[Behçet's disease in children: cortical calcifications].

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.