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Behçet Disease
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Neuro-Behçet disease
            • Definitions

              • Chronic, idiopathic relapsing-remitting multisystem vascular-inflammatory disease characterized by recurrent orogenital ulcerations and uveitis
                • CNS involved in up to 20-25% of patients

            IMAGING

            • General Features

              • Imaging Recommendations

                • CT Findings

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Miller JJ et al: Neuro-behçet disease and autoinflammatory disorders. Semin Neurol. 34(4):437-43, 2014
                                  2. Noel N et al: Long-term outcome of neuro-Behçet's disease. Arthritis Rheumatol. 66(5):1306-14, 2014
                                  3. Saip S et al: Neuro-Behçet syndrome. Handb Clin Neurol. 121:1703-23, 2014
                                  4. Mora P et al: Neuro-Behçet's disease in childhood: a focus on the neuro-ophthalmological features. Orphanet J Rare Dis. 8:18, 2013
                                  5. Farahangiz S et al: Magnetic resonance imaging findings and outcome of neuro-Behçet's disease: the predictive factors. Int J Rheum Dis. 15(6):e142-9, 2012
                                  6. Peño IC et al: Neurobehçet disease: clinical and demographic characteristics. Eur J Neurol. 19(9):1224-7, 2012
                                  7. Uluduz D et al: Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 77(21):1900-5, 2011
                                  8. Kale N et al: The presentation of headache in neuro-Behçet's disease: a case-series. Headache. 49(3):467-70, 2009
                                  9. Siva A et al: The spectrum of nervous system involvement in Behçet's syndrome and its differential diagnosis. J Neurol. 256(4):513-29, 2009
                                  10. Taskapilioglu O et al: Delayed diagnosis of a neuroBehçet patient with only brainstem and cerebellar atrophy: literature review. J Neurol Sci. 277(1-2):160-3, 2009
                                  11. Deprez FC et al: MRI features of Neuro-Behçet disease. JBR-BTR. 91(5):221, 2008
                                  12. Heo JH et al: Neuro-Behcet's disease mimicking multiple brain tumors: diffusion-weighted MR study and literature review. J Neurol Sci. 264(1-2):177-81, 2008
                                  13. Moratalla MB: Central nervous system involvement in Behçet's syndrome. J Rheumatol. 35(1):175-6, 2008
                                  14. Haghighi AB et al: The pathological presentations of neuro-Behçet disease: a case report and review of the literature. Neurologist. 13(4):209-14, 2007
                                  15. Joseph FG et al: Neuro-Behçet's disease in Caucasians: a study of 22 patients. Eur J Neurol. 14(2):174-80, 2007
                                  16. Appenzeller S et al: Brain tumor-like lesion in Behçet disease. Rheumatol Int. 26(6):577-80, 2006
                                  17. Baysal T et al: Diffusion-weighted imaging in chronic Behçet patients with and without neurological findings. Neuroradiology. 47(6):431-7, 2005
                                  18. Hasegawa T et al: Neuro-Behçet's disease presenting initially as mesiotemporal lesions mimicking herpes simplex encephalitis. Eur J Neurol. 12(8):661-2, 2005
                                  19. Matsuo K et al: Neuro-Behçet disease mimicking brain tumor. AJNR Am J Neuroradiol. 26(3):650-3, 2005
                                  20. Cengiz N et al: Correlation of clinical, MRI and Tc-99m HMPAO SPECT findings in neuro-Behçet's disease. Acta Neurol Belg. 104(3):100-5, 2004
                                  21. Park KS et al: Magnetic resonance imaging and proton magnetic resonance spectroscopy in neuro-Behçet's disease. Clin Exp Rheumatol. 22(5):561-7, 2004
                                  22. Scully SE et al: Magnetic resonance spectroscopic findings in neuro-Behçet disease. Neurologist. 10(6):323-6, 2004
                                  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

                                          • Diagnostic Checklist

                                            TERMINOLOGY

                                            • Synonyms

                                              • Neuro-Behçet disease
                                            • Definitions

                                              • Chronic, idiopathic relapsing-remitting multisystem vascular-inflammatory disease characterized by recurrent orogenital ulcerations and uveitis
                                                • CNS involved in up to 20-25% of patients

                                            IMAGING

                                            • General Features

                                              • Imaging Recommendations

                                                • CT Findings

                                                  • MR Findings

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Miller JJ et al: Neuro-behçet disease and autoinflammatory disorders. Semin Neurol. 34(4):437-43, 2014
                                                                  2. Noel N et al: Long-term outcome of neuro-Behçet's disease. Arthritis Rheumatol. 66(5):1306-14, 2014
                                                                  3. Saip S et al: Neuro-Behçet syndrome. Handb Clin Neurol. 121:1703-23, 2014
                                                                  4. Mora P et al: Neuro-Behçet's disease in childhood: a focus on the neuro-ophthalmological features. Orphanet J Rare Dis. 8:18, 2013
                                                                  5. Farahangiz S et al: Magnetic resonance imaging findings and outcome of neuro-Behçet's disease: the predictive factors. Int J Rheum Dis. 15(6):e142-9, 2012
                                                                  6. Peño IC et al: Neurobehçet disease: clinical and demographic characteristics. Eur J Neurol. 19(9):1224-7, 2012
                                                                  7. Uluduz D et al: Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 77(21):1900-5, 2011
                                                                  8. Kale N et al: The presentation of headache in neuro-Behçet's disease: a case-series. Headache. 49(3):467-70, 2009
                                                                  9. Siva A et al: The spectrum of nervous system involvement in Behçet's syndrome and its differential diagnosis. J Neurol. 256(4):513-29, 2009
                                                                  10. Taskapilioglu O et al: Delayed diagnosis of a neuroBehçet patient with only brainstem and cerebellar atrophy: literature review. J Neurol Sci. 277(1-2):160-3, 2009
                                                                  11. Deprez FC et al: MRI features of Neuro-Behçet disease. JBR-BTR. 91(5):221, 2008
                                                                  12. Heo JH et al: Neuro-Behcet's disease mimicking multiple brain tumors: diffusion-weighted MR study and literature review. J Neurol Sci. 264(1-2):177-81, 2008
                                                                  13. Moratalla MB: Central nervous system involvement in Behçet's syndrome. J Rheumatol. 35(1):175-6, 2008
                                                                  14. Haghighi AB et al: The pathological presentations of neuro-Behçet disease: a case report and review of the literature. Neurologist. 13(4):209-14, 2007
                                                                  15. Joseph FG et al: Neuro-Behçet's disease in Caucasians: a study of 22 patients. Eur J Neurol. 14(2):174-80, 2007
                                                                  16. Appenzeller S et al: Brain tumor-like lesion in Behçet disease. Rheumatol Int. 26(6):577-80, 2006
                                                                  17. Baysal T et al: Diffusion-weighted imaging in chronic Behçet patients with and without neurological findings. Neuroradiology. 47(6):431-7, 2005
                                                                  18. Hasegawa T et al: Neuro-Behçet's disease presenting initially as mesiotemporal lesions mimicking herpes simplex encephalitis. Eur J Neurol. 12(8):661-2, 2005
                                                                  19. Matsuo K et al: Neuro-Behçet disease mimicking brain tumor. AJNR Am J Neuroradiol. 26(3):650-3, 2005
                                                                  20. Cengiz N et al: Correlation of clinical, MRI and Tc-99m HMPAO SPECT findings in neuro-Behçet's disease. Acta Neurol Belg. 104(3):100-5, 2004
                                                                  21. Park KS et al: Magnetic resonance imaging and proton magnetic resonance spectroscopy in neuro-Behçet's disease. Clin Exp Rheumatol. 22(5):561-7, 2004
                                                                  22. Scully SE et al: Magnetic resonance spectroscopic findings in neuro-Behçet disease. Neurologist. 10(6):323-6, 2004