadic neurodegenerative disease

• Synucleinopathy: alpha-synuclein metabolism

• Autonomic (Shy-Drager), Striaonigral, Olivopontocerebellar

• High T2: pontocerebellar tracts

• Pons: “Hot Cross Bun sign”

MultisystemAtrophy

“Hot cross bun”

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Brain stem disease:

1. CPM

2. Multiple System

Atrophy

3. Huntington’s

4. Progressive

supranuclear palsy

5. Olivary Pontine

Degenerative

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Progressive supranuclear palsy

“Normal”


“Hummingbirdsign”

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Progressive Supranuclear Palsy (PSP)

• AKA: Steele-Richardson-Olszewski syndrome

• Sporadic neurodegenerative disease

• Cognitive decline, abnormal eye movements

• Midbrain atrophy (“Hummingbird sign”)

• High T2: Pontine tegmentum, midbrain tectum

Reversible Post Leukoencephalopathy Syndrome (RPLS or PRES)

• Most often caused by abrupt changes in blood pressure, seizures, or certain immunosuppressive medications.

• Vasogenic edema predom in WM related to loss of autoregulatory ability

• Overall prognosis is good.

• DWI negative (Vs acute infarct cytotoxic edema)

• Usually in occipital, parietal, and temporal areas

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PRES

48 year old postseizure

2 months later

HypertensiveEncephalopathy

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HypertensiveEncephalopathy

58 yo cardiac Txw Seizure

45 yo w impairedvision

Which one is PRES?

1 2

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Which one is PRES?

1. 1

2. 2

58 yo cardiac Txw Seizure

45 yo w impairedvision

PRES PML

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PML: Progressive Multifocal Leukoencephalopathy

• JC papovavirus

• Most commonly HIV or other Immunocompromised

• Classically: WM “geographic” lesions without enhancement or mass effect

• Formerly endstage HIV w poor prognosis

PML

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PML

PML

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PML: Which is FALSE?

• 1) It is caused by the JC virus

• 2) It is more common in males than females

• 3) CT frequently shows ringlike enhancement

• 4) Predilection for the parietal and occipital lobes

• 5) The spinal cord is rarely involved

PML: Which is False?

1. JC Virus

2. Males > Females

3. Ring Enhances

4. Parietal/Occipital

5. Spinal Cord rarely

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Signs:

• Tigroid WM: Metachromatic leukodystrophy

• Eye of the Tiger: Hallervorden-Spatz (PKAN)

• Panda sign: Wilsons

• Hot Cross Bun: Multiple system atrophy

• Hummingbird Sign: Progressive Supranuclear palsy

Metachromatic leukodystrophy(MLD)


. Tigroid WM

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Hallervorden-SpatzPantothenate kinase-associated neurodegeneration (PKAN)

©2002 by Radiological Society of North AmericaGuillerman R P Radiology 2000;217:895-896


“Hummingbirdsign”

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Wilson’s “Panda”

MultisystemAtrophy

“Hot cross bun”

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CPM

??“Duck face sign”

??“Crocodile sign”

new department of radiology, university of washington · 2017. 9. 7. · 4/2/2015 1 white matter,...

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