Hydrocephalus and macrocephaly: New manifestations of neonatal lupus erythematosus

Abstract

Objective. Neurologic involvement has been reported to occur in infants with neonatal lupus erythematosus (NLE), although the significance of these findings is unknown. The purpose of this study was to determine if hydrocephalus/ macrocephaly is a manifestation of NLE. Methods. Infants from the Hospital for Sick Children in Toronto, Canada whose mothers had anti-Ro antibodies were followed prospectively. A total of 87 infants were seen in the study period (1999 -2004). The maternal autoantibody status of all infants was documented, and all infants underwent full clinical examination at each visit. Results. Of the 87 infants, 47 had NLE. Five of the 47 infants with NLE and 2 of the healthy infants had hydrocephalus, resulting in a prevalence of 8.0% (95% confidence interval 4.0 -15.0%) in the entire cohort. This is significantly higher than the prevalence in the general population of 0.048 -0.081%. Head circumference measurements of infants in the Toronto cohort were largest between 12-24 months of age (Z score difference ‫؍‬ 0.71, P ‫؍‬ 0.008). Conclusion. We suggest that hydrocephalus and macrocephaly are manifestations of NLE and that infants born to mothers with anti-Ro antibodies should be carefully monitored for hydrocephalus as part of their routine physical examination.

Neonatal Lupus Erythematosus

“…A statistically significant association has been observed between neonatal intraventricular hemorrhage and lenticulostriate vasculopathy in infants with NLS [66]. The prevalence of hydrocephalus and macrocephaly also is increased in infants born to mothers who have anti-SSA/Ro antibodies [63].…”

“…Radiologic evaluations have shown evidences of white matter disease, cerebral edema, hypomyelination, echogenic lenticulostriate vessels, basal ganglia calcifications, gangliothalamic vasculopathy, subependymal cysts, haemorrhages, and ventriculomegaly [57,[63][64][65]. Less common radiological findings in infants who had NLS include thalamostriatal malformations, absent septum pellucidum, prominent cavum septum pellucidum, optic chiasm and optic nerve hypoplasia, and cortical dysgenesis.…”

Lupus eritematoso neonatal: reporte de cuatro casos

“…A greater frequency of abnormalities was found in these patients compared to healthy controls. 15,16 Differential diagnoses of skin lesions are made with seborrheic dermatitis, tinea corporis, psoriasis, atopic dermatitis, granuloma annulare, neonatal acne, erythema multiforme, Langerhans cell histiocytosis and congenital infections. 3 The diagnosis of NLE is established from characteristic clinical findings and the identification of the abovementioned antibodies in the mother and child.…”

Neonatal Lupus Erythematosus (NLE)

“…They are seen in less than 1% of patients in the form of myelopathy, aseptic meningitis, seizures with or without hypocalcaemia, myasthenia gravis (transient) [Kaye, 1987], hydrocephalus, microcephaly, macrocephaly, non-specific white matter changes, calcification of basal ganglia, vasculopathy and neuropsychiatric dysfunction/attention deficient disorders [Boros, 2007].…”