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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
Insights
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.
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.
- 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.
Introduction:
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.
Case Report:
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.
Conclusion:
We present a girl with multisystemic disorders characteristic of the Behçet s disease. Initially she had an aseptic meningoencephalitis developing cortical calcifications.