Pediatric Stroke and its Mimics 12 May 2022 Kevin OConnor MD Assistant Professor of Neurology University of Kentucky Introducing Kevin OConnor MD Associate Professor Neurology UK HealthCare ID: 1034435
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1. Is this a Pediatric Stroke?Pediatric Stroke and its Mimics12 May 2022Kevin O’Connor, M.D.Assistant Professor of NeurologyUniversity of Kentucky
2. Introducing…Kevin O’Connor, MD Associate Professor Neurology UK HealthCare Before medicine, Dr. Kevin O'Connor pursued European history at Penn State University and the University of Southern Mississippi. After spending 6 years learning how to write a history, he finally felt prepared to go to the Indiana University School of Medicine. He then completed his neurology residency and vascular neurology fellowship at the University of Kentucky.Afterward, he joined the Department as an Assistant Professor of Neurology in July 2021. He is the medical director of the Frankfort Regional Primary Stroke Center. His research interests include pediatric stroke and adorable cat videos.
3. DisclosuresNone.
4. DisclaimerPediatric stroke includes everyone up to age 18Childhood strokeChildren 28 days of life to 18 years of agePerinatal stroke20 weeks gestation to 28 days postnatal life
5. Pediatric Stroke SeriesWhat is Pediatric Stroke?Review the epidemiology of pediatric strokeOutline causes of pediatric strokeDescribe morbidity and mortality associated with pediatric strokeIs this a Pediatric Stroke: Pediatric Stroke and its MimicsDetail causes of under recognition of pediatric strokeIdentify various presentations of pediatric strokeDiscuss common pediatric stroke mimicsThis Might Be a Pediatric Stroke, What Now: Triaging and Treating Pediatric StrokeDescribe strategies for evaluating potential pediatric strokesDiscuss acute therapies for pediatric strokeReview utility of diagnostic testing in pediatric strokeThis is Definitely a Pediatric Stroke: Interactive Case ReviewReview best practices to triage potential pediatric stroke patientsApply knowledge of evidence-based acute treatments for pediatric strokeEvaluate different approaches to managing pediatric stroke
6. ObjectivesUpon completion of this activity, participants will be able to Detail causes of under recognition of pediatric strokeIdentify various presentations of pediatric strokeDiscuss common pediatric stroke mimics
7. Why Does This Matter?Pediatric stroke is less common than adult strokeMorbidity and mortalityAround 40-70% of stroke survivors have residual deficitsPediatric ischemic/hemorrhagic stroke mortality ranges 4%-8%Pediatric CVST mortality ranges 3%-12% (including perinatal CVST)Elbers et al. J Child Neurol 2014;29:782-788Ferriero et al. Stroke 2019;50:e51-e96Ichord et al. Arch Dis Child 2015;100:174-179Sporns et al. Nat Rev 2022;8
8. Polling QuestionPediatric strokes are just like adult strokes?YesNoI wish they were
9. Polling QuestionI have had to evaluate a patient for a possible pediatric stroke.YesNo
10. Polling QuestionI feel comfortable managing pediatric patients who might be having a stroke.YesNo
11. Case Presentation: ShaunShaun is a 10-year-old boy without significant medical history who presented to University Hospital at 1600 with right facial droop, right-sided weakness, and slurred speech. He had spilled something on the floor at home and was cleaning it when his mother found him crying on the floor at 1500. She had last seen him normal about 10 minutes earlier.
12. Case Presentation: ShaunShaun had apparently urinated on himself at home and when he got to the ED, he received 2 mg IV midazolam. He initially is uncooperative. His exam is non-focal and he appears altered.
13. Polling QuestionShould this patient be evaluated emergently for a stroke?YesNo
14. Polling QuestionDo you think this patient has had a stroke?YesNo
15. Polling QuestionWhat do you think is going on?Ischemic StrokeHemorrhagic StrokeCVSTSeizureMigraineInfectionSomething else
16. Polling QuestionWhat would you like to do next?Image with CT head and CTA head/neckImage with MRI brainCheck my institution’s pediatric stroke algorithmCall a neurologistTalk to the family/patient more and try to get a better exam
17. Case Presentation: ShaunDespite his initial uncooperativeness, he gradually improved over the course of his interview and exam.With repeated coaxing, he had no focal motor deficits.He had an NIHSS of 5 for not being alert, missing two orientation questions, reading comprehension issues (mild aphasia), and dysarthria.
18. Case Presentation: ShaunCT head is unremarkable.CTA head/neck is read as having severe focal stenosis of the proximal A1 branch of the left ACA and the proximal and distal M1 branch of the left MCA.
19. Polling QuestionWhat do you think is going on?Ischemic StrokeHemorrhagic StrokeCVSTSeizureMigraineInfectionSomething else
20. Polling QuestionWho contributes to delays in pediatric stroke recognition and care?ParentsProvidersInstitutions/SystemsAll of the above
21. DelaysMallick et al. J Neurol Neurosurg Psychiatry 2015; 86:917-921
22. DelaysParental/caregiver factorsNon-emergent transport to the hospitalLow suspicion for stroke due to mild severity of symptomsProgressive nature of symptomsMackay et al. Stroke 2016;47:2638-2640
23. DelaysSubtle or non-specific presentationsLower diagnostic consideration because of multiple potential mimicsCT head scans can be unrevealing in ischemic stroke34%-47% of children with normal CT head scan later found to have ischemic stroke on MRILimited access to neuroimaging (MRI) or to sedation, especially at night or on weekendsFerriero et al. Stroke 2019;50:e51-e96 Mackay et al. Stroke 2016;47:2638-2640Mallick et al. J Neurol Neurosurg Psychiatry 2015; 86:917-921 Rafay et al. Stroke 2009;40:58-64
24. DelaysFerriero et al. Stroke 2019;50:e51-e96 Mackay et al. Stroke 2016;47:2638-2640Mallick et al. J Neurol Neurosurg Psychiatry 2015; 86:917-921
25. DelaysMedian time from symptom onset to seeking medical care1.7-21 hoursMost present within 6 hoursMedian time for radiologic confirmation of a stroke15-24 hoursIncludes patients in the ED and those already admitted for another illness/injuryFerriero et al. Stroke 2019;50:e51-e96
26. DelaysED providers correctly identify about 60% of children presenting with strokeIncorrectly diagnose ~40% with a mimicInitial providers correctly identified 38% of a cohort of children later found to have acute ischemic strokeFerriero et al. Stroke 2019;50:e51-e96Mackay et al. Expert Rev Neurother 2017;17:1157-1165 Rafay et al. Stroke 2009;40:58-64
27. Mitigating DelaysMackay et al. Health Syst 2019;10:73-88
28. Childhood StrokePresenting signs and symptoms are similar to adult strokeFocal deficits tend to be more common in stroke than mimicsHemiparesis and/or hemifacial weakness in 67%-90%Speech and/or language difficulty in 20%-50%Vision abnormality in 10%-15%Visual field deficit, diplopiaAtaxia in 8%-10%Frequency of nonspecific symptoms is similar in stroke and stroke mimicHeadache, altered mentationFerriero et al. Stroke 2019;50:e51-e96
29. Childhood StrokeNon-specific symptomsHeadache (20%-50%)Altered mentation (17%-38%)Seizure (15%-36%)More common in children than in adultsFerriero et al. Stroke 2019;50:e51-e96
30. Childhood Ischemic StrokePresent with focal deficits, like adultsChildren <6 years of age (particularly those <1 year of age) are more likely to present with seizures and altered mentationIn one English study, ≥70% of children <1 year of age with ischemic stroke presented with seizureSeizures may occur in 11%-52%Baldovsky and Okada J Am Coll Emerg Physicians Open 2020;1:1578-1586Mackay et al. Stroke 2016;47:2476-2481Mallick et al. J Neurol Neurosurg Psychiatry 2015; 86:917-921
31. Arteriopathic Ischemic StrokeFluctuating symptomsMoyamoya and similar arteriopathies are more likely to produce transient ischemic attacks, stuttering progression, silent infarctsHemiparesis and/or hemisensory deficits are common (~72%)Headaches in about 52%Seizures are less common (<10%)Ferriero et al. Stroke 2019;50:e51-e96
32. Cardiogenic Ischemic StrokeMore likely in the inpatient settingMore common in children 6 months to 3 yearsMore likely to be abrupt rather than stutteringSeizures in up to 40%Hemiparesis in 36%-75%Clinically silent in 14%-40%Discovered incidentallyFerriero et al. Stroke 2019;50:e51-e96
33. Posterior Circulation StrokeMost common at 7-8 years of ageMost are previously healthyMales account for 67%-77%Preceding minor head/neck trauma leading to vertebral artery dissectionSigns/symptoms that can localize to posterior circulation in ≥70%Ataxia, dysarthria, hemiparesis, oculomotor dysfunction, visual field deficitsNonspecific symptoms in 60%-70%Altered mentation, headache, vomitingFerriero et al. Stroke 2019;50:e51-e96
34. Childhood Hemorrhagic Stroke PresentationSuddenSevere headacheSigns of increased intracranial pressure (ICP)Nausea, vomiting, altered mentation/consciousness, optic disc edema Focal deficitsHemiparesis, language deficitsSeizures may occur in 15-41%Baldovsky and Okada J Am Coll Emerg Physicians Open 2020;1:1578-1586Ferriero et al. Stroke 2019;50:e51-e96Mackay et al. Stroke 2016;47:2476-2481
35. Childhood CSVT PresentationNon-specific, gradual-onset symptoms May be signs of increased ICPConfusion, lethargy, nausea, vomiting, headacheSeizuresDeficits that may result from increased ICPCranial nerve problems such as diplopia, pupil abnormalitiesFocal deficitsHemiparesis, visual field deficits, ataxia, speech/language deficitsBaldovsky and Okada J Am Coll Emerg Physicians Open 2020;1:1578-1586Ferriero et al. Stroke 2019;50:e51-e96
36. Polling QuestionStroke-like symptoms are more likely to be pediatric stroke than a mimic.YesNo
37. Polling QuestionWhat is the most frequent mimic of stroke-like symptoms?SeizureMigraineConversion disorderBell’s palsyInfectionTumorSomething else
38. Children with Acute Neurologic SymptomsMost children (60%-90%) with acute-onset neurologic symptoms do not have a stroke Ferriero et al. Stroke 2019;50:e51-e96 Mackay et al. Neurology 2014;82:1434-1440
39. Children with Acute Neurologic SymptomsA stroke mimic is more likely to cause acute neurologic problems in children than in adultsMackay et al. Neurology 2014;82:1434-1440
40. Stroke MimicsUp to 63% of stroke mimic patients have serious disease or time-sensitive treatment implicationsPosterior Reversible Encephalopathy Syndrome (PRES)Reversible Posterior Leukoencephalopathy Syndrome (RPLS)InfectionTumorIdiopathic Intracranial hypertensionEpilepsyFerriero et al. Stroke 2019;50:e51-e96 Shellhaas et al. Pediatrics 2006;118:704-709
41. Polling QuestionA thorough history and physical examination will reliably distinguish a stroke from a mimicTrueFalseIt depends
42. Sorting It OutMimics are less likely to have focal signsMimics are more likely to have pain/stiffness, impaired mentation, fever, involuntary movements, nausea, irritabilityBut some stroke patients will have these signs/symptomsHealthy in the week prior to presentation, inability to walk, focal face/arm weakness were likelier to be stroke Mackay et al. Stroke 2016;47:2476-2481
43. Sorting It OutSeizures (more common presenting problem) and loss of consciousness (less common) may not discriminate between stroke and mimic
44. Pediatric Stroke MimicsMigraine with auraBell’s palsySeizure with Todd’s paresis/paralysisBrain tumorDemyelinating diseaseMultiple sclerosisAcute disseminated encephalomyelitisCerebellitisEncephalitisEpidural abscessTraumatic brain injurySyncopeMetabolic diseasePsychogenic disorderHypoglycemiaPRES/RPLS
45. MimicsDisorderClinical Distinction from StrokeImaging Distinction from StrokeMigraineEvolving symptoms, visual/sensory complaints more common, nausea/vomiting, short duration and/or complete resolution, headache, personal/family historyTypically normalSeizurePositive symptoms, Todd’s paresis, post-ictal confusion, personal/family historyNormal or may identify prior insultInfectionFever, encephalopathy, gradual onset, meningismusNormal or signs of encephalitis/cerebritis (bilateral or diffuse). May see associated ischemic injury or CVSTDemyelinationGradual onset, multifocal symptoms, encephalopathy, accompanying optic neuritis and/or transverse myelitisMultifocal lesions, characteristic appearance (ADEM, MS, NMO), less likely to restrict diffusionHypertensive encephalopathyHistory of hypertension, bilateral visual symptoms, encephalopathyPosterior dominant findings, bilateral, may appear patchy, usually does not restrict diffusionVestibulopathySymptoms limits to vertigo and imbalance, gradual onsetNormalAcute cerebellar ataxiaSudden-onset bilaterally symmetric ataxia, post-viralNormalAlternating hemiplegia of childhoodHistory of contralateral events, choreoathetosis/dystonianormalDlamini and deVeber. in Nelson Textbook of Pediatrics 21st ed.
46. MimicsDlamini and deVeber. in Nelson Textbook of Pediatrics 21st ed.
47. MigraineHemiplegic migraineFamilial hemiplegic migraineMigraine with aura (complicated migraine)Visual, sensory, speech/language, motor aurasMigraine with brainstem aura
48. MigraineAuras may occur without the migrainous headache componentNeurologic deficits (auras) may come on slowlyMotor auras may occur <10%Altered mentation is rare (~3%)Auras usually resolve within 60 minutesMigraines more commonly have nausea/vomiting than strokesPersonal and/or family history can be usefulMackay et al. Dev Med Child Neurol 2018;60:1117-1122
49. SeizureFocal or generalized seizures may result in a post-ictal paralysis (Todd’s paresis)Todd’s paresis usually resolves over several hoursFocal seizures will, very rarely, result in only focal deficits as the symptomNew-onset seizures should prompt a thorough investigation as they can herald new strokesPersonal and/or family history can be useful
50. Demyelinating DiseaseAcute disseminated encephalomyelitis (ADEM), clinically isolate syndrome, multiple sclerosis, and neuromyelitis optica may all present with focal neurologic deficitsOnset is usually more gradualMultifocal deficits that would not localize to a reasonable lesion would point away from stroke
51. PRES/RPLSPosterior reversible encephalopathy syndrome (PRES) or reversible posterior leukoencephalopathy syndrome (RPLS) is typically seen in the setting of elevated blood pressuresAssociated with encephalopathy, seizures, headache, and vision troubleMay have focal deficits
52. PRES/RPLS
53. Rarer ConsiderationsATP1A3 Spectrum disordersAlternating hemiplegia of childhoodCAPOS (Cerebellar ataxia, Areflexia, Pes cavus, Optic atrophy, Sensorineural hearing loss)
54. Alternating Hemiplegia of ChildhoodOnset before 18 months (occurs in most patients before 12 months)Paroxysmal eventsVariable frequencyAbnormal eye movements can be first symptomFor example, monocular nystagmusRepeated attacks of hemiplegia involving either sideEpisodes of quadriplegiaImmediate resolution of symptoms upon sleepingDevelopmental delay or other neurologic abnormalitiesDystonia, choreoathetosis, ataxia Environmental triggersActivity, food, light, water, medication, etc.
55. Alternating Hemiplegia of ChildhoodAlpha 3 subunit of Na/K-ATPase is exclusively expressed in neuronsTheory: dysfunctional subunit can’t reestablish resting membrane potential after repeated action potentialsAbrupt paroxysmal symptoms of dystonia or hemiplegia50% of patients with AHC devlope epilepsy later in lifeMost patients experience progressive cognitive declineKetogenic diet potentially beneficial with AHC
56. ATP1A3 Spectrum MutationsConsider in any child presenting with episodic or fluctuating ataxia, weakness, dystoniaPresented with paroxysmal neurologic symptomsOculomotor abnormalitiesHypotoniaDystoniaSeizure-like episodesParalysisAtaxiaEncephalipathy
57. Case Presentation: ShaunPresented at 1600 Last known normal was just 10 minutes before 1500Non-focal examCT head is unremarkableCTA head/neck with severe focal stenosis of the proximal A1 branch of the left ACA and the proximal and distal M1 branch of the left MCA
58. Polling QuestionWhat do you think is going on?Ischemic StrokeHemorrhagic StrokeCVSTSeizureMigraineInfectionSomething else
59. Polling QuestionWhat is the next best step?Consent for administration of IV chemical thrombolysisAdmit for additional observation/investigationDischarge to homeObtain MRI head
60. Case Presentation: ShaunBecause Shaun’s condition seemed to improve over the interview and exam, suspicion for a stroke became lower. Additionally, his NIHSS of 5 for not being alert, getting orientation questions wrong, reading errors, and dysarthria was felt to be related to the previously administered midazolam.His CT head was unremarkable, and the focal stenosis of the proximal branches of the left ACA and MCA was felt to be inconsequential. Thus, the decision was to defer tPA because it was felt that the risks would have outweighed a potential benefit.He was admitted and an MRI head was ordered, to complete his workup.
61. Case Presentation: Shaun
62. Case Presentation: ShaunWhy did Shaun have his stroke? What was his mechanism?His CTA head had shown focal severe stenosis of the proximal branches of the left ACA and MCA. Conventional angiogram showed a “long segment of mild narrowing involving the left ICA ophthalmic and supraclinoid segments with complete occlusion of the proximal portion of the A1 segment and nonsignificant focal narrowing in the left MCA bifurcation.”This was felt to be consistent with focal cerebral arteriopathy.
63. Case Presentation: ShaunHe was discharged to acute rehabilitation on aspirin. Antithrombotic therapy is indicated in focal cerebral arteriopathy.Repeat CTA head in November 2019 showed stable stenosis in the ACA segments and improved stenosis in the MCA segments without evidence of new or worsened stenosis.
64. Case Presentation: Sam17-year-old boyNo significant medical history1200: goes to sleep1300: wakes up and has headache, left ear pain, facial numbness, and facial droop
65. Case Presentation: Sam1330: Presented to University Hospital for further evaluationLeft facial droopSlurred speechImproving headache pain
66. Case Presentation: SamExam is unremarkable aside from his left facial asymmetry and dysarthriaNIHSS= 1Facial weakness (1)Dysarthria (1)
67. Polling QuestionShould this patient be evaluated emergently for a stroke?YesNo
68. Polling QuestionWhat do you think is going on?Ischemic StrokeHemorrhagic StrokeCVSTSeizureMigraineInfectionBell’s PalsySomething else
69. Polling QuestionAt 17, do we treat him like a child or like an adult?ChildAdultAdult-sized child
70. Polling QuestionWhat would you like to do next?Image with CT head and CTA head/neckImage with MRI brainCheck my institution’s pediatric stroke algorithmCall a neurologistTalk to the family/patient more and try to get a better examConsent the patient/family for administration of IV chemical thrombolysis
71. Case Presentation: SamCT head and CTA head/neck were ordered as a stroke alertUnremarkableMRI head was unremarkable
72. Case Presentation: SamIdiopathic Bell’s palsyWithin window for IV chemical thrombolysis and mechanical thrombectomyLow NIHSS thoughRisks likely outweigh benefits for thrombectomyWhat about for IV chemical thrombolysis?
73. Questions???