January 2018

Four-year-old presents with right arm weakness when crying

History & Exam

  • A four-year-old girl with no previous medical history presents with two years of spells of right arm “clumsiness” and right facial droop after crying, which typically resolve in 5-10 minutes. After a recent fever and diarrhea from norovirus (in a preschool outbreak), the spells became more frequent and longer lasting, prompting imaging.
  • Review of further history was unremarkable other than noted Korean ancestry and mother reporting severe migraines and similar spells of left hand weakness at times of stress. The mother also had a sibling who died in childhood from a stroke. The patient has an older sibling, who is healthy.
  • Exam was normal.


Figure 1

Figure 2

Figure 3

Figure 4


Please submit your answers to see the case explanation.


  • Direct versus indirect revascularization procedures for moyamoya disease: a comparative effectiveness study.
  • Macyszyn L1, Attiah M1, Ma TS1, Ali Z1, Faught R1, Hossain A1, Man K1, Patel H1, Sobota R1, Zager EL1, Stein SC1.
  • J Neurosurg. 2017 May;126(5):1523-1529. doi: 10.3171/2015.8.JNS15504. Epub 2016 Jul 29.
  • PMID:27471892
  • Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series.
  • Gaillard J, Klein J, Duran D, Storey A, Scott RM, Kahle K, Smith ER.
  • J Neurosurg Pediatr. 2017 May;19(5):553-559. doi: 10.3171/2016.12.PEDS16468. Epub 2017 Mar 10.
  • PMID:28291427
  • National Analysis of 2454 Pediatric Moyamoya Admissions and the Effect of Hospital Volume on Outcomes.
  • Titsworth WL, Scott RM, Smith ER.
  • Stroke. 2016 May;47(5):1303-11. doi: 10.1161/STROKEAHA.115.012168. Epub 2016 Apr 5.
  • PMID:27048697
  • Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review.
  • Smith ER, Scott RM.
  • J Neurosurg Pediatr. 2012 Apr;9(4):353-60. doi: 10.3171/2011.12.PEDS1172. Review.
  • PMID: 22462697
  • Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young.
  • Roach ES, Golomb MR, Adams R, Biller J, Daniels S, Deveber G, Ferriero D, Jones BV, Kirkham FJ, Scott RM, Smith ER; American Heart Association Stroke Council; Council on Cardiovascular Disease in the Young.
  • Stroke. 2008 Sep;39(9):2644-91. doi: 10.1161/STROKEAHA.108.189696. Epub 2008 Jul 17. Review. Erratum in: Stroke. 2009 Jan 1;40(1):e8-10.
  • PMID:18635845


Recommended Products:

SANS Pediatric Module

SANS Pediatric Module for Residents

SANS Vascular Module

Nexus Occlusive Vascular Cases