Unusual presentation of neonatal Behcets disease

Intrauterine growth retardation and neonatal transient mucocutaneous lesions ("transient Behcet syndrome") have been reported in pregnancies complicated by Behcets disease (BD). Neonatal neurological manifestations have not been reported in such pregnancies. Vascular and neurological involvement is known to worsen the prognosis in adults with BD. The clinical course and outcome of a 34-weeks' gestation neonate born to a mother with BD is reported. Progressive recovery from minimal respiratory distress syndrome was followed by catastrophic presentation on 6th day of life with generalized seizures. Cranial ultrasound revealed multiple hyperechoic lesions in the frontal, parietal, and periventricular regions with a few surrounded by a ring of reduced echogenicity suggesting haemorrhage into ischemic areas. Death occurred after withdrawal of life support on Day 9, after extensive discussions with parents in view of the progressive deterioration in the neonates' general condition and the cranial ultrasound findings. Strong family history of BD, clinical course, and laboratory results (no evidence of disseminated intravascular coagulation, normal levels of protein C and S, absence of factor V Leiden and anticardiolipin antibodies) suggested neurological manifestations of BD as the most probable diagnosis.