link
Bookmarks
Sickle Cell Disease, Brain
Luke L. Linscott, MD; Anna Illner, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
6
1
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Sickle cell disease (SCD)
              • Definitions

                • Abnormality in hemoglobin (Hgb) → change in shape (sickling) → increased "stickiness" of erythrocytes (RBCs) → capillary occlusions, ischemia, infarctions, premature RBC destruction (hemolytic anemia)

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Angiographic Findings

                          • Nuclear Medicine Findings

                            • Imaging Recommendations

                              DIFFERENTIAL DIAGNOSIS

                                PATHOLOGY

                                • General Features

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Issar P et al: Conventional and advanced brain MR imaging in patients with sickle cell anemia. Indian J Radiol Imaging. 28(3):305-11, 2018
                                                2. Miao X et al: Increased brain iron deposition in patients with sickle cell disease: an MRI quantitative susceptibility mapping study. Blood. 132(15):1618-21, 2018
                                                3. Green NS et al: Enhanced long-term brain magnetic resonance imaging evaluation of children with sickle cell disease after hematopoietic cell transplantation. Biol Blood Marrow Transplant. 23(4):670-6, 2017
                                                4. Kawadler JM et al: Brain atrophy in paediatric sickle cell anaemia: findings from the silent infarct transfusion (SIT) trial. Br J Haematol. 177(1):151-3, 2017
                                                5. Andreotti C et al: The association of cytokine levels with cognitive function in children with sickle cell disease and normal MRI studies of the brain. J Child Neurol. 30(10):1349-53, 2015
                                                6. Chen R et al: A prospective longitudinal brain morphometry study of children with sickle cell disease. AJNR Am J Neuroradiol. 36(2):403-10, 2014
                                                7. Helton KJ et al: Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 124(6):891-8, 2014
                                                8. Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
                                                9. Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
                                                10. Arkuszewski M et al: Sickle cell anemia: reference values of cerebral blood flow determined by continuous arterial spin labeling MRI. Neuroradiol J. 26(2):191-200, 2013
                                                11. Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
                                                12. Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
                                                13. DeBaun MR et al: Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 119(20):4587-96, 2012
                                                14. Thangarajh M et al: Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia. Br J Haematol. 159(3):352-9, 2012
                                                15. Helton KJ et al: Arterial spin-labeled perfusion combined with segmentation techniques to evaluate cerebral blood flow in white and gray matter of children with sickle cell anemia. Pediatr Blood Cancer. 52(1):85-91, 2009
                                                16. Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
                                                17. Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
                                                18. Gold JI et al: Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging. Pediatr Hematol Oncol. 25(5):409-21, 2008
                                                19. Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
                                                20. Al-Kandari FA et al: Regional cerebral blood flow in patients with sickle cell disease: study with single photon emission computed tomography. Ann Nucl Med. 21(8):439-45, 2007
                                                21. Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
                                                22. Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
                                                23. Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
                                                24. Riebel T et al: Transcranial Doppler ultrasonography in neurologically asymptomatic children and young adults with sickle cell disease. Eur Radiol. 13(3):563-70, 2003
                                                25. Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
                                                26. Steen RG et al: Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. AJNR Am J Neuroradiol. 24(3):382-9, 2003
                                                27. Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
                                                28. Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
                                                29. Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990
                                                Related Anatomy
                                                Loading...
                                                Related Differential Diagnoses
                                                Loading...
                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Sickle cell disease (SCD)
                                                            • Definitions

                                                              • Abnormality in hemoglobin (Hgb) → change in shape (sickling) → increased "stickiness" of erythrocytes (RBCs) → capillary occlusions, ischemia, infarctions, premature RBC destruction (hemolytic anemia)

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic Findings

                                                                      • Angiographic Findings

                                                                        • Nuclear Medicine Findings

                                                                          • Imaging Recommendations

                                                                            DIFFERENTIAL DIAGNOSIS

                                                                              PATHOLOGY

                                                                              • General Features

                                                                                • Gross Pathologic & Surgical Features

                                                                                  • Microscopic Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Issar P et al: Conventional and advanced brain MR imaging in patients with sickle cell anemia. Indian J Radiol Imaging. 28(3):305-11, 2018
                                                                                              2. Miao X et al: Increased brain iron deposition in patients with sickle cell disease: an MRI quantitative susceptibility mapping study. Blood. 132(15):1618-21, 2018
                                                                                              3. Green NS et al: Enhanced long-term brain magnetic resonance imaging evaluation of children with sickle cell disease after hematopoietic cell transplantation. Biol Blood Marrow Transplant. 23(4):670-6, 2017
                                                                                              4. Kawadler JM et al: Brain atrophy in paediatric sickle cell anaemia: findings from the silent infarct transfusion (SIT) trial. Br J Haematol. 177(1):151-3, 2017
                                                                                              5. Andreotti C et al: The association of cytokine levels with cognitive function in children with sickle cell disease and normal MRI studies of the brain. J Child Neurol. 30(10):1349-53, 2015
                                                                                              6. Chen R et al: A prospective longitudinal brain morphometry study of children with sickle cell disease. AJNR Am J Neuroradiol. 36(2):403-10, 2014
                                                                                              7. Helton KJ et al: Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 124(6):891-8, 2014
                                                                                              8. Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
                                                                                              9. Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
                                                                                              10. Arkuszewski M et al: Sickle cell anemia: reference values of cerebral blood flow determined by continuous arterial spin labeling MRI. Neuroradiol J. 26(2):191-200, 2013
                                                                                              11. Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
                                                                                              12. Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
                                                                                              13. DeBaun MR et al: Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 119(20):4587-96, 2012
                                                                                              14. Thangarajh M et al: Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia. Br J Haematol. 159(3):352-9, 2012
                                                                                              15. Helton KJ et al: Arterial spin-labeled perfusion combined with segmentation techniques to evaluate cerebral blood flow in white and gray matter of children with sickle cell anemia. Pediatr Blood Cancer. 52(1):85-91, 2009
                                                                                              16. Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
                                                                                              17. Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
                                                                                              18. Gold JI et al: Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging. Pediatr Hematol Oncol. 25(5):409-21, 2008
                                                                                              19. Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
                                                                                              20. Al-Kandari FA et al: Regional cerebral blood flow in patients with sickle cell disease: study with single photon emission computed tomography. Ann Nucl Med. 21(8):439-45, 2007
                                                                                              21. Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
                                                                                              22. Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
                                                                                              23. Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
                                                                                              24. Riebel T et al: Transcranial Doppler ultrasonography in neurologically asymptomatic children and young adults with sickle cell disease. Eur Radiol. 13(3):563-70, 2003
                                                                                              25. Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
                                                                                              26. Steen RG et al: Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. AJNR Am J Neuroradiol. 24(3):382-9, 2003
                                                                                              27. Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
                                                                                              28. Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
                                                                                              29. Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990