Download PDF
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)

    Permissions and Reprints

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

References

  • 1 Criteria for diagnosis of Behcet's disease . International Study Group for Behçet's Disease.  Lancet. 1990;  335 (8697) 1078-1080
    PubMedGoogle Scholar
  • 2 Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management.  Lancet Neurol. 2009;  8 192-204
    CrossrefPubMedGoogle Scholar
  • 3 Al-Araji A, Sharquie K, Al-Rawi Z. Prevalence and patterns of neurological involvement in Behçet's disease: a prospective study from Iraq.  J Neurol Neurosurg Psychiatry. 2003;  74 608-613
    CrossrefPubMedGoogle Scholar
  • 4 Alper G, Yilmaz Y, Ekinci G. et al . Cerebral vein thrombosis in Behçet's disease.  Pediatr Neurol. 2001;  25 332-335
    CrossrefPubMedGoogle Scholar
  • 5 Ammann AJ, Johnson A, Fyfe GA. et al . Behçet syndrome.  J Pediatr. 1985;  107 41-43
    PubMedGoogle Scholar
  • 6 Amoura Z, Guillaume M, Caillat-Zucman S. et al . Pathophysiology of Behçet's disease.  Rev Med Interne. 2006;  27 843-853
    CrossrefPubMedGoogle Scholar
  • 7 Atkinson M, Moore E, Altinok D. et al . Cerebral infarct in pediatric neuro-Behçet's disease.  J Child Neurol. 2008;  23 1331-1335
    CrossrefPubMedGoogle Scholar
  • 8 Bahabri SA, al-Mazyed A, al-Balaa S. et al . Juvenile Behçet's disease in Arab children.  Clin Exp Rheumatol. 1996;  14 331-335
    PubMedGoogle Scholar
  • 9 Behçet H. Über Rezidivierende Aphtose durch ein Virus Verursachte Geschwüre am Mund, am Augeund an den Genitalien.  Dermatol Wochenschr. 1937;  105 1152-1157
    PubMedGoogle Scholar
  • 10 Benamour S, Naji T, Alaoui FZ. et al . Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature].  Rev Neurol (Paris). 2006;  162 1084-1090
    CrossrefPubMedGoogle Scholar
  • 11 Budin C, Ranchin B, Glastre C. et al . Neurologic signs revealing a Behçet's disease: two pediatric case reports.  Arch Pediatr. 2002;  9 1160-1162
    CrossrefPubMedGoogle Scholar
  • 12 Cheng YK, Thong BY, Chng HH. Behçet's disease: experience in a tertiary rheumatology centre in Singapore and a review of the literature.  Ann Acad Med Singapore. 2004;  33 510-514
    PubMedGoogle Scholar
  • 13 Colvard DM, Robertson DM, O’Duffy JD. The ocular manifestations of Behçet's disease.  Arch Ophthalmol. 1977;  95 1813-1817
    CrossrefPubMedGoogle Scholar
  • 14 Devlin T, Gray L, Allen NB. et al . Neuro-Behçet's disease: factors hampering proper diagnosis.  Neurology. 1995;  45 1754-1757
    PubMedGoogle Scholar
  • 15 Di Biasi C, Accorinti M, Trasimeni G. et al . Magnetic resonance of the encephalon in 17 patients with ocular Behçet's disease.  Radiol Med. 1997;  93 348-351
    PubMedGoogle Scholar
  • 16 Eldem B, Onur C, Ozen S. Clinical features of pediatric Behçet's disease.  J Pediatr Ophthalmol Strabismus. 1998;  35 159-161
    PubMedGoogle Scholar
  • 17 Fujikawa S, Suemitsu T. Behçet disease in children: a nationwide retrospective survey in Japan.  Acta Paediatr Jpn. 1997;  39 285-289
    PubMedGoogle Scholar
  • 18 Herskovitz S, Lipton RB, Lantos G. Neuro-Behçet's disease: CT and clinical correlates.  Neurology. 1988;  38 1714-1720
    PubMedGoogle Scholar
  • 19 Kari JA, Shah V, Dillon MJ. Behçet's disease in UK children: clinical features and treatment including thalidomide.  Rheumatology (Oxford). 2001;  40 933-938
    CrossrefPubMedGoogle Scholar
  • 20 Kone-Paut I. Behçet's disease: pediatric features.  Ann Med Interne (Paris). 1999;  150 571-575
    PubMedGoogle Scholar
  • 21 Koné-Paut I. Autoinflammatory gene mutations in Behçet's disease.  Ann Rheum Dis. 2007;  66 832-834
    CrossrefPubMedGoogle Scholar
  • 22 Kone-Paut I, Bernard JL. Behçet disease in children in France.  Arch Fr Pediatr. 1993;  50 561-565
    PubMedGoogle Scholar
  • 23 Kone-Paut I, Chabrol B, Riss JM. et al . Neurologic onset of Behçet's disease: a diagnostic enigma in childhood.  J Child Neurol. 1997;  12 237-241
    CrossrefPubMedGoogle Scholar
  • 24 Koné-Paut I, Darce Bello M, Shahram F. et al . Paediatric Behçet's Disease, PED-BD: Registries in rheumatological and musculoskeletal conditions. paediatric Behçet's disease: an international cohort study of 110 patients, one-year follow-up data.  Rheumatology. 2010;  Oct 29. [Epub ahead of print]
    PubMedGoogle Scholar
  • 25 Kone-Paut I, Yurdakul S, Bahabri SA. et al . Clinical features of Behçet's disease in children: an international collaborative study of 86 cases.  J Pediatr. 1998;  132 721-725
    PubMedGoogle Scholar
  • 26 Krause I, Uziel Y, Guedj D. et al . Mode of presentation and multisystem involvement in Behçet's disease: the influence of sex and age of disease onset.  J Rheumatol. 1998;  25 1566-1569
    PubMedGoogle Scholar
  • 27 Laghmari M, Karim A, Allali F. et al . Childhood Behçet's disease: clinical and evolutive aspects. About 13 cases.  J Fr Ophtalmol. 2002;  25 904-908
    PubMedGoogle Scholar
  • 28 Lang BA, Laxer RM, Thorner P. et al . Pediatric onset of Behçet's syndrome with myositis: case report and literature review illustrating unusual features.  Arthritis Rheum. 1990;  33 418-425
    CrossrefPubMedGoogle Scholar
  • 29 Medejel A. Neurologic manifestations in Behçet disease.  Sem Hop Paris. 1986;  62 1325-1328
    PubMedGoogle Scholar
  • 30 Menassa J, Sawaya R, Masri AF. et al . Recurrent peripheral facial paresis may constitute the sole clinical manifestation in neuro-Behçet disease.  Neurologist. 2008;  14 77
    PubMedGoogle Scholar
  • 31 Motomura S, Tabira T, Kuroiwa Y. A clinical comparative study of multiple sclerosis and neuro-Behçet's syndrome.  J Neurol Neurosurg Psychiatry. 1980;  43 210-213
    CrossrefPubMedGoogle Scholar
  • 32 Ohno S, Ohguchi M, Hirose S. et al . Close association of HLA-Bw51 with Behçet's disease.  Arch Ophthalmol. 1982;  100 1455-1458
    CrossrefPubMedGoogle Scholar
  • 33 Oueslati S, Douira W, Charrada-Ben Farhat L. et al . Imaging of neuro-Behçet.  Tunis Med. 2005;  83 681-684
    PubMedGoogle Scholar
  • 34 Saltik S, Saip S, Kocer N. et al . MRI findings in pediatric neuro-Behçet's disease.  Neuropediatrics. 2004;  35 190-193
    Article in Thieme ConnectPubMedGoogle Scholar
  • 35 Serdaroglu P, Yazici H, Ozdemir C. et al . Neurologic involvement in Behçet's syndrome. A prospective study.  Arch Neurol. 1989;  46 265-269
    CrossrefPubMedGoogle Scholar
  • 36 Shimizu T. Clinical studies of neuro-Behçet's syndrome – with special reference to epidemiological and clinicopathological studies.  Shinkei Kenkyu No Shimpo. 1972;  16 167-178
    PubMedGoogle Scholar
  • 37 Strouth JC, Dyken M. Encephalopathy of Behçet's disease. Report of a case.  Neurology. 1964;  14 794-805
    PubMedGoogle Scholar
  • 38 Tohme A, Haddad F, Ghayad E. Neurologic manifestations in Behçet's disease. 16 cases in a cohort of 110 patients.  Ann Med Interne (Paris). 1997;  148 118-124
    PubMedGoogle Scholar
  • 39 Vaiopoulos G, Kaklamani VG, Markomichelakis N. et al . Clinical features of juvenile Adamantiades-Behçet's disease in Greece.  Clin Exp Rheumatol. 1999;  17 256-259
    PubMedGoogle Scholar
  • 40 Wechsler B, Gerber S, Vidailhet M. et al . Neurologic manifestations of Behçet's disease.  Ann Med Interne (Paris). 1999;  150 555-561
    PubMedGoogle Scholar
  • 41 Wechsler B, Piette JC, Conard J. et al . Deep venous thrombosis in Behçet's disease. 106 localizations in a series of 177 patients.  Presse Med. 1987;  16 661-664
    PubMedGoogle Scholar

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

      >