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Autoimmune Encephalitis
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Paraneoplastic syndromes (PS), paraneoplastic disease
          • Definitions

            • Autoimmune encephalitis (AE): Immune-mediated disease with antibody-mediated inflammation of brain
              • Includes autoimmune and paraneoplastic encephalitis
            • Paraneoplastic: Remote neurologic effects of cancer, associated with extra-CNS tumors
              • Most common tumor: Small cell lung carcinoma
            • Limbic encephalitis (LE) is most common clinical paraneoplastic syndrome
              • Only PS with clearly defined imaging features

          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

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Iorio R et al: Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic etiology. Eur J Neurol. ePub, 2020
                                          2. Mongay-Ochoa N et al: Anti-Hu-associated paraneoplastic syndromes triggered by immune-checkpoint inhibitor treatment. J Neurol. ePub, 2020
                                          3. Vogrig A et al: Central nervous system complications associated with immune checkpoint inhibitors. J Neurol Neurosurg Psychiatry. ePub, 2020
                                          4. Bradshaw MJ et al: An overview of autoimmune and paraneoplastic encephalitides. Semin Neurol. 38(3):330-43, 2018
                                          5. Long JM et al: Autoimmune dementia. Semin Neurol. 38(3):303-15, 2018
                                          6. Kelley BP et al: Autoimmune encephalitis: pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol. 38(6):1070-8, 2017
                                          7. da Rocha AJ et al: Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders. AJNR Am J Neuroradiol. 36(12):2196-205, 2015
                                          8. Höftberger R et al: Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 84(24):2403-12, 2015
                                          9. Thomas AC et al: Autoimmune limbic encephalitis detected on FDG brain scan performed for the evaluation of dementia. Clin Nucl Med. 40(4):358-9, 2015
                                          10. Zekeridou A et al: Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis. J Neurol. 262(8):1859-66, 2015
                                          11. Kotsenas AL et al: MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis. AJNR Am J Neuroradiol. 35(1):84-9, 2014
                                          12. Masangkay N et al: Brain 18F-FDG-PET characteristics in patients with paraneoplastic neurological syndrome and its correlation with clinical and MRI findings. Nucl Med Commun. 35(10):1038-46, 2014
                                          13. Sarria-Estrada S et al: Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis. Clin Radiol. 69(8):795-803, 2014
                                          14. Baumgartner A et al: Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol. 260(11):2744-53, 2013
                                          15. Aye MM et al: CD8 positive T-cell infiltration in the dentate nucleus of paraneoplastic cerebellar degeneration. J Neuroimmunol. 208(1-2):136-40, 2009
                                          16. Berger JR et al: A brainstem paraneoplastic syndrome associated with prostate cancer. J Neurol Neurosurg Psychiatry. 80(8):934-5, 2009
                                          17. Damek DM: Cerebral edema, altered mental status, seizures, acute stroke, leptomeningeal metastases, and paraneoplastic syndrome. Emerg Med Clin North Am. 27(2):209-29, 2009
                                          18. Honnorat J et al: Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry. 80(4):412-6, 2009
                                          19. Jaster JH: Reversible autoimmune encephalopathy spectrum. Arch Neurol. 66(7):916, 2009
                                          20. Khan NL et al: Histopathology of VGKC antibody-associated limbic encephalitis. Neurology. 72(19):1703-5, 2009
                                          21. Kröll-Seger J et al: Non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis in a pre-pubertal girl. Epileptic Disord. 11(1):54-9, 2009
                                          22. McKeon A et al: Reversible extralimbic paraneoplastic encephalopathies with large abnormalities on magnetic resonance images. Arch Neurol. 66(2):268-71, 2009
                                          23. Modoni A et al: Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies. Cogn Behav Neurol. 22(1):63-6, 2009
                                          24. Saiz A et al: Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry. 80(4):404-7, 2009
                                          25. Uribe-Uribe NO et al: Paraneoplastic sensory neuropathy associated with small cell carcinoma of the gallbladder. Ann Diagn Pathol. 13(2):124-6, 2009
                                          26. Anderson NE et al: Limbic encephalitis - a review. J Clin Neurosci. 15(9):961-71, 2008
                                          27. Basu S et al: Role of FDG-PET in the clinical management of paraneoplastic neurological syndrome: detection of the underlying malignancy and the brain PET-MRI correlates. Mol Imaging Biol. 10(3):131-7, 2008
                                          28. Blaes F et al: Autoantibodies in childhood opsoclonus-myoclonus syndrome. J Neuroimmunol. 201-202:221-6, 2008
                                          29. Corapcioglu F et al: Response to rituximab and prednisolone for opsoclonus-myoclonus-ataxia syndrome in a child with ganglioneuroblastoma. Pediatr Hematol Oncol. 25(8):756-61, 2008
                                          30. Dalakas MC: Invited article: inhibition of B cell functions: implications for neurology. Neurology. 70(23):2252-60, 2008
                                          31. Eker A et al: Testicular teratoma and anti-N-methyl-D-aspartate receptor-associated encephalitis. J Neurol Neurosurg Psychiatry. 79(9):1082-3, 2008
                                          32. Fitzpatrick AS et al: Opsoclonus-myoclonus syndrome associated with benign ovarian teratoma. Neurology. 70(15):1292-3, 2008
                                          33. Geschwind MD et al: Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol. 65(10):1341-6, 2008
                                          34. Hassan KA et al: Long-term survival in paraneoplastic opsoclonus-myoclonus syndrome associated with small cell lung cancer. J Neuroophthalmol. 28(1):27-30, 2008
                                          35. Jarius S et al: Relative frequency of VGKC and 'classical' paraneoplastic antibodies in patients with limbic encephalitis. J Neurol. 255(7):1100-1, 2008
                                          36. Johnson V et al: Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma. J Neurooncol. 90(3):279-81, 2008
                                          37. Ko MW et al: Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 28(1):58-68, 2008
                                          38. Musunuru K et al: Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma. J Neurooncol. 90(2):213-6, 2008
                                          39. Novillo-López ME et al: Treatment-responsive subacute limbic encephalitis and NMDA receptor antibodies in a man. Neurology. 70(9):728-9, 2008
                                          40. Pellkofer HL et al: Lambert-eaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel. J Neuroimmunol. 204(1-2):136-9, 2008
                                          41. Sabater L et al: SOX1 antibodies are markers of paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology. 70(12):924-8, 2008
                                          42. Sekiguchi Y et al: Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction. J Neurol Sci. 269(1-2):176-9, 2008
                                          43. Tan KM et al: Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 70(20):1883-90, 2008
                                          44. Darnell RB et al: Paraneoplastic syndromes affecting the nervous system. Semin Oncol. 33(3):270-98, 2006
                                          45. Dropcho EJ: Update on paraneoplastic syndromes. Curr Opin Neurol. 18(3):331-6, 2005
                                          46. Dadparvar S et al: Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings. Clin Nucl Med. 28(11):893-6, 2003
                                          47. Messori A et al: Resolution of limbic encephalitis with detection and treatment of lung cancer: clinical-radiological correlation. Eur J Radiol. 45(1): 78-80, 2003
                                          48. Barnett M et al: Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody. J Neurol Neurosurg Psychiatry. 70(2):222-5, 2001
                                          49. Gultekin SH et al: Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 123 ( Pt 7):1481-94, 2000
                                          50. Dalmau J et al: Paraneoplastic neurologic syndromes: pathogenesis and physiopathology. Brain Pathol. 9(2):275-84, 1999
                                          51. Scaravilli F et al: The neuropathology of paraneoplastic syndromes. Brain Pathol. 9(2):251-60, 1999
                                          52. Voltz R et al: A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 340(23): 1788-95, 1999
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Paraneoplastic syndromes (PS), paraneoplastic disease
                                                  • Definitions

                                                    • Autoimmune encephalitis (AE): Immune-mediated disease with antibody-mediated inflammation of brain
                                                      • Includes autoimmune and paraneoplastic encephalitis
                                                    • Paraneoplastic: Remote neurologic effects of cancer, associated with extra-CNS tumors
                                                      • Most common tumor: Small cell lung carcinoma
                                                    • Limbic encephalitis (LE) is most common clinical paraneoplastic syndrome
                                                      • Only PS with clearly defined imaging features

                                                  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

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Iorio R et al: Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic etiology. Eur J Neurol. ePub, 2020
                                                                                  2. Mongay-Ochoa N et al: Anti-Hu-associated paraneoplastic syndromes triggered by immune-checkpoint inhibitor treatment. J Neurol. ePub, 2020
                                                                                  3. Vogrig A et al: Central nervous system complications associated with immune checkpoint inhibitors. J Neurol Neurosurg Psychiatry. ePub, 2020
                                                                                  4. Bradshaw MJ et al: An overview of autoimmune and paraneoplastic encephalitides. Semin Neurol. 38(3):330-43, 2018
                                                                                  5. Long JM et al: Autoimmune dementia. Semin Neurol. 38(3):303-15, 2018
                                                                                  6. Kelley BP et al: Autoimmune encephalitis: pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol. 38(6):1070-8, 2017
                                                                                  7. da Rocha AJ et al: Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders. AJNR Am J Neuroradiol. 36(12):2196-205, 2015
                                                                                  8. Höftberger R et al: Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 84(24):2403-12, 2015
                                                                                  9. Thomas AC et al: Autoimmune limbic encephalitis detected on FDG brain scan performed for the evaluation of dementia. Clin Nucl Med. 40(4):358-9, 2015
                                                                                  10. Zekeridou A et al: Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis. J Neurol. 262(8):1859-66, 2015
                                                                                  11. Kotsenas AL et al: MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis. AJNR Am J Neuroradiol. 35(1):84-9, 2014
                                                                                  12. Masangkay N et al: Brain 18F-FDG-PET characteristics in patients with paraneoplastic neurological syndrome and its correlation with clinical and MRI findings. Nucl Med Commun. 35(10):1038-46, 2014
                                                                                  13. Sarria-Estrada S et al: Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis. Clin Radiol. 69(8):795-803, 2014
                                                                                  14. Baumgartner A et al: Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol. 260(11):2744-53, 2013
                                                                                  15. Aye MM et al: CD8 positive T-cell infiltration in the dentate nucleus of paraneoplastic cerebellar degeneration. J Neuroimmunol. 208(1-2):136-40, 2009
                                                                                  16. Berger JR et al: A brainstem paraneoplastic syndrome associated with prostate cancer. J Neurol Neurosurg Psychiatry. 80(8):934-5, 2009
                                                                                  17. Damek DM: Cerebral edema, altered mental status, seizures, acute stroke, leptomeningeal metastases, and paraneoplastic syndrome. Emerg Med Clin North Am. 27(2):209-29, 2009
                                                                                  18. Honnorat J et al: Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry. 80(4):412-6, 2009
                                                                                  19. Jaster JH: Reversible autoimmune encephalopathy spectrum. Arch Neurol. 66(7):916, 2009
                                                                                  20. Khan NL et al: Histopathology of VGKC antibody-associated limbic encephalitis. Neurology. 72(19):1703-5, 2009
                                                                                  21. Kröll-Seger J et al: Non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis in a pre-pubertal girl. Epileptic Disord. 11(1):54-9, 2009
                                                                                  22. McKeon A et al: Reversible extralimbic paraneoplastic encephalopathies with large abnormalities on magnetic resonance images. Arch Neurol. 66(2):268-71, 2009
                                                                                  23. Modoni A et al: Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies. Cogn Behav Neurol. 22(1):63-6, 2009
                                                                                  24. Saiz A et al: Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry. 80(4):404-7, 2009
                                                                                  25. Uribe-Uribe NO et al: Paraneoplastic sensory neuropathy associated with small cell carcinoma of the gallbladder. Ann Diagn Pathol. 13(2):124-6, 2009
                                                                                  26. Anderson NE et al: Limbic encephalitis - a review. J Clin Neurosci. 15(9):961-71, 2008
                                                                                  27. Basu S et al: Role of FDG-PET in the clinical management of paraneoplastic neurological syndrome: detection of the underlying malignancy and the brain PET-MRI correlates. Mol Imaging Biol. 10(3):131-7, 2008
                                                                                  28. Blaes F et al: Autoantibodies in childhood opsoclonus-myoclonus syndrome. J Neuroimmunol. 201-202:221-6, 2008
                                                                                  29. Corapcioglu F et al: Response to rituximab and prednisolone for opsoclonus-myoclonus-ataxia syndrome in a child with ganglioneuroblastoma. Pediatr Hematol Oncol. 25(8):756-61, 2008
                                                                                  30. Dalakas MC: Invited article: inhibition of B cell functions: implications for neurology. Neurology. 70(23):2252-60, 2008
                                                                                  31. Eker A et al: Testicular teratoma and anti-N-methyl-D-aspartate receptor-associated encephalitis. J Neurol Neurosurg Psychiatry. 79(9):1082-3, 2008
                                                                                  32. Fitzpatrick AS et al: Opsoclonus-myoclonus syndrome associated with benign ovarian teratoma. Neurology. 70(15):1292-3, 2008
                                                                                  33. Geschwind MD et al: Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol. 65(10):1341-6, 2008
                                                                                  34. Hassan KA et al: Long-term survival in paraneoplastic opsoclonus-myoclonus syndrome associated with small cell lung cancer. J Neuroophthalmol. 28(1):27-30, 2008
                                                                                  35. Jarius S et al: Relative frequency of VGKC and 'classical' paraneoplastic antibodies in patients with limbic encephalitis. J Neurol. 255(7):1100-1, 2008
                                                                                  36. Johnson V et al: Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma. J Neurooncol. 90(3):279-81, 2008
                                                                                  37. Ko MW et al: Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 28(1):58-68, 2008
                                                                                  38. Musunuru K et al: Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma. J Neurooncol. 90(2):213-6, 2008
                                                                                  39. Novillo-López ME et al: Treatment-responsive subacute limbic encephalitis and NMDA receptor antibodies in a man. Neurology. 70(9):728-9, 2008
                                                                                  40. Pellkofer HL et al: Lambert-eaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel. J Neuroimmunol. 204(1-2):136-9, 2008
                                                                                  41. Sabater L et al: SOX1 antibodies are markers of paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology. 70(12):924-8, 2008
                                                                                  42. Sekiguchi Y et al: Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction. J Neurol Sci. 269(1-2):176-9, 2008
                                                                                  43. Tan KM et al: Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 70(20):1883-90, 2008
                                                                                  44. Darnell RB et al: Paraneoplastic syndromes affecting the nervous system. Semin Oncol. 33(3):270-98, 2006
                                                                                  45. Dropcho EJ: Update on paraneoplastic syndromes. Curr Opin Neurol. 18(3):331-6, 2005
                                                                                  46. Dadparvar S et al: Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings. Clin Nucl Med. 28(11):893-6, 2003
                                                                                  47. Messori A et al: Resolution of limbic encephalitis with detection and treatment of lung cancer: clinical-radiological correlation. Eur J Radiol. 45(1): 78-80, 2003
                                                                                  48. Barnett M et al: Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody. J Neurol Neurosurg Psychiatry. 70(2):222-5, 2001
                                                                                  49. Gultekin SH et al: Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 123 ( Pt 7):1481-94, 2000
                                                                                  50. Dalmau J et al: Paraneoplastic neurologic syndromes: pathogenesis and physiopathology. Brain Pathol. 9(2):275-84, 1999
                                                                                  51. Scaravilli F et al: The neuropathology of paraneoplastic syndromes. Brain Pathol. 9(2):251-60, 1999
                                                                                  52. Voltz R et al: A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 340(23): 1788-95, 1999