Central Nervous System Involvement in Neonatal Lupus Erythematosus

Abstract

Computerized tomography (CT) of the brain was performed in 10 of 11 consecutive infants with neonatal lupus erythematosus (NLE) (five boys and six girls). Ten of the 11 infants had brain neurosonography. Nine of 10 infants had abnormal CT scans. There was diffuse, markedly reduced attenuation of the cerebral white matter in four infants studied in the first week of life, and also in an infant 5 weeks of age. Patchy reduced subcortical white matter attenuation was observed in another 5-week-old infant. Basal ganglia calcifications were present in two infants at 2 months of age, one of whom also had mild ventriculomegaly. A patient with macrocephaly studied at 4 months of age had enlarged ventricles and subarachnoid spaces consistent with benign macrocephaly of infancy. Cerebral ultrasound examination was abnormal in all five infants studied in the first week of life and in one infant at 2 months of age. Findings included subependymal cysts (4), echogenic white matter (3), and echogenic lenticulostriate vessels (3). Apart from one case of macrocephaly, there was no clinical evidence of neurologic disease and the subsequent development of these infants has been normal. Subclinical central nervous system (CNS) disease in NLE is likely to be a transient phenomenon that resolves as maternal antibodies are cleared from the infant's circulation. It is important to be aware of these neuroimaging abnormalities to avoid misdiagnosis of congenital viral infection in a newborn with multisystem NLE. The potential for neurologic sequelae is uncertain.

Proliferating brain cells are a target of neurotoxic CSF in systemic autoimmune disease

“…Although neonatal lupus is often a self-limiting condition, brain imaging analyses of infants reveal subclinical CNS disease, including vasculopathy (Cabanas et al, 1996), attenuations in white matter, and mild ventricular enlargement (Prendiville et al, 2003). The supportive evidence for autoimmunity-induced brain damage during embryogenesis and behavioral dysfunction in adulthood has been obtained by transfer of fertilized ova from healthy animals to the uterus of autoimmune females (Denenberg et al, 1991).…”

Neonatal Lupus Erythematosus

“…Trans-placental passage of maternal auto-antibodies results in lenticulostriate vasculopathy and subsequent brain abnormalities as cerebral dysmaturation, cortical dysgenesis, ventriculomegaly, and dysgenesis of structures supplied by the lenticulostriate vasculature in infants with NLS [57][58][59].…”

“…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.…”