1. Intern Seminar Presenter: intern 傅蓓安 Supervisor: 杜依芳 醫師
Date: 2016/08/26

2. Basic Information
Name: 陳O辰
Chart No.: 1279XXXX
Age: 7 years old

3. Recurrent but transient stroke-like episodes and seizures
2008
(Birth)
2010
(2y/o)
2015
(7y/o)
Recurrent but transient stroke-like episodes and seizures
Diagnosed with Sturge-Weber syndrome, with congenital glaucoma

4. Falling event, vomiting, fever with URI Sx
2016
05/08
05/15
Falling event, vomiting, fever with URI Sx
Conscious disturbance, left side weakness, complex partial seizures
GCS E3V1M4
Muscle power: RUL/RLL 3-4/3-4, LUL/LLL: 1/1
DTR: all +, Babinski sign: -/-
CT: no ICH

5. T1 3D
T2 Flair

6. DWI
ADC

7. muscle power recovered
2016
05/08
05/15
muscle power recovered
LUL/LLL: 2/4

8. Head trauma(-), vomiting, fever with URI Sx
2016
07/21
08/01
Head trauma(-), vomiting, fever with URI Sx
Conscious disturbance, slurred speach, right side weakness, complex partial seizures
GCS E4V4M5
Muscle power: RUL/RLL 1/1, LUL/LLL 3/3
DTR: all ++, Babinski sign: -/-
Dysphagia

9. T1 3D with contrast
T2 Flair

10. DWI
ADC

11. Muscle power improved on day 7 RUL/RLL: 3/3 before discharge
2016
07/21
08/01
Muscle power improved on day 7 RUL/RLL: 3/3 before discharge
Dysphagia improved on day 11

12. Conscious disturbance
Head
contusion
Conscious disturbance
vomiting
hemiparesis
URI
seizure

13. Discussion Sturge-Weber syndrome (SWS) & Its stroke-like episode
Sturge-Weber syndrome (SWS)
& Its stroke-like episode

14. Outline Overview of Sturge-Weber syndrome
Risk factors associated with its stroke-like episode
Prevention and control for stroke-like episode

15. Overview of Sturge-Weber syndrome
Congenital vascular malformation disease
1/20,000-50,000, sporadic
Neurocutaneous syndrome
Leptomeningeal
angiomatosis
Facial cutaneous vascular malformation (port-wine stain)
[1]Pediatr Neurol 2004;30: , [2] EJPN 18(2014)

16. Leptomeningeal angiomatosis
Arachnoid mater
Pia mater

17. Facial cutaneous vascular malformation (port-wine stain)
Occuring in 0.3% live birth
If PWS involving entire CN V1 distribution  78% with neurological/ocular disorders

18. Pathogenesis of SWS
GNAQ gene single-nucleotide mutation (c.548GA, p.Arg183Gln) ?
Failure of primitive cephalic venous plexus to regress in first trimester
NEJM 2013;368:1971-9

19. Pathogenesis of SWS Ectoderm forming upper portion of facial skin
Neural tube
forming parietal-occipital brain

20. Atrophy /dystrophy
Lack of superficial cortical veins
Ischemia
CBF↓
Venous stasis
Venous outflow ↓
High flow to deep venous system

21. Neurological complication in SWS
Stroke-like episode
Stroke-like episode
Seizure
Mental retardation
Behavioral problem

22. Stroke-like episode in SWS
Transient focal deficits
Hemiparesis, visual field defect
Duration: days, weeks, or months

23. Causes for stroke-like episode
Venous stasis
Venous thrombosis
Stroke-like
episode
Seizure
Head trauma

24. Causes for stroke-like episode
Venous stasis
Venous thrombosis
Stroke-like
episode
Seizure
Head trauma

25. DWI
ADC
The Neurologist 2009;15: 351–354

27. Possible risk factors causing venous stasis
Prothrombotic status
Drugs
Parameningeal infection (ear, sinus, mouth, face and neck)
Local compression
Intracranial hypotension
Other systemic diseases (SLE, nephrotic syndrome, thyroid disease…)
Stroke. 2011;42:

28. Causes for stroke-like episode
Venous stasis
Venous thrombosis
Stroke-like
episode
Seizure
Head trauma

29. Seizure & stroke-like symptoms
left
Rt ↓43%, Lt ↓18%,
Left
Rt↑57%, Lt ↑166%
Developmental Medicine & Child Neurology 1999, 41: 480–485

30. Causes for stroke-like episode
Venous stasis
Venous thrombosis
Stroke-like
episode
Seizure
Head trauma

31. Head trauma & stroke-like symptoms
Case series of 6 patients
Hypothesized mechanism: 1. Cerebral edema 2. Vascular leakage induced hemiplegic migraine
Developmental Medicine & Child Neurology 2007, 49: 697–699

32. Prevention of stroke-like episode
Control of stroke-like episodes is key for preventing neurological injury progression
Rehab/adaptive training

33. Prevention of stroke-like episode
Low-dose antiplatelet
Venous stasis
Venous thrombosis
Stroke-like
episode
Seizure
Head trauma

34. Stroke-like episodes: 1.1  0.3/month
J Child Neurol. 26(6)

35. Side-effect n=9 Bruising Epitaxis n=6 No side-effect n=58
J Child Neurol February ; 28(2): 213–218

36. Prevention of stroke-like episode
Venous stasis
Venous thrombosis
AED
Seizure
Stroke-like
episode
Operation
Head trauma

37. Anti-epileptic drug (AED)
Prophylactic AED was not recommended
BZD  phenytoin
Fever control Good hydration Good oxygenation Sleep hygiene
Eu J Ped Neurol. 18(2014)

38. Epilepsy surgery Lesionectomy, hemispherectomy
7-8% SWS patients received surgery

39. Prevention of stroke-like episode
Venous stasis
Venous thrombosis
Seizure
Stroke-like
episode
Head trauma
Head protection

40. Venous stasis/ thrombosis
Back to our case
Vaccination, antipyretics, hydration
Head contusion
Vomiting
URI
protection
Venous stasis/ thrombosis
Stroke-like episode
Seizure
AED
Low-dose antiplatelet
Surgery

42. Q &A
Thanks for listening