1 Acute Necrotizing Encephalopathy: Neuroimaging Diagnosis and Differentials
Kevin Y Wang, Doris DM Lin MD PhDDivision of NeuroradiologyDepartment of Radiology and Radiological ScienceJohns Hopkins School of Medicine, Baltimore, MarylandPresentation # eEdE-163Presented by: Kevin Y WangASNR, Chicago, April 25-30, 2015
2 DisclosuresWe have no financial disclosures or conflicts of interest to declare.
3 PurposeTo review neuroimaging features of acute necrotizing encephalopathy (ANE)To evaluate the current diagnostic criteria of ANETo provide a radiologic differential diagnosis of ANE
4 Methods Reviewed English literature for reported cases of ANE
Retrieved 11 publications with both neuroimaging and histopathology findings of ANERetrieved 53 publications with general neuroimaging findings of ANEReviewed imaging features and clinical diagnosis of pediatric cases with bilateral thalamic lesions at home institution
5 Outline Clinical, Imaging, and Pathology Features Literature Review
Radiologic Differential DiagnosisAppendixClick here to return to title slideClick here to return to this slide at anytimeClick on any link above to jump directly to a particular section
6 Clinical, imaging, & pathology features
Acute Necrotizing EncephalopathyClinical, imaging, & pathology features
7 Epidemiology and Clinical Features
Acute encephalopathy with distinct imaging featuresFirst described in 1995 by Mizuguchi et alHigher incidence in children and in Far EastRapid deterioration of consciousness until comatose, usually within 24 hoursAntecedent acute febrile illnessAssociated with wide range of viral agents (Influenza A most common)
8 Neuroimaging Features
Symmetric, bilateral, multifocal lesionsThalami always involvedVariable additional involvement:Lentiform nucleiBrainstem tegmentumCerebral white matterCerebellumVariable enhancement, hemorrhageImaging findings evident after coma ensues
9 Illustrative Case9-month old previously healthy female presenting with 4 days of upper respiratory symptoms and 1 day of suspected seizures
10 Acute T2-Weighted Signal Changes
Symmetrically increased signal in caudate nucleiIncreased signal in thepontine tegmentumSymmetrically increased signal and edema in thalami
11 Acute Diffusion-Weighted Changes
DWIThalamiCerebellar hemispheresPontine tegmentumMidbrain tegmentumPeriventricular WMADCCorresponding decreased apparent diffusion coefficient (ADC) values
12 Distinct ADC Pattern of Injury
A: High ADC – Hemorrhagic necrosisB: Low ADC – Cytotoxic edemaC: Very high ADC – Vasogenic edemaAdapted from Albayram S et al. AJNR Am J Neuroradiol 2004;25:792–97
13 Chronic T2-Weighted Changes
Cystic degeneration of the periventricular white matter at 2 months following insultCystic degeneration of the pontine tegementum at 4 years following insult
14 Familial Form of ANE (ANE1)
Autosomal dominantRecurrent episodesAtypical distribution compared to sporadic form of ANE:External capsuleMedial temporal lobesHippocampi and amygdalaSpinal cordMay omit thalamiT2WINaidu S, Lin DD. Expert Opin Med Diagn. 2012;6(4):
15 Pathology Features Grey matter White matter
NecrosisPetechial hemorrhageWhite matterPlasma-like substance extravasationMyelin pallorLack of inflammation, apoptosis, and viral pathogen
16 Proposed Diagnostic Criteria for ANE
Acute encephalopathy following febrile illnessNo CSF pleocytosis1No elevated serum ammonia2CT or MRI evidence of symmetric thalamic lesionsExclusion of resembling diseasesModified from Mizuguchi et al, 19971Primarily intended to exclude viral encephalitis and acute disseminated encephalomyelitis2Primarily intended to exclude Reye’s syndrome, given frequent elevation of transaminase levels in ANE
17 Acute Necrotizing Encephalopathy
Literature review
18 Summary of Neuroimaging Findings of 53 Publications (1989-2015)
(2%)Click here for case-by-case summary(3%)(17%)(35%)(36%)(67%)(99%)
19 Summary of Neuroimaging Findings of 53 Publications (1989-2015)
FrequencyPercentage of Total CasesHemorrhage133 / 9435%Contrast enhancement19 / 6927%Atypical involvement2Asymmetric thalamic lesions5 / 1653.0%Medial temporal lobe9 / 1655.4%Spinal cord3 / 1651.8%HypothalamusHippocampus/amygdala1 / 1650.6%MedullaBoth dorsal & ventral brainstem4 / 1652.4%1As detected by T1/T2 shortening, T2*, gradient-echo imaging, or susceptibility-weighted imaging2Cases reporting atypical imaging findings did not report testing for familial form of ANE
20 Summary of 14 Cases with Radiologic-Pathologic Correlation (1993-2014)
StudyYearAgeThalamic LesionsOther LesionsHistopathologyWang et al20141YesWM, PT, CB, BGHypereosinophilic neuronsLyon et al201012WM, PT, CBHemorrhagic NecrosisNg et al6 NoCB, BG, PT, MTSan Milan et al2007CB, WMMastroyianna et al20062Kirton et al2005PT, MT, WMMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia
21 Summary of 14 Cases with Radiologic-Pathologic Correlation (1993-2014)
StudyYearAgeThalamic LesionsOther LesionsHistopathologySazgar et al20039YesBG, MT, PTHemorrhagic NecrosisRavidet al200112BG, MT, WMChronic inflammation and microgliaMizuguchi et al20021BG, WM, PT, CB3 BGYagishita et al1995WM, PT, CBNakano et al1993CB, PT, MTMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia
22 SummarySporadic ANE may rarely demonstrate atypical neuroimaging findings not encompassed in current, proposed diagnostic criteriaRarely, imaging features may also be consistent with ANE but not confirmed on neuropathologyAccuracy of neuroimaging to positively and negatively predict ANE is high but not perfect
23 ImplicationA combination of clinical and radiological features may at most be suggestive of ANENeuropathology may ultimately be necessary for definitive diagnosisHowever, biopsy may be of little clinical and practical utility, and unlikely to change managementPost-test probability from imaging is likely sufficiently high to treat presumptively for ANE in most cases
24 Radiologic Differential Diagnosis
Acute Necrotizing EncephalopathyRadiologic Differential Diagnosis
25 Radiologic Differential Diagnosis
Severe Hypoxic-Ischemic InjuryArterial InfarctVenous InfarctAcute Disseminated Encephalomyelitis (ADEM)Hemolytic Uremic Syndrome (HUS) EncephalopathyViral EncephalitisBilateral Thalamic Glioma
26 Severe Hypoxic-Ischemic Injury
Consistent with ANESymmetric basal ganglia, thalamic lesionsRestricted diffusion of lesionsInconsistent with ANELesions within hippocampi, peri-rolandic and occipital cortexDWIADC4d F with term delivery found to be pale, seizing, in respiratory distress
27 Artery of Percheron Infarct
Consistent with ANESymmetric thalamic paramedian lesionsInvolvement of rostral midbrainInconsistent with ANEFails to explain other lesions seen in ANEUncommon in childrenDWIADC89M with DM and HTN found unresponsive by wife on front porch
28 Basilar Artery Thrombosis
Consistent with ANEBilateral thalami, brainstem, cerebellum involvedRestricted diffusion of lesionsInconsistent with ANEPositive MRA findingsHyperdense basilar artery sign on CTDWIADC68F with acute onset of AMS, anisocoria, and eye movement abnormalities
29 Dural Venous Sinus Thrombosis
DWIADCConsistent with ANEBilateral, symmetric thalamic lesionsCentral hemorrhagic transformation possibleInconsistent with ANEFails to explain other lesions seen in ANEPositive MRV findingsMRV4 mo F with fever, vomiting, increased irritability, and hyperdense straight sinus on CT
30 Acute Disseminated Encephalomyelitis
Consistent with ANEVariable symmetric thalami and basal ganglia lesions; variable brainstem lesionsVariable enhancementPreceded by infectionInconsistent with ANENo restricted diffusionAsymmetric, multifocal, patchy, tumefactive white matter lesionsDelayed onset after infectionLymphocytic CSF pleocytosisT2 FLAIR25M with 3 months of intermittent fevers and headaches
31 Hemolytic Uremic Syndrome Encephalopathy
Consistent with ANEBilateral basal ganglia lesions most characteristicVariable thalami, brainstem, cerebellum involvementRestricted diffusion of lesionsMay be preceded by infectionInconsistent with ANEHippocampi, insula, and cerebral peduncle variably involvedResponsive to plasmapheresisSteinborn M et al. Pediatr Radiol 2004;34:
32 Viral Encephalitis Consistent with ANE Inconsistent with ANE
Basal ganglia and thalamic lesions in EBVBilateral thalamic, brainstem, cerebellar lesions in Japanese encephalitis virusBilateral thalamic, basal ganglia, midbrain lesions in West Nile virusInconsistent with ANECSF pleocytosisPositive viral PCR or culture in CSFSmith AB et al. AJR. American journal of roentgenology. Feb 2009;192(2):W53-62.
33 Bilateral Thalamic Glioma
Consistent with ANEBilateral thalamic lesionsInconsistent with ANENo restricted diffusionNo contrast enhancementFails to explain other lesions seen in ANET2 FLAIRPost-Gad T1WI22F with progressive left UE weakness and difficulty concentrating
34 Summary ANE is distinctly characterized by symmetric thalamic lesions
Given clinical and radiologic features may lack specificity, neuropathology may be required for definitive diagnosis of ANEANE, as well as a number of other diagnoses, should be considered in a child with an acute presentation of bilateral thalamic lesions
35 Select References Proposed Diagnostic Criteria
Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain & development. Mar 1997;19(2):81-92.Differential Diagnosis of Bilthalamic LesionsSmith AB, Smirniotopoulos JG, Rushing EJ, Goldstein SJ. Bilateral thalamic lesions. AJR. American journal of roentgenology. Feb 2009;192(2):W53-62.Atypical Cases of Sporadic ANEKim JH, Kim IO, Lim MK, et al. Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean journal of radiology : official journal of the Korean Radiological Society. Jul-Sep 2004;5(3):San Millan B, Teijeira S, Penin C, Garcia JL, Navarro C. Acute necrotizing encephalopathy of childhood: report of a Spanish case. Pediatric neurology. Dec 2007;37(6):Wang KY, Singer HS, Crain B, Gujar S, Lin DD. Hypoxic-ischemic encephalopathy mimicking acute necrotizing encephalopathy. Pediatric neurology. Jan 2015;52(1):
36 Acute Necrotizing Encephalopathy
Appendix
37 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastSuwa et alSymmetricP N YWong et alBS WM CBMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
38 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastWong et alSymmetricBS Y WM CBMizuguchi et alPT NP, GBPT, MTPMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
39 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastMillan et alNoneWM CB NYadav et alSymmetricPT, MTYSener et alBSLyon et alPTY (T2*)Weitkamp et alMTAshtekar et alSazgar et alPBassuk et alKirton et alVoudris et alMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
40 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastOrmitti et alSymmetricPT WM CBY (GRE)YMartin et alP NOouttara et alPT, MTMariotti et alFung et alY (SWI)Kumakura et alKansagra et alTabarki et alAkiyoshi et alMastroyianni et alMTMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
41 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastMastroyianni et alAsymmetricWM CB Y NRavid et alSymmetricC, GB, PMTYoshikawa et alPPT, MTUnilateralAlbayram et alPTY (GRE)Harada et alIchiyama et alBSMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
42 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastKubo et alSymmetricPT CB NOkumura et al3/22 cases10/22 cases5/22 casesMizuguchi et alBS WMManara et alY (GRE)YHuang et alPT, MTNg et alC, GB, PSeo et alYoshida et alKim et alPOffiah et alCampistol et alMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
43 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastCampistol et alAsymmetricWM CB NResaei et alSymmetricP YKim et alCPT, MTPTMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
44 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastKim et alSymmetricPT, MTWM N Y P PTGoo et alY (T2*)Saleiomran et alP, GB, CCBWeng et alSkelton et alVicente-GutiérrezMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
45 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastKhan et alSymmetricPT, MTWM NAlvarenga et alCBPorto et alOkumura et al8/38 cases20/38 cases15/38 casesLee et alBGP, GBPTAsymmetricMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
46 Appendix: Neuroimaging Findings of 53 Publications (1989-2015)
StudyThalamic LesionsBasal GangliaBrainstemWhite MatterCerebellumHemeContrastLee et alSymmetricWMY (GRE)CBPT, MTBGMT = Midbrain TegmentumPT = Pontine TegmentumWM = White MatterCB = CerebellumBG = Basal Ganglia* BS = Brainstem**unspecifiedP = PutamenC = CaudateGB = Globus PallidusReturn to graph
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