Abstract
Sickle cell disease, although infrequent, is a disease with high comorbidity, stroke and vascular malformations by endothelial deformity are major and catastrophic complications in patients with sickle cell disease, which is why proper monitoring of these patients can prevent the high rate of complications that this disease leads. The situation of a 24 years old african-american woman with sickle cell disease and several strokes during adolescence is exposed. The patient had a complicated pregnancy due to secondary intraventricular hemorrhage and multiple vascular malformations type Moyamoya. She developed a convulsive status epilepticus and an irreversible neurological impairment.
References
VILLERS MS, JAMISON MG, DE CASTRO LM, JAMES AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199:e1-125.e5
Informe de la reunión de un grupo de expertos en Genética Médica (La Habana, Cuba, octubre 9-11 de 1987) OPS, OMS, 30 pags. Ejecución de las actividades de Salud Genética en América Latina y el Caribe.
OHENE-FREMPONG K, WEINER SJ, SLEEPER LA, MILLER ST, EMBURY S, MOOHR JW, ET AL. Cerebrovascular Accidents in Sickle Cell Disease: Rates and Risk Factors. Blood 1998; 91: 288-294.
BATEMAN BT, SCHUMACHER HC, BUSH-NELL CD, PILE-SPELLMAN J, SIMPSON LL, ET AL. Intracerebral hemorrhage in pregnancy: Frequency, risk factors, and outcome. Neurology 2006; 67:424-429.
SCOTT RM, SMITH ER. Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009; 19: 360:1226-1237.
GREGORY J ZIPFEL, DOUGLAS J FOX JR, DENNOS J RIVET. Moyamoya Disease in Adults: The Role of Cerebral Revascularization. Skull Base 2005;15 : 27-41.
DOBSON SR, HOLDEN KR, NIETERT PJ, CURE JK, LAVER JH, ET AL. Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events. Blood 2002; 99: 3144-3150.
LANZKRON S, STROUSE JJ, WILSON R, BEACH MC, HAYWOOD C, ET AL. Systematic Review: Hydroxyurea for the Treatment of Adults with Sickle Cell Disease. Ann Intern Med. 2008; 148: 939-955.
CHARACHE S, TERRIN ML, MOORE RD, DOVER GJ, BARTON FB, ET AL. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995; 332: 1317-1322.
ADAMS RJ, MCKIE VC, HSU L, FILES B, VICHINSKY E, PEGELOW C, ET AL. Prevention of first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 1998; 339:5-11.
SHANNON W, KEITH C. Quirolo Current issues in blood transfusion for sickle cell disease. Current Opinion in Pediatrics 2009; 21:15-21.
HANKINSON TC, BOHMAN LE, HEYER G, LICURSI M, GHATAN S, ET AL. Surgical treatment of moya moya síndrome in patients with sickle cell disease: outcome following encephaloduroarterio-synangiosis. J Neurosurg Pediatrics 2008; 1: 211-216.
SCHMUGGE, M. Stroke in hemoglobin (SD) sickle cell disease with moyamoya: successful hydroxyurea treatment after cerebrovascular bypass surgery. Blood. 2001; 97: 2165-2167.
BATEMAN BT, CLAASSEN J, WILLEY JZ, HIRSCH LJ, MAYER SA, ET AL. Convulsive status epilepticus after ischemic stroke and intracerebral hemorrhage: frequency, predictors, and impact on outcome in a large administrative dataset. Neurocrit Care 2007; 7: 187-193.
SWITZER JA, HESS DC, NICHOLS FT, ADAMS RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol 2006; 5: 501-512.
ROGERS DT, MOLOKIE R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am. 2010;37:223-37
HOWARD J, OTENG-NTIM E. The obstetric management of sickle cell disease. Best Pract Res Clin Obstet Gynaecol. 2012; 26: 25-36.
SHETH SS, SHETH KN. Treatment of Neu-rocritical Care Emergencies in Pregnancy. Curr Treat Options Neurol. 2012 Feb 2. (Epub ahead of print)

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
