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

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Primary arteritis of CNS (PACNS)
            • Synonyms

              • Vasculitis, vasculopathy
            • Definitions

              • 2 cardinal features required for histopathologic diagnosis of vasculitis
                • Inflammation and necrosis of blood vessel wall
              • 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. Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. ePub, 2015
                                                2. Rodriguez-Pla A et al: Primary angiitis of the central nervous system in adults and children. Rheum Dis Clin North Am. 41(1):47-62, viii, 2015
                                                3. Salvarani C et al: Adult primary central nervous system vasculitis treatment and course: Analysis of 163 patients. Arthritis Rheumatol. ePub, 2015
                                                4. Vera-Lastra O et al: Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis. Clin Rheumatol. 34(4):729-38, 2015
                                                5. Abdel Razek AA et al: Imaging spectrum of CNS vasculitis. Radiographics. 34(4):873-94, 2014
                                                6. Miller DV et al: Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol. 33(1):35-43, 2009
                                                7. Molloy ES et al: Primary angiitis of the central nervous system. Curr Treat Options Neurol. 9(3):169-75, 2007
                                                8. MacLaren K et al: Primary angiitis of the central nervous system: emerging variants. QJM. 98(9):643-54, 2005
                                                9. Carolei A et al: Central nervous system vasculitis. Neurol Sci. 24 Suppl 1:S8-S10, 2003
                                                10. West SG: Central nervous system vasculitis. Curr Rheumatol Rep. 5(2):116-27, 2003
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Primary arteritis of CNS (PACNS)
                                                          • Synonyms

                                                            • Vasculitis, vasculopathy
                                                          • Definitions

                                                            • 2 cardinal features required for histopathologic diagnosis of vasculitis
                                                              • Inflammation and necrosis of blood vessel wall
                                                            • 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. Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. ePub, 2015
                                                                                              2. Rodriguez-Pla A et al: Primary angiitis of the central nervous system in adults and children. Rheum Dis Clin North Am. 41(1):47-62, viii, 2015
                                                                                              3. Salvarani C et al: Adult primary central nervous system vasculitis treatment and course: Analysis of 163 patients. Arthritis Rheumatol. ePub, 2015
                                                                                              4. Vera-Lastra O et al: Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis. Clin Rheumatol. 34(4):729-38, 2015
                                                                                              5. Abdel Razek AA et al: Imaging spectrum of CNS vasculitis. Radiographics. 34(4):873-94, 2014
                                                                                              6. Miller DV et al: Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol. 33(1):35-43, 2009
                                                                                              7. Molloy ES et al: Primary angiitis of the central nervous system. Curr Treat Options Neurol. 9(3):169-75, 2007
                                                                                              8. MacLaren K et al: Primary angiitis of the central nervous system: emerging variants. QJM. 98(9):643-54, 2005
                                                                                              9. Carolei A et al: Central nervous system vasculitis. Neurol Sci. 24 Suppl 1:S8-S10, 2003
                                                                                              10. West SG: Central nervous system vasculitis. Curr Rheumatol Rep. 5(2):116-27, 2003