Saini Arushi G, Sankhyan Naveen, Bhattad Sagar, Vyas Sameer, Saikia Biman, Singhi Pratibha
Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India.
Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India.
Eur J Paediatr Neurol. 2014 May;18(3):444-8. doi: 10.1016/j.ejpn.2014.01.007. Epub 2014 Jan 25.
Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has been described as transient vasculopathy only. We describe a 2-months-old girl who presented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symptomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case highlights that neonatal lupus erythematosus can present with isolated nervous system manifestations and the vascular damage can be permanent in the form of vasculitis. Early recognition will help pediatricians identify such possible permanent complications in newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented.
新生儿红斑狼疮是指母亲体内存在抗Ro/SSA和La/SSB抗原自身抗体的新生儿所出现的心脏、皮肤及其他全身异常的临床症候群。孤立的中枢神经系统受累非常罕见,仅被描述为短暂性血管病变。我们报告一例2个月大的女孩,她因中枢神经系统血管炎继发急性缺血性卒中,无任何心脏、皮肤或血液学表现。母亲症状轻微,抗Ro自身抗体滴度升高;婴儿抗Ro抗原自身抗体呈阳性。血管造影证实脑血管存在血管炎。我们的病例强调,新生儿红斑狼疮可表现为孤立的神经系统表现,血管损伤可呈血管炎形式而永久存在。早期识别有助于儿科医生识别新生儿红斑狼疮患儿可能出现的此类永久性并发症。本文还对先前报道的新生儿红斑狼疮中枢神经系统表现进行了综述。