[Behçet's disease in children: cortical calcifications].
キーワード
概要
BACKGROUND
Between 5 and 48% of the patients with Behçet s disease have neurological abnormalities. In children, meningoencephalitis as an initial manifestation of the disease is not frequent.
METHODS
We present a four years old girl with a Behçet s disease. When she was six months of age, during an hospitalization caused by malnutrition and respiratory infection, she had developed an aseptic meningoencephalitis and laboratory test showed hypergammaglobulinemia. When she was 21 months old, she had seizures as epilepticus status, and good response was achieve treating with anticonvulsive drugs. She did not repeat episodes of epileptic seizures, but the patient showed language and neuromotor problems. CT was abnormal with asymmetric ventricular dilatation and increased subarachnoides cortical sulci with multiple calcifications in those areas. She also suffered respiratory and gastrointestinal infections that required hospitalization. At the age of 3 years and 6 months, she had occular affection with uveitis, oral and genital ulceration. The clinical history of the girl consisting of meningoencephalitis, gastrointestinal and respiratory disorders, later presentation of uveitis and oral and genital ulcers confirmed the Behçet s disease diagnosis.
CONCLUSIONS
We present a girl with multisystemic disorders characteristic of the Behçet s disease. Initially she had an aseptic meningoencephalitis developing cortical calcifications.
