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Clinical characteristics of pediatric-onset neuro-Behçet disease

D Uluduz1, M Kürtüncü, Z Yapıcı

  • 1Department of Neurology and Division of Rheumatology, Cerrahpaşa School of Medicine, Istanbul University, Istanbul, Turkey.

Neurology
|November 15, 2011
PubMed

Insights

Pediatric neuro-Behçet disease (NBD) is rare, often presenting as dural venous sinus thrombosis in males. Adult NBD predominantly involves parenchymal CNS, suggesting different disease mechanisms based on age at onset.

Area of Science:

  • Neurology
  • Pediatric Rheumatology
  • Autoimmune Diseases

Background:

  • Neurologic involvement in Behçet disease (BD) is infrequently reported in children.
  • Understanding pediatric BD neurological manifestations is crucial for early diagnosis and management.

Purpose of the Study:

  • To determine the frequency and characteristics of neurologic involvement in pediatric patients with Behçet disease (BD).
  • To compare neurologic manifestations between pediatric and adult BD patients.

Main Methods:

  • Retrospective analysis of 728 neuro-Behçet disease (NBD) patients from two large BD cohorts.
  • Identification of pediatric NBD cases (onset ≤16 years) and comparison with adult NBD patients.
  • Evaluation of demographic and clinical data, including CNS imaging.

Main Results:

  • Pediatric NBD accounted for 3.6% of the cohort (26 cases).
  • Pediatric NBD showed a male predominance (5.5:1) and primarily involved dural venous sinus thrombosis (88.5%).
  • Adult NBD predominantly featured parenchymal CNS involvement (74.8%) and venous sinus thrombosis in only 17.2%.

Conclusions:

  • Pediatric NBD is uncommon, male-predominant, and typically presents as dural venous sinus thrombosis.
  • Adult NBD more frequently involves parenchymal CNS lesions.
  • The differing patterns suggest distinct pathogenetic mechanisms for NBD based on age of onset.
Abstract

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