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eEdE -29 Acquired Focal and Diffuse White Matter Pathologies -A Practical approach to Radiologist's Dilemma. Role of Multimodality Imaging, Histopathology and Laboratory correlation Prasad Hanagandi, Asim Bag, Lazaro do Amaral, Santanu Chakraborty Thanh Nguyen, Rafael Glikstein, John Woulfe, Gerard Jansen The Ottawa Hospital, University of Ottawa, Canada University of Alabama at Birmingham, Birmingham, AL Medimagem &Hospital da Beneficência Portuguesa and Hospital São José- São Paulo ,Brazil
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No Disclosures
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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.
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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 there diffusion restriction? Is it holo-lesional? Is the lesion diffuse & confluent, non-discrete or discrete? if discrete, how many are they? Is it at the periphery? Is there any enhancement? Where is/are the lesion(s)? Is it ring-like? Periventricular/pericallosal Is it incomplete ring? Subcortical Is the adjacent cortex involved? No clue? Deep cerebral (in the centrum semiovale an in the corona radiata) How is the perfusion? How is the spectroscopy? Brain stem (Is it central or periphery?) Still no Clue?? Cerebellum What are the CSF findings? Only callosal involvement?? What is the serum chemistry Multidistributional Still no clue??? Additional questions to ask: Let’s consider for biopsy. Is it bilateral symmetrical? Is temporal lobe white matter involved? Is it periventricular? Is it anterior temporal lobe? white matter abnormalities on T2-weighted sequences are common abnormalities in routine clinical practice that can be challenging at times because normal aging and many different diseases can manifest as white matter T2 imaging abnormality. The initial imaging assessment often can be intriguing due to considerable overlapping of imaging features. However, a close introspection and use of multimodality imaging yields certain pertinent clues that can help in narrowing down the probable list of differential diagnoses. A combination of relevant clinical input and laboratory work up with follow-up imaging can further aid in the diagnosis. Very often a biospy is mandatory to distinguish inflammatory and vasculitic pathologies from neoplastic etiology. PML, tumefactive demyelination, vasculopathies like amyloid angiopathy associated inflammation, central nervous system (CNS) angiitis, angiocentric /angioinvasive lymphoma and gliomatosis cerebri will be the highlights of this educational exhibit. We want to highlight these specific abnormalities in this electronic exhibit with a simplified practical approach using clinico-radiological algorithm to evaluate white matter disease.
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Approach to the diagnosis: Ask yourself all these questions sequentially
What is the clinical presentation? Is there diffusion restriction? Careful evaluation of T2-weighted images is the key to the diagnosis: Look for Is it holo-lesional? Is it at the periphery? Where is/are the lesion(s)? Periventricular/pericallosal Is there any enhancement? Subcortical Is the adjacent cortex involved? Is it ring-like? Deep cerebral (in the centrum semiovale an in the corona radiata) Is it incomplete ring? Brain stem (Is it central or periphery?) Cerebellum Only callosal involvement?? No clue? Multidistributional How is the perfusion? Is the lesion diffuse or discrete? if discrete, how many are they? How is the spectroscopy? Additional questions to ask: Still no Clue?? Is it bilateral symmetrical? Is temporal lobe white matter involved? What are the CSF findings? Is it periventricular? What is the serum chemistry Is it anterior temporal lobe? Still no clue??? Let’s consider for biopsy.
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Clinico radiological algorithm
Diffuse confluent white matter disease No neurologic symptoms Small vessel disease Prior radiation treatment HIV encephalopathy CADASIL With neurologic symptoms Asymmetric Infectious 1. HSV encephalitis 2. PML White matter disease of systemic causes Tumor 2. Lymphomatosis cerebri 1. Gliomatosis Cerebri Symmetric
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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. Hyperammonemia 2. Hypoxic injury 3. Hypoglycemia Toxic leukoencphalopathy 1. Methotrexate encephalopathy PRES: Posterior reversible encephalopathy PML: Progressive multifocal encephalopathy
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2. Low grade glioma/cortical dysplasia
CADASIL: Cerebral autosomal dominant arteriopathy, subcortical infarct and leukoencephalopathy ARIA: amyloid related imaging abnormality ABRA: Amyloid beta related angiitis ADEM: Acute disseminated encphalomyelitis MELAS: Mitochondrial enchalopathy, lactic acidosis, stroke-like syndrome MS: Multiple sclerosis NMO: Neuromyelitis optica Focal non-discrete Leukoencphalopathy Subcortical 1. PRES 2. Low grade glioma/cortical dysplasia 3. Vasculitis 4. ARIA, ABRA 5. PML 6. ADEM 7. MELAS 8. Cerebral vein thrombosis Deep cerebral white matter 1. Small vessel disease 2. Infarct 3.PML 4. Vasculitis 5. Methotrexate encephalopathy 6. Angiocentric lymphoma Temporal lobe white matter CADASIL Periventricular 2. MS 3. CNS lymphoma Mesodiencephalic junction Behcet’s disease Brainstem 1. Small Vessel disease 2. Brainstem Glioma 3. Central variant of PRES 4. Osmotic demyelination 5. Rhombencephalitis 6. NMO Cerebellum 1. PML 2. Cerebrotendinous xanthomatosis 3. AV fistula
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Subcortical nonfluent white matter abnormalities
Hypertensive emergency Immunomodulator/cancer chemotherapy PRES History of seizure No 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 restriction Vessel lumen irregularity Vasculitis Age >45-50 History of amyloid modifying drugs Micro- macrohemorrhage ± enhancement ARIA ABRA H/O Immune deficiency NO mass effect Diffusion restriction at the advancing edge NO enhancement PML Prodromal history Lesions at other locations, particularly art basal ganglia ± Enhancement ±Diffusion restriction ADEM Multiple episodes of focal neurologic deficits Family history in the mother ‘s side Diffusion restriction Increased lactate peak MELAS FLAIR signal and T1 hyperintensity in the Adjacent cortical vein Cortical vein thrombosis
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Focal non-discrete deep cerebral white matter lesion Asymptomatic
Small vessel disease Holo-lesional Diffusion restriction Acute onset Infarct Holo-lesional diffusion restriction H/O Methotrexate therapy Subacute onset Methotrexate encephalopathy Diffusion restriction at the margin H/O immunodeficiency Sub acute onset PML H/O autoimmune disease (lupus, Sjogren etc. ) Vessel wall irregularity on angiogram Headache Vasculitis Nodular/Perivascular pattern of enhancement Diffusion restriction Granulomatous vasculitis Angiocentric Lymphoma
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Non discrete brainstem white matter lesions NO symptoms Central pons
Small vessel disease H/O Hyponatremia or alcoholism Central pons, sparing periphery ± Diffusion restriction Osmotic demylaination syndrome Involvement of the midbrain ± involvement of thalamus No enhancement No overt mass effect Behcet disease Diffuse brainstem enlargement with increased FLAIR signal Acute presentation Hypertensive urgency Immune modulator/chemotherapy Lupus Central variant of PRES Subacute presentation 2. Rhombencephalitis 1. Brainstem glioma Involvement of the floor of the 4th ventricle (Area prostrema) NMO
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Discrete white matter lesion Subcortical
4. Ganglioglioma/DNET 3. Metastasis 2. MS 1. Small vessel disease Deep cerebral 4. Border zone infarct 3. MS 2. Lacunar infarct 1. Small Vessel disease Callosal 4. Marchiafav-Bignami syndrome 3. Susac syndrome 1. MS 2. Reversible splenial abnormality Periventricular/pericallosal 3. Lacunar infarct Brain stem MS Cerebellum
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Examples
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Case: 1. 65 Year normotensive male presenting with headache and subacute onset (Over several days) visual field deficits. Image Analysis: Pattern: Patchy non-discrete Location: Subcortical Number: Multifocal Diffusion restriction: No
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Associated Clues: Microhemorrhage No appreciable enhancement
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Pertinent negative clues:
NO sinus thrombosis NO arterial Ocllusion Low perfusion
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Case analysis: Older patient, subacute etiology, no hypertensive urgency Subcortical non-discrete lesions Microhemorrhages No diffusion restriction No enhancement H and E stain and Beta Amyloid stain confirm the diagnosis of Amyloid Angiopathy Diagnosis: Amyloid Angiopathy
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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.
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T2W coronal, FLAIR sagittal images depict similar white matter changes
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.
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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 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
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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.
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Case Analysis Subacute onset
Deep cerebral non-discrete white matter disease No significant mass effect Nodular and perivascular enhancement Diffusion restriction lymphocytes CD20 immunostaining positive for lymphoma Vessel lumen H & E stain shows diffuse sheet of lymphocytes encasing the vessel ( arrows) . CD20 immunostaining positive for lymphoma. Diagnosis: Angiocentric Lymphoma
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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.
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Case analysis Subacute onset
White matter lesions are non discrete, multiple, involves subcortical, deep cerebral and periventricular white matter. Positive diffusion restriction Perivascular enhancement H & E stain showing sludging of the vessel lumen by lymphocytes (arrow) . CD45 immunostaining positive for lymphoma (arrow). Diagnosis Intravascular Lymphoma
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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.
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Case analysis Immunodeficiency patient
Diffuse and confluent white matter lesions Absence of mass effect Absence of enhancement Diffusion restriction at the advancing edge 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
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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.
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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 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
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52 Year male presenting with seizures.
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.
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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.
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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) Ki-67 proliferation marker showing high proliferation index. Diagnosis: Gliomatosis cerebri
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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
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