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[Early onset Neurobehçet's disease].

M Moraleda-Cibrián1, M A Aracil-Martínez, G Ercilla-González

  • 1Servicio de Neuropediatría, Consorci Hospitalari Clínic-Sant Joan de Déu, Esplugues de Ll., Barcelona, Spain. mmoraledac@hotmail.com

Revista De Neurologia
|February 14, 2006
PubMed
Summary
This summary is machine-generated.

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Diagnosing Behçet

Area of Science:

  • Pediatric Rheumatology
  • Neurology
  • Dermatology

Background:

  • Behçet's disease presents diagnostic challenges in infancy due to a lack of early pathognomonic symptoms.
  • Early onset Behçet's disease has been reported, necessitating increased awareness in pediatric populations.

Observation:

  • A case of a 12-year-old female from Pakistan with Behçet's disease onset at 6 months is presented.
  • Initial symptoms included fever and skin lesions, progressing to neurological involvement (ophthalmoplegia, ataxia, dysmetry) by age 5.
  • Diagnosis was aided by MRI, genital ulcers appearing pre-puberty, skin biopsy, and HLA-B08 antigen detection.

Findings:

  • The patient experienced fever, skin lesions, neurological deficits, abdominal pain, arthromyalgia, oral ulcers, and delayed weight gain.

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  • The presence of genital ulcers before puberty and specific skin biopsy findings were crucial for diagnosis.
  • Serological testing confirmed HLA-B08 antigen, and treatment with methylprednisolone and azathioprine induced remission.

    • Implications:

    • Behçet's disease should be considered in young females from high-incidence regions presenting with oral/genital ulcers and neurological symptoms.
    • Early diagnosis through comprehensive evaluation is vital for effective management and treatment of Behçet's disease.
    • This case highlights the importance of recognizing atypical presentations of Behçet's disease in pediatric patients.