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Intracranial Idiopathic Inflammatory Pseudotumor
Bronwyn E. Hamilton, MD; H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Idiopathic extraorbital inflammation (IEI)
          • Synonyms

            • Idiopathic inflammatory disease, Tolosa-Hunt syndrome, hypertrophic cranial pachymeningitis, plasma cell granuloma
          • Definitions

            • Nonspecific, nonneoplastic benign inflammatory lesion without identifiable local or systemic causes characterized by polymorphous lymphoplasmacytic infiltrate
              • IgG4-related disease (IgG4-RD): Subgroup of idiopathic orbital inflammation (IOI) IEI lesions where multisystem manifestation is present with positive cell immunostain showing IgG4-rich plasma cells

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Microscopic Features

                        • IgG4-Related Disease Subtype Additional Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      • Reporting Tips

                                        Selected References

                                        1. Bhatkar S et al: Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome. Neuroradiol J. 33(6):501-7, 2020
                                        2. Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
                                        3. Munawar K et al: Cavernous sinus lesions. Clin Imaging. 68:71-89, 2020
                                        4. Rizzo E et al: Idiopathic hypertrophic pachymeningitis: does earlier treatment improve outcome? Children (Basel). 8(1), 2020
                                        5. Zhao Y et al: Imaging features, clinicopathological analysis and diagnostic strategy of IgG4-related hypertrophic pachymeningitis. Ann Palliat Med. 9(5):2551-8, 2020
                                        6. Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017
                                        7. De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res.65(1):386-94, 2016
                                        8. Dash GK et al: Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. J Neurol Sci. 350(1-2):51-60, 2015
                                        9. Hung CH et al: Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis. Headache. 55(2):252-64, 2015
                                        10. Bhatti RM et al: IgG4-related disease of the head and neck. Adv Anat Pathol. 20(1):10-6, 2013
                                        11. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
                                        12. Tian CL et al: Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis. Chin Med J (Engl). 125(8):1513-6, 2012
                                        13. Toyoda K et al: MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol. 33(11):2136-9, 2012
                                        14. Battineni ML et al: Idiopathic hypereosinophilic syndrome with skull base involvement. AJNR Am J Neuroradiol. 28(5):971-3, 2007
                                        15. Mangiardi JR et al: Extraorbital skull base idiopathic pseudotumor. Laryngoscope. 117(4):589-94, 2007
                                        16. Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
                                        17. Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
                                        18. Lee EJ et al: MR imaging of orbital inflammatory pseudotumors with extraorbital extension. Korean J Radiol. 6(2):82-8, 2005
                                        19. Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
                                        20. Narla LD et al: Inflammatory pseudotumor. Radiographics. 23(3):719-29, 2003
                                        21. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                        22. Cho YS et al: Inflammatory pseudotumour involving the skull base and cervical spine. J Laryngol Otol. 115(7):580-4, 2001
                                        23. Bencherif B et al: Intracranial extension of an idiopathic orbital inflammatory pseudotumor. AJNR Am J Neuroradiol. 14(1):181-4, 1993
                                        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

                                                • Abbreviations

                                                  • Idiopathic extraorbital inflammation (IEI)
                                                • Synonyms

                                                  • Idiopathic inflammatory disease, Tolosa-Hunt syndrome, hypertrophic cranial pachymeningitis, plasma cell granuloma
                                                • Definitions

                                                  • Nonspecific, nonneoplastic benign inflammatory lesion without identifiable local or systemic causes characterized by polymorphous lymphoplasmacytic infiltrate
                                                    • IgG4-related disease (IgG4-RD): Subgroup of idiopathic orbital inflammation (IOI) IEI lesions where multisystem manifestation is present with positive cell immunostain showing IgG4-rich plasma cells

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Microscopic Features

                                                              • IgG4-Related Disease Subtype Additional Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          • Image Interpretation Pearls

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Bhatkar S et al: Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome. Neuroradiol J. 33(6):501-7, 2020
                                                                              2. Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
                                                                              3. Munawar K et al: Cavernous sinus lesions. Clin Imaging. 68:71-89, 2020
                                                                              4. Rizzo E et al: Idiopathic hypertrophic pachymeningitis: does earlier treatment improve outcome? Children (Basel). 8(1), 2020
                                                                              5. Zhao Y et al: Imaging features, clinicopathological analysis and diagnostic strategy of IgG4-related hypertrophic pachymeningitis. Ann Palliat Med. 9(5):2551-8, 2020
                                                                              6. Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017
                                                                              7. De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res.65(1):386-94, 2016
                                                                              8. Dash GK et al: Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. J Neurol Sci. 350(1-2):51-60, 2015
                                                                              9. Hung CH et al: Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis. Headache. 55(2):252-64, 2015
                                                                              10. Bhatti RM et al: IgG4-related disease of the head and neck. Adv Anat Pathol. 20(1):10-6, 2013
                                                                              11. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
                                                                              12. Tian CL et al: Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis. Chin Med J (Engl). 125(8):1513-6, 2012
                                                                              13. Toyoda K et al: MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol. 33(11):2136-9, 2012
                                                                              14. Battineni ML et al: Idiopathic hypereosinophilic syndrome with skull base involvement. AJNR Am J Neuroradiol. 28(5):971-3, 2007
                                                                              15. Mangiardi JR et al: Extraorbital skull base idiopathic pseudotumor. Laryngoscope. 117(4):589-94, 2007
                                                                              16. Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
                                                                              17. Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
                                                                              18. Lee EJ et al: MR imaging of orbital inflammatory pseudotumors with extraorbital extension. Korean J Radiol. 6(2):82-8, 2005
                                                                              19. Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
                                                                              20. Narla LD et al: Inflammatory pseudotumor. Radiographics. 23(3):719-29, 2003
                                                                              21. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                                                              22. Cho YS et al: Inflammatory pseudotumour involving the skull base and cervical spine. J Laryngol Otol. 115(7):580-4, 2001
                                                                              23. Bencherif B et al: Intracranial extension of an idiopathic orbital inflammatory pseudotumor. AJNR Am J Neuroradiol. 14(1):181-4, 1993