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[Behçet's disease in children: cortical calcifications].

J Grippo1, G Zocchi, S Fleiderman

  • 1Departamento de Pediatría, Hospital de Niños R. Gutierrez, Buenos Aires, Argentina. grippo@fibertel.com.ar

Revista De Neurologia
|September 18, 2002
PubMed
Summary

Pediatric Behçet s disease can present with neurological issues like aseptic meningoencephalitis, leading to complications such as cortical calcifications in children. This case highlights rare early neurological manifestations of Behçet s disease.

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Area of Science:

  • Pediatric Neurology
  • Rheumatology
  • Rare Diseases

Background:

  • Behçet's disease, a rare multisystemic inflammatory disorder, affects 5-48% of patients with neurological abnormalities.
  • Neurological manifestations as an initial presentation in children are infrequent.
  • Early diagnosis and management are crucial for improving outcomes in pediatric Behçet's disease.

Observation:

  • A four-year-old girl presented with a history of aseptic meningoencephalitis at six months of age.
  • The patient experienced seizures, language and neuromotor deficits, and later developed uveitis, oral, and genital ulcers.
  • Cranial CT revealed asymmetric ventricular dilatation, enlarged sulci, and cortical calcifications.

Findings:

  • The clinical presentation, including meningoencephalitis, multisystemic involvement, and characteristic lesions, confirmed the diagnosis of Behçet's disease.

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  • Aseptic meningoencephalitis in infancy was the initial manifestation, followed by neurological sequelae and typical Behçet's disease symptoms.
  • Cortical calcifications were noted in association with the meningoencephalitis.

    • Implications:

    • This case underscores the importance of considering Behçet's disease in children presenting with unexplained neurological symptoms, even in the absence of typical mucocutaneous lesions.
    • Early recognition of neurological involvement in pediatric Behçet's disease is vital for timely intervention and management of potential complications.
    • The findings contribute to understanding the diverse clinical spectrum of Behçet's disease in pediatric populations.