title: Recurrent pseudotumor cerebri in childhood: a case of neuro-Behçet disease complicated with thrombotic risk factors.
abstract: Pseudotumor cerebri with or without venous sinus thrombosis is a rare clinical presentation of Behçet disease in childhood. We present here a case of childhood pseudotumor cerebri without a previous diagnosis of Behçet disease. The detailed history and physical examination of the case led to the diagnosis of neuro-Behçet disease. The investigation of predisposition to thrombosis revealed heterozygous factor V Leiden mutation along with the high lipoprotein(a) level. The symptoms resolved dramatically by treatment with the combination of immunosuppression and anticoagulation with regard to the detected factor V Leiden mutation and high lipoprotein(a) level. After a symptom-free period of 9 months, the cerebral vein thrombosis recurred. We present this case to draw attention to this rare cause of pseudotumor cerebri in childhood and to emphasize the importance of additional thrombotic risk factors regarding the potential recurrence of thrombotic events in Behçet disease.
authors.affiliation: Division of Child Neurology, Department of Pediatrics, Ege University Medical School, Bornova-Izmir, Turkey. sanem.yilmaz@ege.edu.tr
authors.forename: Sanem
authors.initials: S
authors.lastname: Yilmaz
authors.forename: Gul
authors.initials: G
authors.lastname: Serdaroglu
authors.forename: Hale
authors.initials: H
authors.lastname: Unver
authors.forename: Ayfer
authors.initials: A
authors.lastname: Akcay
authors.forename: Sarenur
authors.initials: S
authors.lastname: Gokben
authors.forename: Hasan
authors.initials: H
authors.lastname: Tekgul
mesh_terms: D017668:Age of Onset; D001528:Behcet Syndrome / Q000150:complications / Q000453:epidemiology / Q000235:genetics*; D002648:Child; D005165:Factor V / Q000235:genetics; D005260:Female; D006801:Humans; D017270:Lipoprotein(a) / Q000097:blood; D011559:Pseudotumor Cerebri / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics*; D012008:Recurrence; D012851:Sinus Thrombosis, Intracranial / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics*; D016896:Treatment Outcome
chemical_list: D017270:Lipoprotein(a); C095381:factor V Leiden; D005165:Factor V