Welcome to CNS Case of the Month Section. The purpose of this Web area is to provide a forum for discussion and debate regarding the management of both ordinary and extraordinary cases. There are no right or wrong answers, but rather, there are various opinions on what should be the "standard of care." The answers given will be collected anonymously, and be presented in a graphical format at the end of each month. We will then see the spectrum of thought regarding the management of these patients.
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Brian L. Hoh, MD University of Florida
Ashok R. Asthagiri, MD Associate Professor Department of Neurological Surgery University of Virginia
Maryam Rahman, MD MS Assistant Professor
Department of Neurosurgery
University of Florida
Alexander A. Khalessi MD, MS University of California San Diego
Wilson Z. Ray, MD Assistant Professor in Neurosurgery Department of Neurological Surgery Washington University School of Medicine
Edward R. Smith, MD Director, Pediatric Cerebrovascular Surgery Department of Neurosurgery Children's Hospital Boston / Harvard Medical School
December 2013: Exertional Weakness in a Child
12-year-old child with a history of sickle cell disease, on exchange transfusion therapy, presents with progressive headaches over 1 year
With recent exchange transfusions, he developed bilateral arm weakness for 1-2 hours afterwards
Recent sports activity results in brief periods of profound arm weakness, especially on the right side, and word finding problems.
Figure 1: [Click to view larger image]
a. Sickle cell related thrombus and infarction
b. Moyamoya syndrome
c. Transfusion reaction
e. Aneurysmal hemorrhage
2. What test would you order next?
a. Transcranial Doppler
d. Hemoglobin sickle cell (HgBSS) fraction
e. Diamox SPECT
3. What treatment would you offer?
a. Increased transfusion frequency
b. Medical therapy with aspirin and hydration
c. Indirect revascularization using the superficial temporal artery (STA)
d. Direct revascularization with a STA-MCA bypass
e. Bone marrow transplant
4. Which of the following describes you?
b. Private practice
5. I practice in one of the following locations.
a. I practice in one of the following states: WA, OR, CA, AK, HI
b. I practice in one of the following states: AZ, ID, NV, MT, WY, CO, NM, UT
c. I practice in one of the following states: ND, SD, NE, KS, OK, MN, IA, MO, WI, IL MI, IN, OH
d. I practice in one of the following states: TX, AR, AL, LA, KY, TN, MS, WV, NC, SC, GA, FL
e. I practice in one of the following states: ME, VT, NH, MA, RI, CT, NJ, NY, PA, DE, MD, DC, VA
f. I practice in Mexico or Canada
g. I practice in South America
h. I practice in Europe
i. I practice in Asia
j. I practice in Australia/Pacific Rim
k. I practice in Africa
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Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis. Hankinson TC, Bohman LE, Heyer G, Licursi M, Ghatan S, Feldstein NA, Anderson RC. J Neurosurg Pediatr. 2008 Mar;1(3):211-6. doi: 10.3171/PED/2008/1/3/211.
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.
Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Smith ER, McClain CD, Heeney M, Scott RM. Neurosurg Focus. 2009 Apr;26(4):E10. doi: 10.3171/2009.01.FOCUS08307.
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