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Sickle Cell Disease, Brain
Anna Illner, MD
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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. 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. ePub, 2014
                                                2. Chen R et al: A Prospective Longitudinal Brain Morphometry Study of Children with Sickle Cell Disease. AJNR Am J Neuroradiol. ePub, 2014
                                                3. 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
                                                4. Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
                                                5. Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
                                                6. 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
                                                7. Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
                                                8. Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
                                                9. 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
                                                10. 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
                                                11. 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
                                                12. Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
                                                13. Trompeter S et al: Haemoglobin F modulation in childhood sickle cell disease. Br J Haematol. 144(3):308-16, 2009
                                                14. Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
                                                15. 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
                                                16. Michlitsch JG et al: Recent advances in bone marrow transplantation in hemoglobinopathies. Curr Mol Med. 8(7):675-89, 2008
                                                17. Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
                                                18. Sadelain M et al: Stem cell engineering for the treatment of severe hemoglobinopathies. Curr Mol Med. 8(7):690-7, 2008
                                                19. 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
                                                20. Henry M et al: Pseudotumor cerebri in children with sickle cell disease: a case series. Pediatrics. 113(3 Pt 1):e265-9, 2004
                                                21. Alam M et al: Cerebrovascular accident in sickle cell disease. J Coll Physicians Surg Pak. 13(1):55-6, 2003
                                                22. Driscoll MC et al: Stroke risk in siblings with sickle cell anemia. Blood. 101(6):2401-4, 2003
                                                23. Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
                                                24. Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
                                                25. Moster ML: Coagulopathies and arterial stroke. J Neuroophthalmol. 23(1):63-71, 2003
                                                26. Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
                                                27. 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
                                                28. Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
                                                29. 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
                                                30. Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
                                                31. Yerys BE et al: Memory strategy training in children with cerebral infarcts related to sickle cell disease. J Pediatr Hematol Oncol. 25(6):495-8, 2003
                                                32. Carvalho KS et al: Arterial strokes in children. Neurol Clin. 20(4):1079-100, vii, 2002
                                                33. Fixler J et al: Sickle cell disease. Pediatr Clin North Am. 49(6):1193-210, vi, 2002
                                                34. Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
                                                35. Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990
                                                36. Rothman SM et al: Sickle cell anemia and central nervous system infarction: a neuropathological study. Ann Neurol. 20(6):684-90, 1986
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                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. 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. ePub, 2014
                                                                                              2. Chen R et al: A Prospective Longitudinal Brain Morphometry Study of Children with Sickle Cell Disease. AJNR Am J Neuroradiol. ePub, 2014
                                                                                              3. 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
                                                                                              4. Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
                                                                                              5. Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
                                                                                              6. 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
                                                                                              7. Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
                                                                                              8. Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
                                                                                              9. 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
                                                                                              10. 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
                                                                                              11. 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
                                                                                              12. Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
                                                                                              13. Trompeter S et al: Haemoglobin F modulation in childhood sickle cell disease. Br J Haematol. 144(3):308-16, 2009
                                                                                              14. Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
                                                                                              15. 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
                                                                                              16. Michlitsch JG et al: Recent advances in bone marrow transplantation in hemoglobinopathies. Curr Mol Med. 8(7):675-89, 2008
                                                                                              17. Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
                                                                                              18. Sadelain M et al: Stem cell engineering for the treatment of severe hemoglobinopathies. Curr Mol Med. 8(7):690-7, 2008
                                                                                              19. 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
                                                                                              20. Henry M et al: Pseudotumor cerebri in children with sickle cell disease: a case series. Pediatrics. 113(3 Pt 1):e265-9, 2004
                                                                                              21. Alam M et al: Cerebrovascular accident in sickle cell disease. J Coll Physicians Surg Pak. 13(1):55-6, 2003
                                                                                              22. Driscoll MC et al: Stroke risk in siblings with sickle cell anemia. Blood. 101(6):2401-4, 2003
                                                                                              23. Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
                                                                                              24. Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
                                                                                              25. Moster ML: Coagulopathies and arterial stroke. J Neuroophthalmol. 23(1):63-71, 2003
                                                                                              26. Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
                                                                                              27. 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
                                                                                              28. Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
                                                                                              29. 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
                                                                                              30. Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
                                                                                              31. Yerys BE et al: Memory strategy training in children with cerebral infarcts related to sickle cell disease. J Pediatr Hematol Oncol. 25(6):495-8, 2003
                                                                                              32. Carvalho KS et al: Arterial strokes in children. Neurol Clin. 20(4):1079-100, vii, 2002
                                                                                              33. Fixler J et al: Sickle cell disease. Pediatr Clin North Am. 49(6):1193-210, vi, 2002
                                                                                              34. Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
                                                                                              35. Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990
                                                                                              36. Rothman SM et al: Sickle cell anemia and central nervous system infarction: a neuropathological study. Ann Neurol. 20(6):684-90, 1986