NEUROLOGIC ONSET OF BEHCETS-DISEASE - A DIAGNOSTIC ENIGMA IN CHILDHOOD

Neuro-Behcet's disease, which is uncommonly reported in childhood, enc ompasses a wide variety of clinical features since any part of the neu raxis may be involved. It carries a serious prognosis and represents a leading cause of death or severe disability. Neuro-Behcet's disease m ay occur in 5% to 50% of adults with Behcet's disease and is usually s ubsequent to other systemic manifestations. In this report, we express the possibility of a primary neurologic presentation of BehCet's dise ase in childhood with pseudotumor cerebri and meningoencephalitis as e xclusive initial features. We focus on diagnostic problems when major features of Behcet's disease are missing at the outset. We emphasize t he high sensitivity of magnetic resonance imaging to make the diagnosi s of cerebral vasculitis or thrombosis with a good reliability to clin ical features.