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MR and CT Imaging, Part II
Differential diagnostic evaluation of white matter disorders
Joshua P. Klein, M.D., Ph.D.Departments of Neurology and Radiology
Brigham and Women’s Hospital and Harvard Medical School
American Society of Neuroimaging36th Annual Meeting, Las Vegas, NV
American Society of Neuroimaging36th Annual Meeting, Las Vegas, NV
Disclosures
No financial disclosures relevant to this presentation
Author and Editor compensation and royalties from McGraw-Hill and Oakstone Publishers
Approach to imaging
I. Pattern recognition
II. Acute vs. chronic abnormalities
III. Longitudinal changes
WHITE MATTER
Optic nerves and pathways
Corpus callosum
Cerebral/cerebellar peduncles
Medial longitudinal fasciculus
Posterior columns
Pyramidal tracts
GRAY MATTER
Basal ganglia and thalamus
Juxtacortical & cortical
statdx.com
I. Pattern recognition
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I. Pattern recognition
Klein JP, ACP Medicine 2012
I. Pattern recognition
Klein JP, ACP Medicine 2012
Intra-cortical and deep gray matter lesions
1) Difficult to detect due to reduced contrast between normal and affected GM (compared to WM)
2) Majority of lesions occur adjacent to cortical veins
3) Intra-cortical demyelination occurs as much as WMdemyelination in SP MS, and more than WMdemyelination in PP MS.
4) GM lesions likely contribute independently to clinicaldisability
Neurologist 2011;17:185J Neurol Sci 2005;233:55
I. Pattern recognition I. Pattern recognition
History / lab red flagsa. onset age < 10 or > 50b. clinicoradiographic mismatchc. hearing lossd. progressive or acute onsete. seizures at onsetf. simultaneous bilateral vision loss g. complete transverse myelitish. elevated ESRi. CSF protein > 100 and/or CSF WBC > 50j. h/o rheum, autoimmune, or psych diagnoses
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Neurology 2012;79:S1
I. Pattern recognition I. Pattern recognition
History / lab red flags
I. Pattern recognition
Imaging red flagsa. hypercellularityb. hemorrhagec. infarctiond. necrosise. unusual lesion appearancef. unusual enhancement patterng. hypervascularityh. infiltration/membrane turnover
Neuromyelitis optica
Longitudinally extensive central cord lesionsBilateral or sequential optic neuritis is more common
than in MS
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Sarcoidosis
CSF normal, no OCBs, serum ACE elevated5-15% involvement of CNS
Behçet disease
CSF lymphocytic pleocytosis, no OCBs Relapsing course, brainstem lesions are common
Cobalamin deficiency
Seen in pernicious anemia (anti-parietal cell Abs), and after gastric bypass. Copper deficiency and nitrous oxide exposure can produce similar lesions.
Lupus
+ANA, +dsDNA, elevated APLA IgM, and 3 OCBs
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Primary CNS vasculitis
Lymphocytic pleocytosis, ESR nl, biopsy negative; relapsing course; can see enhancing lesions
CADASIL
Notch-3 gene mutation. +FH of early strokes
Anaplastic astrocytoma (multicentric)
Hypercellularity, hypervascularity, and necrosis are atypical for MS
Intravascular lymphoma
Small vessel vasculitis, CSF lymphocytic pleocytosis, angiogram negative. Skin and kidneys involvement.
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Migraine
Clinical, epidemiologic, radiographic overlap with MS
Meier DS, et al. AJNR 2007;28(10):1956-63 Meier DS, Guttmann CR. NeuroImage 2006;32(2):531-7
Demyelination is dynamic
T1 PD T2
T2
PD
T1
II. Acute vs. chronic abnormalities
II. Acute vs. chronic abnormalities
Re-identify all previously seen lesions (T1 & T2)
Identify any new lesions (T1 & T2)
Identify acute demyelination (T1-post & DWI)
Identify non-lesional volumetric changes (T1 & T2)
Reporting: interval or acute demyelination
1.5T 3.0T
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T1-post T2-FLAIR
II. Acute vs. chronic abnormalities
Neurology 2000;54;1427
II. Acute vs. chronic abnormalities
T1-post
T1-post DWI
FLAIR ADC
Radiographics 2006;26:S173
II. Acute vs. chronic abnormalities
Meier DS and Guttmann CR
III. Longitudinal changes
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III. Longitudinal changes
Can longitudinal MRIs predict clinical phenotype and future disability? Therapeutic response?
Parenchymal volume loss (absolute, rate of change)- gray matter fraction- white matter fraction- cervical spinal cord
White and gray matter lesion burden- T2 hyperintense lesions- T1 hypointense lesions
Bakshi R et al, Lancet Neurol 2008;7:615
III. Longitudinal changesvolume gain volume loss
Klein JP et al, AJNR 2011;32:1138
Quantifying atrophy in MS
Klein JP et al, AJNR 2011;32:1138
Quantifying atrophy in MS
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Quantifying atrophy in MS
cervical upper thoracic lower thoracic
Klein JP et al, AJNR 2011;32:1138 Klein JP et al, AJNR 2011;32:1138
Quantifying atrophy in MS
Opposing pathological processes impact CNS volume in MS
• volume loss due to neuronal/axonal degeneration/gliosis• volume gain due to inflammation/edema
Spot measurements of volume may be uninterpretable with respect to progression, disease activity, or prognosis.
Longitudinally, there is clearly accelerated volume loss in patients with all forms of MS compared to controls.
III. Longitudinal changes
JNNP 2011;82:1125
year 0 year 1 subtracted
new
regressed
enlarged
III. Longitudinal changes
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Bakshi R et al, Arch Neurol 2008;65:1449
III. Longitudinal changes
MRI-based continuous scale as a marker of MS disease severity, the “MRDSS”
- T2 lesion volume (T2LV) - T1 lesion volume (T1LV) - Brain parenchymal fraction (BPF)- T1:T2 lesion volume (assessment of lesion severity)
Distinguishes RR from SP phenotypes
Bakshi R et al, Lancet Neurol 2008;7:615
III. Longitudinal changes
DTI tractography of the descending corticospinal tracks
Giussani et al, Neuroimage 2010;52:217
Three cases of pediatric infiltrating glioma
tumor
Giussani et al, Neuroimage 2010;52:217
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Two cases of pediatric demyelinating disease
Giussani et al, Neuroimage 2010;52:217
Brainstem tuberculoma
Lyons JL et al, submitted
Summary
I. Pattern recognition
II. Acute vs. chronic abnormalities
III. Longitudinal changes