Pattern of neuropsychiatric manifestations and outcome in juvenile systemic lupus erythematosus

Abstract

The aim of this study was to study the neuropsychiatric (NP) manifestations, diagnostic evaluation, treatment and outcome in juvenile systemic lupus erythematosus (SLE). We reviewed the charts of all children with SLE and evidence of NP manifestations as defined by the presence of at least one of the following: headache, cerebrovascular accident (CVA), chorea, seizure, papilledema, and psychiatric or spinal cord manifestations. Out of 90 children with SLE, 20 (16 female) had NP manifestations. The mean age at onset was 8.8 years. The mean period between onset of SLE and NP manifestations was 10.2 months. NP manifestations were the presenting feature in 3 patients. Eleven patients had headache, 10 had psychiatric manifestations, 10 had seizure and 6 had CVA. Coma was seen in 5 patients, chorea in 4, transverse myelitis in 2 and papilledema in 2. Anticardolipin antibodies were high in 12 patients. Five patients had an abnormal CSF study. Nine patients had EEG abnormalities and 13 showed MRI abnormalities. All patients received oral prednisone and 17 were treated with IVMP and immunosuppressive therapy (cyclophosphamide or azathioprine); 85% of our patients recovered completely, but 15% had persistent NP sequelae; 10% died from severe infection. In conclusion, NP involvement in juvenile SLE is common. However, early diagnosis and early treatment with adjunctive intravenous pulse cyclophosphamide may improve the outcome.

Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system

“…Prevalence of abnormal EEG findings in JSLE with neuropsychiatric manifestations has been reported in 45-71% of the patients 14,27,30 , which is in agreement with our 53.8% rate. EEG analysis in adult SLE patients has suggested that, in those with neurological involvement, the left hemisphere, namely the left temporal region, is selectively involved 48,49 .…”

“…This may be due to a bias generated by the presence of a large Nephrology Unit at our hospital. As observed in our series, the most frequent cause of death reported is infection 30 .…”

“…Mortality rates of JSLE vary from 4 to 10% 14,27,30,31 , which is lower than the 12.8% found at our series. The high prevalence of renal involvement in our sample (70.2%) and in the group of lethal evolution (100%) implicates in disease severity.…”

“…Pediatric series report prevalences of 7-10% 26,31 , 5% 25 and 2-3.5% 26,31 respectively at disease presentation. These rates rise to 10-20% for headache and 6.6-12% for stroke during the course of the disease 27,30,35 .…”

“…Although it is considered a rare SLE manifestation in adults 2 , it is more frequent in JSLE 2 and has been observed in 0.6-2.3% at disease onset 26,31 and in 1-6% during disease course 30,32,35 .…”