MRI Findings in Pediatric Neuro-Behçet's Disease

Abstract

Behçet's disease (BD), a systemic vasculitis of unknown cause, affects many organs and systems. Neurological involvement is seen in 5 - 15 % of the patients, and the two major forms of neurological disease seen in BD are central nervous system (CNS) parenchymal involvement and cerebral venous sinus thrombosis. We report a 14-year-old boy with BD who had neuro-parenchymal involvement. The diagnosis of the systemic disease was not made until the onset of the neurological manifestations, which led to an MRI study that revealed findings suggestive of CNS involvement of BD. We therefore emphasize the importance of the localization and appearance of other characteristics of the lesions on MRI in the differential diagnosis of parenchymal neuro-Behçet syndrome.

Key words

Behçet's disease - cranial MRI - vasculitis

References

• 1 Akman-Demir G, Baykan-Kurt B, Serdaroglu P. et al . Seven year follow-up of neurologic involvement in Behçet syndrome.  Arch Neurol. 1996;  53 691-694
• 2 Al Kawi M Z, Bohlega S, Banna M. MRI findings in neuro-Behçet's disease.  Neurology. 1991;  41 405-408
• 3 Behçet H. Über rezidivierende, aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien.  Derm Wochenschr. 1937;  105 1152-1157
• 4 International Study Group for Behçet's Disease . Criteria for diagnosis of Behçet's disease.  Lancet. 1990;  335 1078-1080
• 5 Kantarci O, Siva A. Behçet's disease: Diagnosis and management. Noseworthy J Neurological Therapeutics: Principles and Practice. London; Martin Dunitz Publishers 2003 (95): 1084
• 6 Koçer N, Islak C, Siva A. et al . CNS involvement in neuro-Behçet syndrome: An MRI study.  AJNR Am J Neuroradiol. 1999;  20 1015-1024
• 7 Kone-Paut I, Bernard J L. Epidemiology of Behçet's diseases in children: A French nationwide survey.  Arthritis Rheum. 1992;  35 190
• 8 Kone-Paut I, Chabrol B, Riss J M. et al . Neurologic onset of Behçet's disease: A diagnostic enigma in childhood.  J Child Neurol. 1997;  12 237-241
• 9 Kone-Paut I, Yurdakul S, Bahabri S A. et al . Clinical features of Behçet's disease in children: A collaborative study of 86 cases.  J Pediatr. 1998;  132 721-725
• 10 Siva A, Kantarci O, Saip S. et al . Behçet's disease: diagnostic and prognostic aspects of neurological involvement.  J Neurol. 2001;  248 95-103
• 11 Siva A, Yazici H. Nervous system involvement in Behçet's syndrome. Erkan D, Levine S Handbook of Systemic Autoimmune Diseases, The Neurologic Involvement in Systemic Autoimmune Disorders. New York; Elsevier Science B. V. 2004
• 12 Wechsler B, Vidailhet M, Piette J C. et al . Cerebral venous thrombosis in Behçet's disease: Clinical study and long-term follow-up of 25 cases.  Neurology. 1992;  42 614-618