white matter diseases - inova presentations an… · dissemination in space: 1. periventricular ......
TRANSCRIPT
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White Matter Diseases
LTC Robert Shih
Chief of Neuroradiology
Walter Reed Medical Center
Thanks to LTC Alice Smith (retired)
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Disclosures: None.
This presentation reflects the personal views of the speaker and not of the US government or Department of Defense.
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Unknown Case 1
22 year old female was found unresponsive. Her roommate says patient had been vomiting all week.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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Unknown Case 2
68 year old with Waldenstrom’s macroglobulinemia who is presenting with altered mental status.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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Unknown Case 3
45 year old female with history of migraines has
recurrent symptoms suggestive of TIA.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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Myelin – the dielectric insulation
Normal white matter is hyperintense on T1 and hypointense on T2 compared with gray matter because myelin is 80% lipid and 20% protein. White matter diseases are often T2 hyperintense because of myelin loss (decreased lipid and increased water).
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Approach to White Matter Lesions
History & distribution
Common diseases
MS, SVID
Uncommon diseases
PML, CPM
No specific pattern
VITAMIN C
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Primary: Multiple Sclerosis
• Etiology: Unknown
• Peak age: 20-40 years
• Diagnosis: Clinical & paraclinical criteria – CSF oligoclonal bands
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Courtesy of Robert E. Schmidt, MD, PhD
Perivenular Inflammation: “Dawson’s Fingers”
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MS: Imaging
• FLAIR does not detect lesions in posterior fossa, brain stem, & spinal cord as well as T2
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MS: Imaging
• MR spectroscopy: – Decreased N-acetyl aspartate (NAA)
– Increased choline, lipids, & lactate (nonspecific)
32 Year Old
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MS: Spine
Cord lesions can be small and subtle
(more conspicuous on sagittal STIR).
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Revised McDonald Criteria
• Three of the following: – ≥ 1 gadolinium-
enhancing lesion or 9 T2 hyperintense lesions if there is no gadolinium enhancing lesion
– ≥ 1 infratentorial lesion – ≥ 1 juxtacortical lesion – ≥ 3 periventricular
lesions
• Detection of gadolinium enhancement ≥ 3 mos after onset of initial clinical event, if not at site corresponding to initial event.
• Detection of new T2 lesion if it appears at any time compared with reference scan done ≥ 30 days after onset of initial clinical event
TIME SPACE
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Teaching Point: Multiple Sclerosis
Dissemination in space:
1. Periventricular – radiating
2. Callosal – inferior margin
3. Infratentorial – peripheral
Dissemination in time:
New or enhancing lesions
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Tumefactive Demyelination
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Tumefactive Demyelinating Lesion (TDL)
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Tumefactive Demyelinating Lesion (TDL)
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Tumefactive Demyelination
• Difficulty in diagnosing TDLs often leads to surgical biopsy
• Histopathologic findings can be misleading
– Hypercellularity
– Atypical reactive astrocytes
– Mitotic figures
• Unnecessary & potentially harmful surgical resection or radiation therapy
Misdiagnosis
of glial
neoplasm
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Teaching Point: Tumefactive Demyelination
“open ring sign”
Signal abnormality ends at edge of enhancement!
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Neuromyelitis Optica (Devics Disease)
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Rare Multiple Sclerosis Variants
• Balo
• Schilder
• Marburg
Balo’s
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Balo’s Concentric Sclerosis
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Primary Demyelinating Diseases = Autoimmune
CNS PNS
Acute / Monophasic
ADEM Guillain-Barre
Chronic / Multiphasic
MS CIDP
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Acute Disseminated Encephalomyelitis
• Diffuse perivenous inflammatory process
– Results in confluent areas of demyelination
• Cortical & deep gray matter also involved
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ADEM: Imaging
• Multiple lesions, may be large
• No new lesions after 6 months
• May be incomplete resolution of lesions
• Periventricular: 50%
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ADEM: Imaging
• Brainstem, spinal cord, & cerebellar white matter: 30-50%
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Demyelinating Lesions: Imaging
Acute Disseminated Encephalomyelitis ADC DWI
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ADEM
• Etiology: Allergic or autoimmune reaction with viral protein
• 10-30% neurologic sequelae
– Acute hemorrhagic leukoencephalitis (Hurst disease)
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White Matter Diseases
• V
• I
• T
• Autoimmune: MS, NMO, ADEM
• M
• I
• N
• C
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Infectious
• HIV-associated
encephalitis
• Progressive multifocal leukoencephalopathy
• Lyme disease
HIV-associated encephalitis
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HIV Encephalopathy
• HIV-associated chronic, neurodegenerative syndrome
• 15-20% of AIDS patients
• Diagnosis: Clinical
• Pre highly active antiretroviral therapy (HAART) median survival after onset of dementia: 6 months
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HIV Encephalopathy: Imaging
42 Year Old Male
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HIV Encephalopathy
No enhancement, no mass effect
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Progressive Multifocal Leukoencephalopathy (PML)
• Progressive demyelinating disorder
• Results from CNS infection with JC papovavirus
– Up to 80% of people infected prior to adulthood without producing illness
– Latent in CNS - reactivates in setting of immune compromise
• Found in 5% of autopsies in patients with AIDS
• AIDS patients with very low CD4 counts – 50-100 cells/mm3
• Oligodendrocytes infected unable to maintain myelin: Focal demyelination
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Progressive Multifocal Leukoencephalopathy (PML)
• CNS infection with JC papovavirus
– Up to 80% of people infected prior to adulthood Latent in CNS - reactivates in setting of immune compromise
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PML: Confluent Asymmetrical White Matter Lesion (subcortical U-fibers)
T1 Post-contrast
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Lyme disease: CNS 10-15%
+ +
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White Matter Diseases
• V
• Infectious: HIV, PML, Lyme
• T
• Autoimmune: MS, NMO, ADEM
• M
• I
• N
• C
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Vascular White Matter Disease
• Senescent or chronic small vessel ischemic changes
• Cerebral autosomal dominant arteriopathy with subcortical infarction & leukoencephalopathy (CADASIL)
• Vasculitis
T2 FLAIR
T2 FLAIR
Senescent white
matter changes
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CADASIL
• Familial, non-amyloid microangiopathy – Mutation of Notch 3 gene
on chromosome 19
• Early onset ischemic stroke (40-50s) – Migraine in younger
patients
• Multi-infarct dementia
T2 FLAIR
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CADASIL
40 Year Old
T2 FLAIR T2
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Vasculitis / Vasculopathies
• Etiologies:
– Primary: PACNS
– Autoimmune: SLE
– Infection: Syphilis, TB, HIV
– Drugs: Cocaine, methamphetamine
– Radiation
T2
DWI
Methamphetamine
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Vasculitis: Imaging
Methamphetamine
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Vasculitis
T2 FLAIR T1+Gd
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White Matter Diseases
• Vascular: SVID, CADASIL, vasculitis
• Infectious: HIV, PML, Lyme
• T
• Autoimmune: MS, NMO, ADEM
• M
• I
• N
• C
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Metabolic/Nutritional
• Osmotic demyelination
• Alcoholism
• B12 deficiency
Central Pontine Myelinolysis
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Osmotic Myelinolysis
T2 FLAIR T2 FLAIR DWI
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Osmotic Myelinolysis
• Sparing of blood vessels, most nerve cells, & axons
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Osmotic Myelinolysis Stroke
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Alcohol
• Atrophy: Cerebral hemispheres, superior vermis
• Marchiafava-Bignami (rare) – Type A: swelling of corpus, poor
outcome
– Type B: Partial callosal lesions, better outcome
• Wernicke encephalopathy – Thiamine deficiency
– Severe memory impairment
– Mamillary bodies & periaqueductal gray (around 3rd to 4th ventricles)
Wernicke Marchiafava-Bignami
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B12 Deficiency
• Most common cause in US: Pernicious anemia
• Subacute combined degeneration – Loss of position & vibration,
paresthesias of hands & feet, lower extremity weakness
– Can affect brain, optic tracts, & peripheral nerves
– Demyelination & axonal loss posterior & lateral spinal cord columns
– MR: T2 hyperintensity posterior columns
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Whippet Abuse
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White Matter Diseases
• Vascular: SVID, CADASIL, vasculitis
• Infectious: HIV, PML, Lyme
• T
• Autoimmune: MS, NMO, ADEM
• Metabolic: CPM/EPM, WE, SCD
• I
• N
• C
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Iatrogenic / Toxic
• Radiation
• Drugs
13 year old on methotrexate,
vincristine, pegasparaginase
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Radiation
• Can produce demyelination
• May cause mineralizing microangiopathy
• Delayed radionecrosis can occur as late as 20 years after radiation.
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Radiation
Images Courtesy Steven Goldstein, MD
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Radiation
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Toxins/Drugs
• Drug abuse
• Chemotherapy
• Methanol: Optic nerve atrophy with necrosis of putamen & subcortical white matter
Methanol
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Methotrexate
• Major cause of acute neurotoxicity – Leukoencephalopathy
– Incidence: 9-35%
• Pathophysiology: Unclear
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Heroin
• Leukoencephalopathy
• Inhalation of heroin vapor – Drug heated on tinfoil –”chasing the dragon”
• Cerebellar or extrapyramidal syndromes
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Inhaled Heroin
Central
Tegmental Tracts
Spongiform degeneration of these regions on histology
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Delayed Hypoxic-Ischemic Leukoencephalopathy
Carbon Monoxide
Images courtesy Howard Rowley, MD
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Delayed Hypoxic-Ischemic Leukoencephalopathy
Images courtesy Steven Goldstein, MD
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Delayed Post-Hypoxic Leukoencephalopathy
• Rare demyelinating syndrome – Acute onset neuropsychiatric symptoms days
to weeks after recovery from coma after period of prolonged cerebral hypo-oxygenation
– Carbon monoxide poisoning, narcotic overdose, myocardial infarction, other global cerebral hypoxic event.
• Imaging: Diffuse hemispheric increased T2 sparing cerebellar & brainstem tracts
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White Matter Diseases
• Vascular: SVID, CADASIL, vasculitis
• Infectious: HIV, PML, Lyme
• Toxic: Methanol, heroin, anoxia
• Autoimmune: MS, NMO, ADEM
• Metabolic: CPM/EPM, WE, SCD
• Iatrogenic: methotrexate, radiation
• N
• C
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Conclusions
• White matter and demyelinating diseases can have similar imaging appearances
– Patient demographics, clinical history, and lesion distribution may be helpful
– When not helpful, consider lumbar puncture and followup imaging with gadolinium
• May mimic neoplasm – keep in back of mind
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Unknown Case 1
22 year old female was found unresponsive. Her roommate says patient had been vomiting all week.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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Unknown Case 2
68 year old with Waldenstrom’s macroglobulinemia who is presenting with altered mental status.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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Unknown Case 3
45 year old female with history of migraines has
recurrent symptoms suggestive of TIA.
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What is the most likely etiology?
1. Vascular
2. Infectious
3. Traumatic
4. Autoimmune
5. Metabolic
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The End Thank you!