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PP13.13 – 2289: Delayed diagnosis of neuro-Behçet's disease: Report of 3 cases

Objective Behçet's disease is a rare chronic, relapsing multisystem vascular inflammatory disease of unknown cause in young adulthood. Only 8% of Behçet's disease cases are children. Methods We report 3 pediatric cases diagnosed as neuro-Behçet's disease, presenting with symptoms and...

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Bibliographic Details
Published in:European journal of paediatric neurology 2015-05, Vol.19, p.S85-S86
Main Authors: Ünver, O, Thomas, G, Hacıfazlıoğlu, N.E, Kutlubay, B, Direskeneli, H, Türkdoğan, D
Format: Article
Language:English
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Summary:Objective Behçet's disease is a rare chronic, relapsing multisystem vascular inflammatory disease of unknown cause in young adulthood. Only 8% of Behçet's disease cases are children. Methods We report 3 pediatric cases diagnosed as neuro-Behçet's disease, presenting with symptoms and signs of intracranial hypertension such as headache and papilledema due to cerebrovascular thrombosis. Case 1: A 16 year-old boy was admitted with a throbbing headache. Three months prior he was evaluated with fever of unknown origin, weight loss, and headache, however no specific etiology was discovered. His brain magnetic resonance venography revealed venous thrombosis in the right transverse sinus, in the sigmoid sinus extending to jugular bulbus and in the posterior part of superior sagittal sinus. When carefully questioned he had history of recurrent oral and genital ulcers. HLA B51 and patergy tests were positive. Case 2: A 14-year old boy was admitted with vertigo and headache. He was hospitalized elsewhere in a pediatric neurology clinic with 6th cranial nerve palsy and venous thrombosis, 3 years before. He had been suffering from aphthous lesions in relapsing and remitting manner for 4 years. His brain magnetic resonance venography revealed venous thrombosis in the right sigmoid sinus. Patergy test was negative, however, HLA B51 was positive. Case 3: A 17-year old boy was admitted with a headache and weight loss. His brain magnetic resonance venography revealed diffuse venous thrombosis in the superior sagittal sinus and in bilateral transverse sinuses. His father had established Behçet's disease. HLA B51 and patergy tests were positive. Results Cerebrovascular thrombosis is one of the diverse manifestations of neuro-Behçet's disease. Conclusion All of the three cases' diagnoses were delayed. The presence of intracranial hypertension related to a cerebral venous thrombosis in an adolescent boy should alert pediatric neurologists about the diagnosis of neuro-Behçet's disease.
ISSN:1090-3798
1532-2130
DOI:10.1016/S1090-3798(15)30284-1