Stroke does occur in Children: Children Incidence: 1 per 3,500 - - PowerPoint PPT Presentation

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Stroke does occur in Children: Children Incidence: 1 per 3,500 - - PowerPoint PPT Presentation

Aug 15, 2023 439 likes •520 views

2/10/2017 Patient X: History Part 1 Recent Advances in Previously healthy 14-year old boy Neurology While playing basketball with friends, he had a witnessed convulsion lasting six minutes. Difficult Cases Afterwards he had a mild

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SLIDE 1

2/10/2017 1

Recent Advances in Neurology Difficult Cases

Heather J. Fullerton, MD, MAS Professor of Neurology & Pediatrics Director, Pediatric Brain Center

Patient X: History Part 1

  • Previously healthy 14-year old boy
  • While playing basketball with friends, he had a

witnessed convulsion lasting six minutes.

  • Afterwards he had a mild headache and right-

sided weakness.

  • He was transported by EMS to a hospital where

he was found to be aphasic with a right hemiparesis.

2

Q1: True or false? A child with a seizure followed by hemiparesis can be observed without emergent brain imaging because the hemiparesis is most likely a Todd’s.

  • A. True
  • B. False

3

T r u e F a l s e

92% 8%

DDX for acute hemiparesis in a child – Migraine – Seizure (Post-ictal Todd’s)

– Stroke/TIA

– Brain tumor

– Super rare things: mitochondrial disorders (MELAS), channelopathies (alternating hemiplegia of childhood)…

  • Very hard to distinguish clinically because seizure and

headache common in children with acute stroke

  • A child with first-ever acute hemiparesis needs urgent brain

imaging to rule out stroke—even if preceding seizure or headache

4

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SLIDE 2

2/10/2017 2

Stroke does occur in Children:

  • Incidence:

– 4.6 per 100,000 children/year in US – About 5,000 US kids/year

– 1 per 3,500 neonates

Agrawal, Stroke, 2009

Half of strokes in kids are hemorrhagic

Hemorrhagic Ischemic

Children

Adults Ischemic Hemorrhagic Broderick, J Child Neuro, 1993

Approach to Imaging for Suspected Stroke

  • Emergent MRI with

DWI/ADC

  • Followed by

immediate brain MRA if + infarct

  • Especially if

within 6 hour thrombectomy window

  • More thorough

vascular imaging later

Approach to Imaging

  • What about CT/CTA?

– Use sparingly: radiation more concerning in young kids – Useful if MR can’t be done (pacer leads) or can’t be done quickly & thrombectomy would be considered – Sensitive for hemorrhage, not acute infarct – Don’t add CT perfusion (not worth the radiation)

10 hr old infarct in 5 month old with congenital heart dz

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SLIDE 3

2/10/2017 3 Patient X: History Part 2

Back to our 14 year old boy with a convulsion followed by right hemiparesis and aphasia…

  • Emergent MRI: small infarcts
  • f L MCA territory
  • MRA: mild narrowing of his

left supraclinoid internal carotid artery (ICA)— thrombus versus arteriopathy?

9

Q2: What is the most common cause of arterial ischemic stroke in a previously healthy child?

  • A. Embolism from congenital heart disease
  • B. Embolism from endocarditis
  • C. Sickle cell disease
  • D. Genetic thrombophilia
  • E. Arteriopathy

10 E m b

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i s m f r

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c

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g e n i t a . . . E m b

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i s m f r

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e n d

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a r d i t i s S i c k l e c e l l d i s e a s e G e n e t i c t h r

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b

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h i l i a A r t e r i

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a t h y

34% 5% 41% 2% 17%

Arteriopathy (disease of a cervical or cerebral vessel) is the most common cause

  • f childhood arterial ischemic stroke….

And the strongest predictor of recurrent stroke.

11

Kids with arteriopathy are at highest risk of recurrence

Fullerton, Stroke, 2015

0.00 0.25 0.50 0.75 1.00 Proportion recurrent stroke free

3 6 9 12 15 18 21 24

Time from index AIS to first recurrent stroke, months

Idiopathic Definite arteriopathy Possible arteriopathy Cardioembolic

Almost 1 in 4

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SLIDE 4

2/10/2017 4 Patient X: History Part 3

  • Admitted to the hospital and placed on IV heparin. An

echocardiogram was normal. His deficits improved dramatically over the next few days.

  • 3-days post stroke: severe left frontal headache.
  • 4-days post-stroke: agitated with worsened aphasia and right

hemiparesis.

  • His deficits were positional, improving when placed flat, and

responded to IV hydration and pressors.

  • Repeat MRI/A: new infarction in the left MCA territory and

severe narrowing of his left distal ICA and proximal MCA.

  • Transferred to UCSF

13

MRA on transfer

14

Q3: What causes rapidly progressive stenosis of the distal ICA in children?

  • A. Focal Cerebral Arteriopathy—Inflammatory (FCA-i), also

known as Transient Cerebral Arteriopathy (TCA)

  • B. Moyamoya disease (idiopathic moyamoya)
  • C. Moyamoya syndrome (secondary moyamoya)
  • D. Kawasaki disease

15 F

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a l C e r e b r a l A r t e r i

  • p

. . . M

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a m

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a d i s e a s e ( i d i

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e ( s . . . K a w a s a k i d i s e a s e

62% 14% 5% 18%

FCAi

16

Lenticulostriate infarcts Beading of M1 on conventional angiography

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SLIDE 5

2/10/2017 5 Natural History of FCA-i

  • Monophasic disease
  • Initial progression
  • Nonprogression after

6 months

  • Improvement or

stabilization; rarely normalization

– Chabrier & Sebire, J Child Neurol 1998; Danchaivijitr, Ann Neurol 2006

Courtesy of G. DeVeber

Acute 2 months 12 months

What causes FCA-i?

  • Varicella zoster virus (VZV)—strong evidence from

histopathology studies

  • Other herpesviruses, like herpes simplex virus, type 1

(HSV-1)—evidence from case reports and a prospective case- control study (Vascular effects of Infection in Pediatric Stroke, VIPS study)

  • Other pathogens?
  • Abnormal host immune response?

18

Q4: What are proven therapies for FCAi?

  • A. Aspirin
  • B. Revascularization Surgery (STA-MCA bypass)
  • C. Acyclovir
  • D. Corticosteroids
  • E. None of the above

19 A s p i r i n R e v a s c u l a r i z a t i

  • n

S u r g e r y . . . A c y c l

  • v

i r C

  • r

t i c

  • s

t e r

  • i

d s N

  • n

e

  • f

t h e a b

  • v

e

18% 9% 37% 23% 13%

Patient X: History Part 4

  • Admitted to the PICU, placed on IVF (1.5 x maintenance), head
  • f bed flat, on IV pressors
  • Had flow-dependent deficits: when upright, hemiparesis

worsened

  • Serologies positive for HSV-1
  • After several days of observation, with no improvement in flow-

dependent deficits, treated with IV corticosteroids and acylovir

  • After several days, patient stabilized
  • Slowly weaned off IVF and pressors
  • 2 month follow-up: dramatic improvement in ICA stenosis
  • 2 year follow-up: no further strokes

20

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SLIDE 6

2/10/2017 6 Follow-up MRA

21

Before his arteriopathy progressed, could we have diagnosed him with FCAi?

  • FCA differential diagnosis:

– FCAi – FCAd (focal cerebral arteriopathy, dissection subtype)

  • Intracranial dissection
  • Can also show early progression (spiral dissection)
  • Difficult to distinguish from FCAi
  • Vessel Wall Imaging (VWI) can help

– Early, unilateral moyamoya

  • Lenticulostriate collaterals
  • Chronic, silent infarcts

22

Enhancement on VWI: suggests FCAi

Courtesy of Max Wintermark, MD

Moyamoya disease/syndrome

  • Slow narrowing of

bilateral supraclinoid ICAs

  • 2ary lenticulostriate

collaterals

  • Can be unilateral at
  • nset
  • Secondary form: sickle

cell disease, Down syndrome, NF-1, cranial radiation for cancer

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SLIDE 7

2/10/2017 7 Childhood arteriopathy diagnosis important for management…and prevention of recurrent stroke

Aspirin Heparin

Revascularization Surgery (EDAS, STA-MCA bypass) Activity Restriction Hydration & BP

augmentation

FCAi x Rarely Acutely Dissection x

Sometimes

Lifelong Moyamoya x Routinely Long term

25

Clinical trials planned for corticosteroids and acyclovir for FCAi.

P.S. FCAi has also been reported in young adults