AI Article Synopsis

  • The study examined the clinical features and prognosis of neuro-Behçet's syndrome (NBS) in 5 children, analyzing their medical data over a span of 10 years from various pediatric departments.
  • The patients, aged between 8 to 17 years, exhibited variable symptoms like headaches, convulsions, and impaired motor functions; some showed no symptoms, highlighting the non-specific nature of NBS.
  • Treatment involved corticosteroids alongside immunosuppressants, resulting in positive outcomes for most patients, although one individual discontinued treatment; imaging showed diverse brain lesions not easily identifiable as NBS.

Article Abstract

To investigate the clinical features and prognosis of neuro-Behçet's syndrome (NBS) in children. The clinical, brain magnetic resonance imaging and laboratory data of 5 children with NBS diagnosed in the Department of Pediatrics, General Hospital of Ningxia Medical University and Department of Rheumatology and Immunology, Children's Hospital Affiliated to Capital Institute of Pediatrics from April 2014 to April 2024 were analyzed retrospectively. The follow-up method was retrospective outpatient or inpatient visit to evaluate the treatment effect of NBS. Among the 5 NBS cases, 2 were male and 3 were female. The age of admission ranged from 8 to 17 years, the time from onset to diagnosis was 2 days to 4 years. Two patients had dizziness, headache and convulsions during the treatment of NBS, 1 patient had disturbance of consciousness, 1 patient gradually developed aphasia, limb movement disorder, dysphagia and muscle weakness after 4 years of Behçet's syndrome, and 1 patient had no clinical symptoms. C-reactive protein and erythrocyte sedimentation rate were increased in 4 cases, and cerebrospinal fluid white blood cells and immunoglobulin G were increased in 1 case. Brain magnetic resonance imaging of 4 children showed multiple lesions, including bilateral frontal lobe, occipital lobe, parietal lobe, periventricular and corpus callosum lesions. Brain magnetic resonance imaging showed multiple demyelinating diseases in 1 case, and cervical and thoracic magnetic resonance imaging showed slender cervical and thoracic spinal cord. All patients were treated with corticosteroids combined with immunosuppressants or biological agents. The children were followed up for 6 months to 4 years, and 4 cases had good treatment results, and 1 case finally gave up treatment. The clinical manifestations of NBS are not specific, and brain magnetic resonance imaging shows that the lesion location and morphology are not specific. NBS children treated with corticosteroids combined with immunosuppressive agents or biological agents have a good prognosis.

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http://dx.doi.org/10.3760/cma.j.cn112140-20240810-00568DOI Listing

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