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Rasmussen Encephalitis
Miral D. Jhaveri, MD; Gary L. Hedlund, DO
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Rasmussen encephalitis (RE)
              • Synonyms

                • Chronic focal (localized) encephalitis
              • Definitions

                • Chronic, progressive, unilateral brain inflammation of uncertain etiology
                • Characterized by hemispheric volume loss and difficulty controlling focal seizure activity

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Leypoldt F et al: Autoimmune encephalopathies. Ann N Y Acad Sci. ePub, 2014
                                            2. Press C et al: The Janus-faced nature of Rasmussen's encephalitis. Semin Pediatr Neurol. 21(2):129-36, 2014
                                            3. Varadkar S et al: Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 13(2):195-205, 2014
                                            4. Wang DD et al: Unilateral holohemispheric central nervous system lesions associated with medically refractory epilepsy in the pediatric population: a retrospective series of hemimegalencephaly and Rasmussen's encephalitis. J Neurosurg Pediatr. 1-12, 2014
                                            5. Nabbout R: Autoimmune and inflammatory epilepsies. Epilepsia. 53 Suppl 4:58-62, 2012
                                            6. Takei H et al: Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature. Neuropathology. 30(4):381-91, 2010
                                            7. Cauley KA et al: Diffusion tensor imaging and tractography of Rasmussen encephalitis. Pediatr Radiol. 39(7):727-30, 2009
                                            8. Faingold R et al: MRI appearance of Rasmussen encephalitis. Pediatr Radiol. 39(7):756, 2009
                                            9. Terra-Bustamante VC et al: Rasmussen encephalitis: long-term outcome after surgery. Childs Nerv Syst. 25(5):583-9, 2009
                                            10. Tessonnier L et al: Perfusion SPECT findings in a suspected case of Rasmussen encephalitis. J Neuroimaging. 19(4):378-80, 2009
                                            11. Misra UK et al: The prognostic role of magnetic resonance imaging and single-photon emission computed tomography in viral encephalitis. Acta Radiol. 49(7):827-32, 2008
                                            12. Cook SW et al: Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients. J Neurosurg. 100(2 Suppl Pediatrics):125-41, 2004
                                            13. Granata T et al: Experience with immunomodulatory treatments in Rasmussen's encephalitis. Neurology. 61(12):1807-10, 2003
                                            14. Maeda Y et al: Rasmussen syndrome: multifocal spread of inflammation suggested from MRI and PET findings. Epilepsia. 44(8):1118-21, 2003
                                            15. Bien CG et al: Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology. Neurology. 58(2):250-7, 2002
                                            16. Kim SJ et al: A longitudinal MRI study in children with Rasmussen syndrome. Pediatr Neurol. 27(4):282-8, 2002
                                            17. Fiorella DJ et al: (18)F-fluorodeoxyglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis. AJNR Am J Neuroradiol. 22(7):1291-9, 2001
                                            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

                                                          • Rasmussen encephalitis (RE)
                                                        • Synonyms

                                                          • Chronic focal (localized) encephalitis
                                                        • Definitions

                                                          • Chronic, progressive, unilateral brain inflammation of uncertain etiology
                                                          • Characterized by hemispheric volume loss and difficulty controlling focal seizure activity

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Leypoldt F et al: Autoimmune encephalopathies. Ann N Y Acad Sci. ePub, 2014
                                                                                      2. Press C et al: The Janus-faced nature of Rasmussen's encephalitis. Semin Pediatr Neurol. 21(2):129-36, 2014
                                                                                      3. Varadkar S et al: Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 13(2):195-205, 2014
                                                                                      4. Wang DD et al: Unilateral holohemispheric central nervous system lesions associated with medically refractory epilepsy in the pediatric population: a retrospective series of hemimegalencephaly and Rasmussen's encephalitis. J Neurosurg Pediatr. 1-12, 2014
                                                                                      5. Nabbout R: Autoimmune and inflammatory epilepsies. Epilepsia. 53 Suppl 4:58-62, 2012
                                                                                      6. Takei H et al: Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature. Neuropathology. 30(4):381-91, 2010
                                                                                      7. Cauley KA et al: Diffusion tensor imaging and tractography of Rasmussen encephalitis. Pediatr Radiol. 39(7):727-30, 2009
                                                                                      8. Faingold R et al: MRI appearance of Rasmussen encephalitis. Pediatr Radiol. 39(7):756, 2009
                                                                                      9. Terra-Bustamante VC et al: Rasmussen encephalitis: long-term outcome after surgery. Childs Nerv Syst. 25(5):583-9, 2009
                                                                                      10. Tessonnier L et al: Perfusion SPECT findings in a suspected case of Rasmussen encephalitis. J Neuroimaging. 19(4):378-80, 2009
                                                                                      11. Misra UK et al: The prognostic role of magnetic resonance imaging and single-photon emission computed tomography in viral encephalitis. Acta Radiol. 49(7):827-32, 2008
                                                                                      12. Cook SW et al: Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients. J Neurosurg. 100(2 Suppl Pediatrics):125-41, 2004
                                                                                      13. Granata T et al: Experience with immunomodulatory treatments in Rasmussen's encephalitis. Neurology. 61(12):1807-10, 2003
                                                                                      14. Maeda Y et al: Rasmussen syndrome: multifocal spread of inflammation suggested from MRI and PET findings. Epilepsia. 44(8):1118-21, 2003
                                                                                      15. Bien CG et al: Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology. Neurology. 58(2):250-7, 2002
                                                                                      16. Kim SJ et al: A longitudinal MRI study in children with Rasmussen syndrome. Pediatr Neurol. 27(4):282-8, 2002
                                                                                      17. Fiorella DJ et al: (18)F-fluorodeoxyglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis. AJNR Am J Neuroradiol. 22(7):1291-9, 2001