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Prasad Hanagandi, Asim Bag, Lazaro do Amaral, Santanu Chakraborty Thanh Nguyen, Rafael Glikstein, John Woulfe, Gerard Jansen
1. The Ottawa Hospital, University of Ottawa, Canada2. University of Alabama at Birmingham, Birmingham, AL3. Medimagem &Hospital da Beneficência Portuguesa and Hospital São José- São Paulo ,Brazil
Acquired Focal and Diffuse White Matter Pathologies -A Practical approach to Radiologist's Dilemma. Role of Multimodality Imaging, Histopathology and Laboratory correlation
eEdE -29
No Disclosures
Purpose:
• In this education exhibit, we will describe• An image-based practical approach to acquired white
matter abnormalities in adults.
• A clinico-radiological algorithm demonstrating how to approach different white matter lesions in a day-to-day clinical practice.
• Examples of how to use this algorithm in some common and rare diseases.
Approach to the diagnosis:Ask yourself all these questions sequentially
• What is the clinical presentation?• Careful evaluation of T2-weighted images
is the key to the diagnosis:
– Is the lesion diffuse & confluent, non-discrete or discrete? if discrete, how many are they?
– Where is/are the lesion(s)?• Periventricular/pericallosal• Subcortical
– Is the adjacent cortex involved?
• Deep cerebral (in the centrum semiovale an in the corona radiata)
• Brain stem (Is it central or periphery?)• Cerebellum• Only callosal involvement??• Multidistributional
– Additional questions to ask:• Is it bilateral symmetrical?• Is temporal lobe white matter involved?
– Is it periventricular?– Is it anterior temporal lobe?
• Is there diffusion restriction?• Is it holo-lesional?• Is it at the periphery?
• Is there any enhancement?– Is it ring-like?– Is it incomplete ring?– Is it holo-lesional?
• No clue?– How is the perfusion?– How is the spectroscopy?
• Still no Clue??– What are the CSF findings?– What is the serum chemistry
• Still no clue???– Let’s consider for biopsy.
Approach to the diagnosis:Ask yourself all these questions sequentially
• What is the clinical presentation?• Careful evaluation of T2-weighted images is the
key to the diagnosis: Look for– Where is/are the lesion(s)?
• Periventricular/pericallosal• Subcortical
– Is the adjacent cortex involved?• Deep cerebral (in the centrum semiovale an in the
corona radiata)• Brain stem (Is it central or periphery?)• Cerebellum• Only callosal involvement??• Multidistributional
– Is the lesion diffuse or discrete? if discrete, how many are they?
– Additional questions to ask:• Is it bilateral symmetrical?• Is temporal lobe white matter involved?
– Is it periventricular?– Is it anterior temporal lobe?
• Is there diffusion restriction?
• Is it holo-lesional?• Is it at the periphery?
• Is there any enhancement?– Is it ring-like?– Is it incomplete ring?– Is it holo-lesional?
• No clue?– How is the perfusion?– How is the spectroscopy?
• Still no Clue??– What are the CSF findings?– What is the serum chemistry
• Still no clue???– Let’s consider for biopsy.
Clinico radiological algorithm
Diffuse confluent white matter disease
No neurologic symptoms
Small vessel disease
Prior radiation treatment
HIV encephalopathy
CADASIL
With neurologic symptoms
Asymmetric
Infectious1. HSV encephalitis
2. PML
White matter disease of systemic causes
Tumor1. Gliomatosis Cerebri
2. Lymphomatosis cerebri
Symmetric
CADASIL
White matter disease of systemic causes
Clinico-radiological algorithm
Diffuse white matter diseases of systemic
causes
Asymmetric
Infection
PML
HSV encephalitis
Metabolic 1. PRES
Bilateral symmetrical
Autoimmune 1. Hashimoto encephalitis
Metabolic disturbances
1. Hyperammonemia2. Hypoxic injury3. Hypoglycemia
Toxic leukoencphalopathy 1. Methotrexate encephalopathy
PRES: Posterior reversible encephalopathyPML: Progressive multifocal encephalopathy
Focal non-discreteLeukoencphalopathy
Subcortical
1. PRES2. Low grade glioma/cortical dysplasia3. Vasculitis4. ARIA, ABRA5. PML6. ADEM7. MELAS8. Cerebral vein thrombosis
Deep cerebral white matter
1. Small vessel disease2. Infarct3.PML4. Vasculitis5. Methotrexate encephalopathy6. Angiocentric lymphoma
Temporal lobe white matter
CADASIL
Periventricular
1. Small vessel disease2. MS3. CNS lymphoma
Mesodiencephalic junction
Behcet’s disease
Brainstem
1. Small Vessel disease2. Brainstem Glioma3. Central variant of PRES 4. Osmotic demyelination5. Rhombencephalitis6. NMO
Cerebellum
1. PML2. Cerebrotendinous xanthomatosis3. AV fistula
CADASIL: Cerebral autosomal dominant arteriopathy, subcortical infarct and leukoencephalopathy
ARIA: amyloid related imaging abnormalityABRA: Amyloid beta related angiitisADEM: Acute disseminated encphalomyelitisMELAS: Mitochondrial enchalopathy,lactic acidosis, stroke-like syndromeMS: Multiple sclerosisNMO: Neuromyelitis optica
Subcortical nonfluent white
matter abnormalities
Hypertensive emergencyImmunomodulator/cancer
chemotherapyPRES
History of seizureNo enhancement
No diffusion restriction
±Focal mass effect Low grade glioma
No mass effect±
Radiation band towards ventricle
Cortical dysplasia± H/O connective tissue
disorder±enhancement±Hemorrhage
± diffusion restrictionVessel lumen irregularity
Vasculitis
Age >45-50History of amyloid modifying drugs
Micro- macrohemorrhage± enhancement
± diffusion restriction
ARIAABRA
H/O Immune deficiencyNO mass effect
Diffusion restriction at the advancing edgeNO enhancement
PML
Prodromal historyLesions at other locations,
particularly art basal ganglia± Enhancement
±Diffusion restriction ADEM
Multiple episodes of focal neurologic deficits
Family history in the mother ‘s sideDiffusion restriction
Increased lactate peak
MELAS
FLAIR signal and T1 hyperintensity in the
Adjacent cortical vein± diffusion restriction
Cortical vein thrombosis
Focal non-discrete deep cerebral white matter lesion
Asymptomatic Small vessel disease
Acute onsetHolo-lesional Diffusion restriction
Infarct
H/O Methotrexate therapySubacute onset
Holo-lesional diffusion restrictionMethotrexate encephalopathy
H/O immunodeficiencySub acute onset
Diffusion restriction at the margin PML
H/O autoimmune disease (lupus, Sjogren etc. )
HeadacheVessel wall irregularity on
angiogram
Vasculitis
Subacute onsetDiffusion restriction
Nodular/Perivascular pattern of enhancement
Angiocentric LymphomaGranulomatous vasculitis
Non discrete brainstem white matter lesions
Central ponsNO symptoms
Small vessel disease
Central pons, sparing peripheryH/O Hyponatremia or alcoholism
± Diffusion restrictionOsmotic demylaination syndrome
Involvement of the midbrain± involvement of thalamus
No overt mass effectNo enhancement
Behcet disease
Diffuse brainstem enlargement with increased FLAIR signal
Acute presentation Hypertensive urgencyImmune modulator/chemotherapy
LupusCentral variant of PRES
Subacute presentation1. Brainstem glioma
2. Rhombencephalitis
Involvement of the floor of the 4th ventricle
(Area prostrema)NMO
Discrete white matter lesion
Subcortical
1. Small vessel disease2. MS3. Metastasis4. Ganglioglioma/DNET
Deep cerebral
1. Small Vessel disease2. Lacunar infarct3. MS4. Border zone infarct
Callosal
1. MS2. Reversible splenial abnormality3. Susac syndrome4. Marchiafav-Bignami syndrome
Periventricular/pericallosal1. Small vessel disease2. MS3. Lacunar infarct
Brain stem MS
Cerebellum MS
Examples
Case: 1.65 Year normotensive male presenting with headache and subacute onset (Over several days) visual field deficits.
Image Analysis:Pattern: Patchy non-discreteLocation: SubcorticalNumber: Multifocal Diffusion restriction: No
Associated Clues: 1. Microhemorrhage2. No appreciable
enhancement
Pertinent negative clues:NO sinus thrombosisNO arterial OcllusionLow perfusion
H and E stain and Beta Amyloid stain confirm the diagnosis of Amyloid Angiopathy
Diagnosis: Amyloid Angiopathy
Case analysis:– Older patient, subacute etiology, no
hypertensive urgency– Subcortical non-discrete lesions– Microhemorrhages– No diffusion restriction– No enhancement
Case 2:24 Year female presenting with multiple episodes of headache seizures and right sided weakness.
T1W , FLAIR and T2W images depict confluent area of T2-FLAIR signal abnormality in the left posterior frontal and parietal shite matter with no significant mass effect.
T2W coronal, FLAIR sagittal images depict similar white matter changes.Multiple punctate foci of microhemorrhage are noted on the GRE sequence. Focal leptomeningeal and perivascular space enhancement is noted on the post gadolinium axial image.
MR spectroscopy reveals Decreased peak heights of all metabolites.
Lumen
Adventitia
Media
Internal elastic lamina
H & E stain reveals thickening of the tunica media with inflammatory changes surrounding the adventia representing features of Primary CNS angiitis
Diagnosis:Primary CNS Angiitis
• Case analysis– Young female patient (Unlikely to be ABRA)– Non discrete subcortical white matter lesion– Absence of other lesions (unlikely to be ADEM)– Presence of microhemorrhage– Leptomeningeal enhancement– Lack of choline peak (Unlikely to be tumor or acute
demyelination
Case 3.63 Year male presenting with subacute onset seizures and left sided weakness.
T2W and FLAIR images depict confluent area of hyperintense signal abnormality in the right frontal subcortical white matter with minimal mass effect and Diffusion restriction. Nodular and linear enhancement is predominantly centered around the perivascular spaces on the post gadolinium images.
H & E stain shows diffuse sheet of lymphocytes encasing the vessel ( arrows) .CD20 immunostaining positive for lymphoma.
Diagnosis:Angiocentric Lymphoma
Vessel lumen
lymphocytes
CD20 immunostaining positive for lymphoma
• Case Analysis– Subacute onset– Deep cerebral non-discrete white matter disease– No significant mass effect– Nodular and perivascular enhancement– Diffusion restriction
Case 4:65 Year male presenting with subacute onset of altered level of consciousness and seizures.
T2W and FLAIR images depict confluent area of hyperintense signal abnormality in both cerebral hemispheres with nodular and linear enhancement on the post gadolinium images. Diffusion restriction is predominantly noted in the central deep white matter.
DiagnosisIntravascular Lymphoma
H & E stain showing sludging of the vessel lumen by lymphocytes (arrow) . CD45 immunostaining positive for lymphoma (arrow).
• Case analysis– Subacute onset– White matter lesions are non discrete, multiple,
involves subcortical, deep cerebral and periventricular white matter.
– Positive diffusion restriction– Perivascular enhancement
Case 5. 51 Year female HIV Positive and low CD4 count presenting with seizures and abnormal behavior
T2W and FLAIR images depict confluent areas of hyperintense signal abnormality in both cerebral hemispheres extending into the parieto-occipital subcortical and deep white matter. Diffusion restriction is noted along the periphery of these lesions with no enhancement on the post gadolinium images. Generalized Volume loss is noted . Diffusion restriction ,lack of mass effect and no enhancement are the key features.
H & E stain showing enlarged oligodendrocyte infected with PML virus (arrow). PML virus probe stain demonstarting the viral inclsuion bodies within the infected oligodendrocyte (arrow).
Diagnosis:PML
• Case analysis– Immunodeficiency patient– Diffuse and confluent white matter
lesions– Absence of mass effect– Absence of enhancement– Diffusion restriction at the advancing
edge
Case 6. 46 Year male presenting with left sided weakness and facial paresthesia.
T2W and FLAIR images depict confluent hyperintense signal abnormality in the right posterior frontal and parietal subcortical white matter. Please note the mainted morphology of the adjacent cortex. Incomplete ring enhancement is noted with peripheral diffusion restriction . MR spectroscopy shows elevated choline peak and reduced NAA.
LFB stain (Luxol fast blue stain) demonstrates the myelin pallor corresponding to the demyelinating zone and the area of transition with normal myelin staining( arrow) Myelin pallor Normal Myelin
Zone of transition Diagnosis:
Tumefactive MS
• Case analysis– Focal discrete subcortical lesion– Maintained morphology of the adjacent cortex– “Open- ring” or “C” type enhancement with the open
end facing the the gray matter (Key finding)– Peripheral diffusion restriction – High choline peak, low NAA peak
Case 7. 52 Year male presenting with seizures.
T2W and FLAIR images demonstrate confluent areas of infiltrative pattern of hyperintense signal abnormality predominantly involving the right temporal lobe and insular cortex with extension across the midline. Focal diffusion restriction is noted in the right anterior putamen. There is minimal mass effect with effacement of cortical sulci. However there is no midline shift.
Intense enhancement with elevated rCBV is noted in the right anterior putamen focus on perfusion study. Rest of the infiltrative white matter changes do not exhibit any obvious enhancement.
Ki-67 proliferation marker showing high proliferation index.
Diagnosis:Gliomatosis cerebri
• Case analysis– Older patient– Diffuse confluent involvement of >3 brain lobes
associated with mass effect (Key finding)– Focal area of enhancement with increased rCBV (Key
finding)
Conclusion
• Pattern recognition of the T2 abnormality is the key to successful diagnosis of the white matter lesions– Remember the clinical presentation– Assess lesion morphology– Assess lesion(s) distribution– Look for suggestive clues from
• Post contrast imaging• Diffusion imaging• Perfusion imaging• Spectroscopy• Angiography
– If no clue: look for appropriate CSF and blood tests– If still no clue: Suggest biopsy