1. Sudden Headache and Unresponsiveness in a 10 year-old boy Sudden Headache and Unresponsiveness in a 10 year-old boy J. Stephen Huff, MD, FACEP Emergency Medicine and Neurology University of Virginia Health System Charlottesville, Virginia

2. J. Stephen Huff, MD Case presentation… A 10 year old young man was having breakfast with his family. He complained of sudden onset of headache, walked to his mother, and became unresponsive. His father (a physician) said that he was unresponsive except for muttering incomprehensible words when stimulated. No abnormal movements were noticed.

3. J. Stephen Huff, MD Case presentation… Father carried the child to his car and drove him to the emergency department. The child arrived in the emergency department with diminished level of consciousness but with eyes open. He would not follow commands or speak but would look briefly at the examiner when questioned….

4. J. Stephen Huff, MD Case presentation… Pulse was 50; BP 100/62; temperature 36.3; oxygen saturation 99%. Pupils were equal, mid-position, and responsive to light. Gaze tended to be downward. The boy would withdraw the extremities to painful stimulation; muscle tone seemed diminished. He did not speak but would briefly look at people in the room….

5. J. Stephen Huff, MD What is your differential diagnosis? Anatomic? Pathophysiologic? Etiologic?

6. J. Stephen Huff, MD What is your differential diagnosis? Pathophysiologic / Etiologic? Subarachnoid hemorrhage Intracranial hemorrhage

7. J. Stephen Huff, MD What is your differential diagnosis? What is causing the lesion? Aneurysm? Arteriovenous malformation? Leukemia / blood dyscrasia? Medications? Tumor?

8. J. Stephen Huff, MD What is your management plan? Stabilization A, B, C’s Diagnostic plan?

9. J. Stephen Huff, MD What is your management plan? Stabilization A, B, C’s Diagnostic plan? Physician accompanies patient to CT with proper equipment…

10. J. Stephen Huff, MD

11. What is your CT diagnosis?

12. J. Stephen Huff, MD Cerebellar hemorrhage

13. J. Stephen Huff, MD What is your management plan? Stabilization A, B, C’s…. What now?

14. J. Stephen Huff, MD CT: Cerebellar hemorrhage Clinical-- Risk of sudden deterioration brainstem compression “Neurosurgical emergency”…

15. J. Stephen Huff, MD Cerebellar hemorrhage May be life threatening… Expanding mass posterior fossa… Brainstem compression… Sudden coma and apnea… Salvage possible… Lateral (hemispheric) better…

16. J. Stephen Huff, MD What is your management plan? Stabilization A, B, C’s…. Neurosurgical consultation… PICU….

17. J. Stephen Huff, MD

19. Arteriovenous malformations Posterior fossa AVM Origin- R superior cerebellar artery Drain-hemispheric veins Options ?

20. J. Stephen Huff, MD What is your management plan? Inpatient course… Diminished level of consciousness Repeat CT-hydrocephalus

21. J. Stephen Huff, MD Inpatient course Ventriculostomy…. Resection of AVM… Good clinical outcome….

22. J. Stephen Huff, MD DDX: Pediatric intracranial hemorrhage Aneurysms Blood dyscrasias Tumors Medications Arteriovenous malformations

23. J. Stephen Huff, MD Brain slice showing cross- section of large AVM

24. J. Stephen Huff, MD Brain slice showing intraparenchymal hemorrhage from AVM

25. J. Stephen Huff, MD Arteriovenous malformations Tangle of abnormal arteries and veins Linked by fistulas High-flow AV shunting Lacks capillary bed Congenital Course not predictable….

26. J. Stephen Huff, MD Arteriovenous malformations Variety of architectures Some small, close to surface Others large, wedge-shaped straddle vascular border zones extend to ventricular wall Associated aneurysms - about half

27. J. Stephen Huff, MD Arteriovenous malformations Present before age 40 Only 12% symptomatic 2% of strokes “Advances in…therapy…have come at a faster pace than information on the natural history…associated morbidity…and risks of invasive therapies.” The Arteriovenous Malformation Study Group: Arteriovenous malformations of the brain in adults. N Engl J Med. 1999;340:1812-8.

28. J. Stephen Huff, MD Arteriovenous malformations Presentations Hemorrhage most common Seizures Partial Generalized Headache - nonspecific Focal neurologic deficits The Arteriovenous Malformation Study Group: Arteriovenous malformations of the brain in adults. N Engl J Med. 1999;340:1812-8.

29. J. Stephen Huff, MD Arteriovenous malformations Re-hemorrhage -18% risk (vs. 2%) annual risk -may decline with time Posterior fossa AVM’s -about 7-18% of all AVM’s higher hemorrhage risk? higher morbidity? Symon L, et al: Arteriovenous malformations of the posterior fossa: a report on 28 cases and review of the literature. Brit J Neurosurg. 1995; 9:721

30. J. Stephen Huff, MD Arteriovenous malformations Treatment - (or not?) Surgery if superficial, non-eloquent Interventional radiology coils, glues, balloons Radiation therapy, gamma-knife Aminoff MJ: Treatment of unruptured cerebral arteriovenous malformations. Neurology. 1987; 37:815

31. J. Stephen Huff, MD Arteriovenous malformations Controlled trials of therapy lacking… Natural history unclear… Goal: complete removal AVM For symptomatic AVM’s -hemorrhage=>surgery if small -other therapies if larger If asymptomatic?

32. J. Stephen Huff, MD SAH and ICH can occur in children and young adults Different causes and treatments than in adults… AVM likely cause, not aneurysm Take home messages:

33. J. Stephen Huff, MD Questions?