Behçet disease – a rare differential diagnosis of inflammatory CNS disease in children

Case report: Behçet disease is a perivasculitis of the small vessels resulting in a multisystem relapsing inflammatory disorder of unknown origin. It was Hulusi Behçet, a dermatologist, who first characterized the triad of oral and genital ulcers and uveitis in 1937. In approximately 13% of patients with Behçet's disease neural involvement can be observed.

We report the cases of two boys, 11 and 7 years old, who both presented with signs of encephalitis. The diagnosis of Behçet disease could be made using the criteria of the International Behçet study group. Start of treatment with high dose intravenous methylprednisolone (20 mg/kg/day) resulted in immediate improvement of the clinical picture in both cases.

With this poster, we would like to illustrate the very rare and versatile clinical picture of Neurobehçet's disease in these boys. Thereby, we would like to attract notice on the diagnosis of Behçet disease while considering differential diagnoses for inflammatory or demyelinating CNS disease in children.