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Primary Arteritis of CNS
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Primary arteritis of CNS (PACNS)
              • Synonyms

                • Vasculitis, vasculopathy
              • Definitions

                • Descriptive term rather than specific disease
                • 2 cardinal features required for histopathologic diagnosis of vasculitis
                  • Inflammation and necrosis/destruction of blood vessel wall
                • Often associated with occlusion, infarction
                • PACNS: Arteritis confined to intracranial CNS without evidence for systemic vasculitis

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic 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. Leao DJ et al: Intracranial vessel wall imaging: applications, interpretation, and pitfalls. Clin Radiol. ePub, 2020
                                                  2. Rice CM et al: The diagnosis of primary central nervous system vasculitis. Pract Neurol. 20(2):109-14, 2020
                                                  3. Saygin D et al: Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features. Clin Exp Rheumatol. 38 Suppl 124(2):135-8, 2020
                                                  4. Guggenberger K et al: High-resolution compressed-sensing T1 black-blood MRI: a new multipurpose sequence in vascular neuroimaging? Clin Neuroradiol. ePub, 2019
                                                  5. Huang NF et al: Cerebellar PACNS in an elderly patient present as a tumor-like mass lesion: a case report. Acta Neurol Taiwan. 28(2):38-43, 2019
                                                  6. Huang X et al: Susceptibility-weighted imaging in the differential diagnosis of autoimmune central nervous system vasculitis and multiple sclerosis. Mult Scler Relat Disord. 33:70-4, 2019
                                                  7. Jin H et al: Primary angiitis of the central nervous system mimicking glioblastoma: a case report and literature review. Front Neurol. 10:1208, 2019
                                                  8. Kern KC et al: Vessel wall imaging of cerebrovascular disorders. Curr Treat Options Cardiovasc Med. 21(11):65, 2019
                                                  9. Moseley BD et al: Primary angiitis of the central nervous system presenting with microhemorrhages on gradient echo imaging. Neurol India. 67(5):1374-5, 2019
                                                  10. Rayfield C et al: Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis. BMJ Case Rep. 12(8):e230753, 2019
                                                  11. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                  12. Strunk D et al: Biomarkers in vasculitides of the nervous system. Front Neurol. 10:591, 2019
                                                  13. Thaler C et al: Neuroradiologic characteristics of primary angiitis of the central nervous system according to the affected vessel size. Clin Neuroradiol. 29(1):37-44, 2019
                                                  14. Bathla G et al: Cerebrovascular manifestations of neurosarcoidosis: an underrecognized aspect of the imaging spectrum. AJNR Am J Neuroradiol. 39(7):1194-200, 2018
                                                  15. Chen CY et al: Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study. J Headache Pain. 19(1):74, 2018
                                                  16. Ide S et al: Intracranial vessel wall lesions in patients with systematic lupus erythematosus. J Magn Reson Imaging. 48(5):1237-46, 2018
                                                  17. Mehdipoor G et al: Imaging manifestations of Behcet's disease: key considerations and major features. Eur J Radiol. 98:214-25, 2018
                                                  18. Dutra LA et al: Central nervous system vasculitis in adults: an update. Autoimmun Rev. 16(2):123-31, 2017
                                                  19. Mandal J et al: Primary angiitis of the central nervous system. Rheum Dis Clin North Am. 43(4):503-18, 2017
                                                  20. Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging for differentiation of nonocclusive intracranial vasculopathies. Stroke. 48(11):3026-33, 2017
                                                  21. Schuster S et al: Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels. J Neurol Neurosurg Psychiatry. 88(9):749-55, 2017
                                                  22. Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 36(9):1580-8, 2015
                                                  23. Abdel Razek AA et al: Imaging spectrum of CNS vasculitis. Radiographics. 34(4):873-94, 2014
                                                  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

                                                                • Primary arteritis of CNS (PACNS)
                                                              • Synonyms

                                                                • Vasculitis, vasculopathy
                                                              • Definitions

                                                                • Descriptive term rather than specific disease
                                                                • 2 cardinal features required for histopathologic diagnosis of vasculitis
                                                                  • Inflammation and necrosis/destruction of blood vessel wall
                                                                • Often associated with occlusion, infarction
                                                                • PACNS: Arteritis confined to intracranial CNS without evidence for systemic vasculitis

                                                              IMAGING

                                                              • General Features

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic Findings

                                                                      • Angiographic 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. Leao DJ et al: Intracranial vessel wall imaging: applications, interpretation, and pitfalls. Clin Radiol. ePub, 2020
                                                                                                  2. Rice CM et al: The diagnosis of primary central nervous system vasculitis. Pract Neurol. 20(2):109-14, 2020
                                                                                                  3. Saygin D et al: Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features. Clin Exp Rheumatol. 38 Suppl 124(2):135-8, 2020
                                                                                                  4. Guggenberger K et al: High-resolution compressed-sensing T1 black-blood MRI: a new multipurpose sequence in vascular neuroimaging? Clin Neuroradiol. ePub, 2019
                                                                                                  5. Huang NF et al: Cerebellar PACNS in an elderly patient present as a tumor-like mass lesion: a case report. Acta Neurol Taiwan. 28(2):38-43, 2019
                                                                                                  6. Huang X et al: Susceptibility-weighted imaging in the differential diagnosis of autoimmune central nervous system vasculitis and multiple sclerosis. Mult Scler Relat Disord. 33:70-4, 2019
                                                                                                  7. Jin H et al: Primary angiitis of the central nervous system mimicking glioblastoma: a case report and literature review. Front Neurol. 10:1208, 2019
                                                                                                  8. Kern KC et al: Vessel wall imaging of cerebrovascular disorders. Curr Treat Options Cardiovasc Med. 21(11):65, 2019
                                                                                                  9. Moseley BD et al: Primary angiitis of the central nervous system presenting with microhemorrhages on gradient echo imaging. Neurol India. 67(5):1374-5, 2019
                                                                                                  10. Rayfield C et al: Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis. BMJ Case Rep. 12(8):e230753, 2019
                                                                                                  11. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                                                                  12. Strunk D et al: Biomarkers in vasculitides of the nervous system. Front Neurol. 10:591, 2019
                                                                                                  13. Thaler C et al: Neuroradiologic characteristics of primary angiitis of the central nervous system according to the affected vessel size. Clin Neuroradiol. 29(1):37-44, 2019
                                                                                                  14. Bathla G et al: Cerebrovascular manifestations of neurosarcoidosis: an underrecognized aspect of the imaging spectrum. AJNR Am J Neuroradiol. 39(7):1194-200, 2018
                                                                                                  15. Chen CY et al: Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study. J Headache Pain. 19(1):74, 2018
                                                                                                  16. Ide S et al: Intracranial vessel wall lesions in patients with systematic lupus erythematosus. J Magn Reson Imaging. 48(5):1237-46, 2018
                                                                                                  17. Mehdipoor G et al: Imaging manifestations of Behcet's disease: key considerations and major features. Eur J Radiol. 98:214-25, 2018
                                                                                                  18. Dutra LA et al: Central nervous system vasculitis in adults: an update. Autoimmun Rev. 16(2):123-31, 2017
                                                                                                  19. Mandal J et al: Primary angiitis of the central nervous system. Rheum Dis Clin North Am. 43(4):503-18, 2017
                                                                                                  20. Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging for differentiation of nonocclusive intracranial vasculopathies. Stroke. 48(11):3026-33, 2017
                                                                                                  21. Schuster S et al: Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels. J Neurol Neurosurg Psychiatry. 88(9):749-55, 2017
                                                                                                  22. Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 36(9):1580-8, 2015
                                                                                                  23. Abdel Razek AA et al: Imaging spectrum of CNS vasculitis. Radiographics. 34(4):873-94, 2014