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Transient involvement of the cerebral cortex in a flare of Behçet’s syndrome

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Abstract

We describe a flare of Behçet’s syndrome in a 42-year-old man who presented with florid mucocutaneous manifestations, headache and vomiting, but without meningeal or neurologic signs. A single, non-enhancing cortical lesion was found in the frontal lobe by computed tomography and magnetic resonance (MR) imaging studies. Diffusion-weighted analysis and MR spectroscopy were consistent with focal inflammation. The patient improved with colchicine, and after 5 months, a repeat MR scan demonstrated resolution of the inflammatory changes suggesting that involvement of the cerebral cortex may be a self-limiting phenomenon, distinct from other more frequent and destructive parenchymal manifestations of neuro-Behçet’s disease.

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References

  1. Al-Araji A, Kidd DP (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8:192–204

    Article  PubMed  Google Scholar 

  2. Rubinstein LJ, Urich H (1963) Meningo-encephalitis of Behçet’disease: case report with pathological findings. Brain 86:151–160

    Article  PubMed  CAS  Google Scholar 

  3. Arai Y, Kohno S, Takahashi Y, Miyajima Y, Tsutusi Y (2006) Autopsy case of neuro-Behçet’s disease with multifocal neutrophilic perivascular inflammation. Neuropathology 26:579–585

    Article  PubMed  Google Scholar 

  4. Hirohata S (2008) Histopathology of central nervous system lesions in Behçet’s disease. J Neurol Sci 267:41–47

    Article  PubMed  CAS  Google Scholar 

  5. Akman-Demir G, Bahar S, Coban O, Tasçi B, Serdaroglu P (2003) Cranial MRI in Behçet’s disease: 134 examinations of 98 patients. Neuroradiology 45:851–859

    Article  PubMed  CAS  Google Scholar 

  6. McMenemey WH, Lawrence BJ (1957) Encephalomyelopathy in Behçet’s disease. Report of necropsy findings in two cases. Lancet 273:353–358

    Article  PubMed  CAS  Google Scholar 

  7. Kawakita H, Nishimura M, Satoh Y, Shibata N (1967) Neurological aspects of Behçet’s disease. A case report and clinico-pathological review of the literature in Japan. J Neurol Sci 5:417–439

    Article  PubMed  CAS  Google Scholar 

  8. Totsuka S, Hattori T, Yazaki M, Nagao K, Mizushima S (1985) Clinicopathologic studies on neuro-Behçet’s disease. Folia Psychiatr Neurol Jpn 39:155–166

    PubMed  CAS  Google Scholar 

  9. Wechsler B, Dell’Isola B, Vidailhet M, Dormont D, Piette JC, Blétry O, Godeau P (1993) MRI in 31 patients with Behçet’s disease and neurological involvement: prospective study with clinical correlation. J Neurol Neurosurg Psychiatry 56:793–798

    Article  PubMed  CAS  Google Scholar 

  10. Heo J-H, Lee S-T, Chu K, Kim M (2008) Neuro-Behçet’s disease mimicking multiple brain tumors: diffusion-weighted MR study and literature review. J Neurol Sci 264:177–181

    Article  PubMed  Google Scholar 

  11. Greer DM, Friday RP, Romero J, Auluck PK (2009) A 30-year-old man with progressive neurologic deficits. N Engl J Med 360:2341–2351

    Article  PubMed  CAS  Google Scholar 

  12. Koçer N, Islak C, Siva A, Saip S, Akman C, Kantarci O, Hamuryudan V (1999) CNS involvement in neuro-Behçet syndrome: an MR study. Am J Neuroradiol 20:1015–1024

    PubMed  Google Scholar 

  13. Nüssel F, Wegmüller H, Laseyras F, Posse S, Herschkowitz N, Huber P (1991) Neuro-Behçet: acute and sequential aspects by MRI and MRS. Eur Neurol 31:399–402

    Article  PubMed  Google Scholar 

  14. Pipitone N, Olivieri I, Padula A, D’Angelo S, Nigro A, Zuccoli G, Boiardi L, Salvarani C (2008) Infliximab for the treatment of neuro-Behçet’s disease: a case series and review of the literature. Arthritis Rheum 59:285–290

    Article  PubMed  CAS  Google Scholar 

  15. Borhani Haghighi A (2009) Treatment of neuro-Behçet’s disease: an update. Exp Rev Neurother 9:565–574

    Article  Google Scholar 

  16. Akman-Demir G, Serdaroglu P, Tasçi B, The Neuro-Behçet Study Group (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain 122:2171–2181

    Article  PubMed  Google Scholar 

  17. Lehnardt S, Massillon L, Follett P, Jensen FE, Ratan R, Rosenberg PA, Volpe JJ, Vartanian T (2003) Activation of innate immunity in the CNS triggers neurodegeneration through a Toll-like receptor 4-dependent pathway. Proc Natl Acad Sci USA 100:8514–8519

    Article  PubMed  CAS  Google Scholar 

  18. Keller M, Spanou Z, Schaerli P, Britschgi M, Yawalkar N, Seitz M, Villiger PM, Pichler WJ (2005) T cell-regulated neutrophilic inflammation in autoinflammatory diseases. J Immunol 175:7678–7686

    PubMed  CAS  Google Scholar 

  19. Gül A (2005) Behçet’s disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy 4:81–83

    Article  PubMed  Google Scholar 

  20. Krause I, Weinberger A (2008) Behçet’s disease. Curr Opin Rheumatol 20:82–87

    Article  PubMed  CAS  Google Scholar 

  21. Baysal T, Ozisik HI, Karlidag R, Sarac K, Baysal O, Dusak A, Hazneci E, Ozcan C (2003) Proton MRS in Behçet’s disease with and without neurological findings. Neuroradiology 45:860–864

    Article  PubMed  CAS  Google Scholar 

  22. Park K-S, Ko H-J, Yoon C-H, Park S-H, Cho C-S, Kim H-Y, Choe B-Y, Kim W-U (2004) Magnetic resonance imaging and proton magnetic resonance spectroscopy in neuro-Behçet’s disease. Clin Exp Rheumatol 22:561–567

    PubMed  CAS  Google Scholar 

  23. Saip S, Siva A, Altintas A, Kiyat A, Seyahi E, Hamuryudan V, Yazici H (2005) Headache in Behçet’s syndrome. Headache 45:911–919

    Article  PubMed  Google Scholar 

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Correspondence to Walter Conca.

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Conca, W., Kamel, S.A., Venne, D. et al. Transient involvement of the cerebral cortex in a flare of Behçet’s syndrome. Rheumatol Int 32, 791–794 (2012). https://doi.org/10.1007/s00296-009-1362-0

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  • DOI: https://doi.org/10.1007/s00296-009-1362-0

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