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Neuropediatrics 2010; 41(5): 228-234
DOI: 10.1055/s-0030-1269909
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Neurological Involvement in Paediatric Behçet's Disease

J. Metreau-Vastel2 , Y. Mikaeloff2 , M. Tardieu2 , I. Koné-Paut1 , T.-A. Tran1
  • 1Department of Paediatrics, Pediatric Rheumatology, Bicêtre University Hospital, Le Kremlin Bicêtre, France
  • 2Department of Paediatric Neurology, Bicêtre University Hospital, Le Kremlin Bicêtre, France
Further Information

Publication History

received 16.07.2010

accepted 22.11.2010

Publication Date:
05 January 2011 (online)

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Abstract

Introduction: Behçet disease (BD) is a systemic vasculitis which can affect the neurological system. Neuro-Behçet's disease (NBD) is not well described in children.

Objective: Our study aimed to analyse the clinical patterns of paediatric NBD and to describe their repercussions on children's schooling.

Methods: We performed a retrospective chart review of 12 NBD children and a phone interview of patients and their physicians to investigate their last physical and schooling status.

Results: In 40 BD patients reviewed, 12 (sex ratio 8M/4F) had neurological involvement. The mean age of onset was 11 years. In 4 cases, neurological symptoms were the initial presentation. In other cases, NBD occurred within a mean time of 10 months after BD was diagnosed. Cerebral venous thromboses were frequent (5/12) as compared to recurrent meningoencephalitis (2/12), rhombencephalitis (2/12), transverse myelitis (1/12), peripheral neuropathy (1/12) or psychiatric disturbances (1/12). 9 patients had sequelae and 8 had difficulties in learning. 6 stopped at middle school level. For the other patients, an arrangement of the teaching environment was needed due to visual, auditory and concentration disorders.

Conclusion: Neurological involvement is frequent in BD children and its consequences could be severe. Timely accommodations are required to facilitate their ability to follow the normal curriculum.

Key words

Behçet's disease - neuro-Behçet's disease - children - neurological involvement

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Correspondence

Tu-Anh Tran

Department of Paediatrics

Pediatric Rheumatology

Bicêtre University Hospital

78 rue du général Leclerc

94275 Le Kremlin Bicêtre

France

Phone: +33/1/01 45 21 32 45

Fax: +33/1/01 45 21 33 43

Email: tu-anh.tran@bct.aphp.fr