05/11/2020 1 MS Mimics Differential diagnosis of white matter lesions Carolina Tramontini, M.D. Neuroradiologist Clínica Reina Sofía Clínica Infantil Santa María del Lago Fundación Universitaria Sanitas ECNR 15th Cycle, Module 4 November 8th, 2020 I have no conflicts of interest MS Mimics Differential diagnosis of white matter lesions White matter lesions • Frequent • Involve periventricular, deep and subcortical white matter • Hyperintense on PD, T2 and FLAIR • Check intensity on T1 • Additional sequences: SWI, diffusion White matter lesions Vascular Vascular Toxic Toxic Infectious Infectious Demyelinating Demyelinating Other Other Leukodystrophies Leukodystrophies 1 2 3 4
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MS Mimics Differential diagnosis of white matter lesions
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MS MimicsDifferential diagnosis of white matter
lesions
Carolina Tramontini, M.D.Neuroradiologist
Clínica Reina SofíaClínica Infantil Santa María del Lago
Girl 11 yo, respiratory symptoms a week ago, now headache, vomiting, dislalia ADEM
• ADEM is an inflammatory demyelinating disease of theSNC
• Monophasic disorder with multifocal neurologic symptoms and encephalopathy
• There is no specific biological marker or test
• Diagnosis of ADEM is based on clinical and radiological features
• In children ADEM is the most important alternative diagnosis to MS
Lee YJ. Korean J Pediatr 2011;54(6):234-240 Krupp, 2013
Clinical findings
• Age 5‐8 years, more frequent in males
• Preceding viral illness
• Prodromal phase
– Fever, myalgias and malaise
• Clinical phase
– Fever, headache, seizures
– Multifocal neurological symptoms
– Encephalopathy
– Commonly consciousness impairment ranging from lethargy to deep coma
• Monophasic (most but not all)
• Patients usually recover, some have sequelae
Extensive white matter lesions (bilateral in 87%)Extensive white matter lesions (bilateral in 87%)
Cortico‐yuxtacortical lesions
Deep grey matter lesions (50%)Deep grey matter lesions (50%)
Infratentorial lesionsInfratentorial lesions
Spinal lesions (40% )
Contrast enhancement (25%)
Imaging findings
Rossi, ECNR 2017Boesen et al, 2018
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• Multifocal, extensive, variable size
• Bilateral, but asymmetrical
• Peripheral white matter and corticosubcortical junction
• Periventricular lesions are notfrequent
White matter lesions
Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 37704
White matter lesions
• Basal ganglia and thalamus
• Involved in 50% of cases
Deep grey matter involvement
Lee YJ. Korean J Pediatr 2011;54(6):234-240Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 2576
Infratentorial lesions
• May involve brainstem, cerebellar peduncles, cerebellar hemispheres
• Different size
• Sometimes tumefactive brainstem lesions
Other lesions in ADEM
Cortico‐subcortical lesions
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Corpus callosum lesions Corpus callosum lesions
MS ADEMSusac MS
• Frequent
• Involvement of whole thickness, large, edematous
• Callososeptal lesions are not frequent
Corpus callosum lesions• Present in 14‐30%
• Depending on the timing of MRI
• Generally enhance simultaneously
• Types
– Nodular
– Ring
– Open ring
– Gyral
– Spotty
Enhancement
Rossi, 2008Berzero et al , 2015Gaillard F, Radiopaedia.org, rID: 2576
Abscence of enhancement does not exclude diagnosis
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Enhancement
Monophasicdisease
Simultaneousenhancementof most of lesions
• Encephalopathy:
ADEM 52% vs EM 7%
• Extensive lesions:
+++ ADEM (older than 10 years)
Callen et al, Neurology 2010
ADEM vs MS MRI
Callen: Two of three criteria
Callen et all, Neurology 2008
ADEM vs MS MRI
Abscense of confluentlesions
Abscense of confluentlesions
Two or more periventricular
lesions
Two or more periventricular
lesions
Black holesBlack holes
ADEMADEM
Preceding viral illness
Monophasic, autolimited
Late imaging changes
Resolution
CONTROL MR
MSMS
Periventricular
Callososeptal surface
Callen Criteria
MS-ADEM
CONTROL MR
Callen et al, Neurology 2008Ketelslegers et al Neurology 2010Zhang et al, MSJ 2014
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Neuromyelitis optica spectrumdiseases
• NMOSD is the most important differential diagnosis for MS in Latam
• Incidence of 0.5 to 5/100.000 in Latinamerica
• Inflammatory white matter disease
• Astrocytopathy : oligodendrocyte and myelin notprimarily involved
• Treatments of NMOSD and MS are different
Lee, McMullen, Carruthers, Traboulsee, BCMJ , 2016Derle, Günes, Konuskan, Tuncer-Kurne, TJP, 2014Chitnis y cols, Neurology, 2016Tenenbaum y cols, Neurology, 2016
• MR atypical for MS
• White matter hyperintensities
• Typical locations– Periventricular
– Hypothalamus
• Cloud like enhancement
Brain lesions in NMO
NMOBrain lesions
Not specific white matter hyperintensities 50‐80%
NMOTypical brain lesions
• Áreas típicas (7% ): Periependimarias, hipotálamo
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NMOTypical brain lesions
• Area postrema
NMOBrain lesions
• Periventricular lining
NMOBrain lesions
• “Cloud‐like” enhancement
NMO AQP4 (+)Lesions that mimic MS
‐ 10% of adults and 25 % children fulfill image criteria for MSDerle y cols, TJP 2014Pittock y cols, Arch Neurol 2006
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MogAD
• Recently described demyelinating disease
• Antibodies against myelin of the oligodencrocyte (MOG‐IgG)
• Female:male ratio of 1,3:1
• Age of first symptoms 31‐37 years
• Clinically :
– In children ADEM (36%)
– In adults optic neuritis (41%), transverse myelitis brainstem encephalytis
• Recurrent or monophasic
Lana-Pexoito, Biomedicines 2019 Zhou y cols, Journal of Inmunology, 2017
MogAD Brain lesions
Juxtacortical WM lesions are usually clowdy and ill defined
Large edematous lesions of the WM
MOG-antibody associated demyelinating disease of the CNS: A clinical and pathological study in Chinese Han patientsLei Zhou a,1, Yongheng Huang b,1, Haiqing Li c,1, Jie Fand,1, Jingzi Zhangbao a, Hai Yua, Yuxin Li c, Jiahong Lua
MOG-antibody associated demyelinating disease of the CNS: A clinical and pathological study in Chinese Han patientsLei Zhou a,1, Yongheng Huang b,1, Haiqing Li c,1, Jie Fand,1, Jingzi Zhangbao a, Hai Yua, Yuxin Li c, Jiahong Lua,
Brain lesions
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• 35% of anti‐MOG (+) patients have supratentorial lesions
• 27% of patients with anti‐MOG (+) and brain lesions fulfill Barkhof MR criteria for MS
• Periventricular lesions do not fulfill Paty criteria.
Brain lesions
NMO MS MogADMR abnormal at first 52% 64% in CIS
Typical areas 7% No
Periependimal Periventricular. Deep gray
Hypothalamus
Área postrema +++ + +
Corpus callosum Yes Yes No
Non specific lesions 50‐80% No Yes Dawson, juxtacortical No Yes No
“Cloud‐like” enhancement Yes No No
Brain lesions
MS vs NMOSD vs MogADBrain lesions
Multiple sclerosis
NMOSD
Mog‐AD
White matter lesions
VascularVascular
ToxicToxic
InfectiousInfectiousDemyelinatingDemyelinating
OtherOtherLeukodystrophiesLeukodystrophies
MS
ADEM
NMOSD
MOGAD
Aging
SVD
SLE
Cadasil
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Infectious diseases with WM lesions
Pandit L. Ann Indian Acad Neurol. 2009 Jan-Mar; 12(1): 12–21.
HIVHIV
HerpesHerpes
SyphilisSyphilis
TBTB
PMLPML
HTLVHTLV
• Multisystemic inflammatory disease
• Caused by borrelia burgdorferi
• Transmited by ticks (mice, deer)
• Garin and Bujadeaux (1920), Bannwarth (1940)
• Many cases in Old Lyme, Connecticut (1975)
Lyme diseaseClinical data
• Stage 1 ‐ early local
• Stage 2 ‐ early disseminated
– Eritema migrans
– Musculoskeletal
– Neuroborreliosis 5‐20%
• Stage 3 (late)
– Subacute encephalopathy
– Chronic progressive encephalomyelitis
– Late axonal neuropathies
Lyme diseaseClinical data
• PeriventricularWM and spinal hyperintense lesionsonT2