Focal seizures as an unusual presentation of neonatal lupus erythematosus

Neonatal lupus erythematosus is an uncommon passive autoimmune disease in which there is a transplacental passage of anti-Ro/SSA and/or anti-La/SSB maternal autoantibodies. Common clinical manifestations include cardiac disease, notably congenital heart block, cutaneous lupus lesions, hematologic disorders, and hepatobiliary disease. During the past decade, however, it has become clear that central nervous disease may also be a manifestation of neonatal lupus. We report a male neonate with the disease who had focal seizures in addition to cutaneous lupus, anemia, and thrombocytopenia. Brain ultrasound revealed normal ventricular size without a midline shift or intracranial or intraventricular hemorrhage. A brain CT showed generalized low density involving the periventricular and deep white matter. A sleep EEG revealed rare spikes axial to the right parietal lobe. The neonate had a high titer of antinuclear antibodies (1:640) with a speckled pattern, anti-Ro/SSA and anti-La/SSB antibodies, but no anti-dsDNA antibodies. He was given anti-convulsant drugs with dramatic improvement of his symptoms. One month later, a sleep EEG was normal, and he had no further seizures.