The Clinical Application Of Cranial MRI, PET/CT And Neuron-specific Enolase In Children With Neuropsychiatric Lupus

Objective:Neuropsychiatric symptoms in patient with NPSLE are various.MRI is the first choice for the assessment of intracranial and spinal cord lesions,but there were few studies on MRI in children with NPSLE.The purpose of this study is to analysis the clinical manifestation,laboratory examination of children with NPSLE,and focus on MRI in children with NPSLE.Methods:Patients with NPSLE diagnosed at the department of paediatric in PUMCH between Jan 2012 and Jan 2017 were involved.The clinical and laboratory characteristics were analyzed.Brain MR images of the first episode of active NPSLE were reviewed.Results:The most common symptoms of NPSLE in children were headache(64.2%),convulsion(22.6%)and acute confusional state(15.1%).The other affected systems included skin(77.3%),hematological system(58.5%),and kidney(56.6%).Laboratory tests showed all patients were positive for ANA(100%)and 64.2%patients were positive for anti ds-DNA antibody.MRI abnormality was observed in19 patients(35.8%).The mainly head MRI findings were as follow:white matter hyper-intensities in 12 cases(63.2%),grey matter hyper-intensities in 2 cases(10.5%),cerebral infarction in 2 cases(10.5%),cerebral atrophy in 5 cases(26.3%),demyelination in one case(5.3%),and lacunar infarction in one case(5.3%).Elevated protein levels in CSF(P=0.003),positive anti-rRNP antibodies(P<0.001)and positive anti-Sm antibodies in serum(P=0.004)associated with abnormal MRI findings.Conclusions:The main symptoms of NPSLE were headache,convulsions and acute confusional state.The brain MRI of NPSLE patients mainly showed white matter hyper-intensities and brain atrophy.Elevated protein levels in CSF and positive anti-rRNP antibodies and positive anti-Sm antibodies in serum strongly associated with abnormal MRI findings.Objective:To investigate abnormalities of brain glucose utilization in relation to neuropsychiatric systemic lupus erythematosus(NPSLE).Methods:A case control study was conducted.Positron emission tomography/computed tomography(PET/CT)using F-18-labelled fluorodeoxy glucose was performed in 39 patients with SLE.Patients were classified as having neuropsychiatric manifestations(NPSLE group)(n=11),and without neuropsychiatric manifestations(non-NPSLE group)(n=28).Magnetic resonance imaging(MRN)was also performed in 39 patients.All patients of NPSLE group follow up PET/CT scans were performed.Results:PET/CT scans showed hypometabolism in at least one brain region in all patients in NPSLE group(100%).The temporal lobe(8/11)was the most susceptible brain regions,followed by the parietal lobe(6/11),occipital lobe(6/11)and frontallobe(6/11).Glucose hypermetabolism was detected in basal ganglia in 3 patients.In contrast,MRI images were abnormal in only 3 of 11(27.3%)patients in NPSLE group.PET/CT scans showed hypometabolism in 3 patients in non-NPSLE group.All patients of NPSLE group examined in follow up PET/CT scans,and improvement of cerebral symptoms were associated with increased brain glucose metabolism.Neuropsychiatric symptoms appeared in 3 patients in non-NPSLE group with abnormal PET/CT in follow up.Conclusions:PET/CT revealed significant glucose hypometabolism in patient with NPSLE.PET/CT imaging represents a sensitive tool to NPSLE or subclinical CNS involvement in SLE.PET/CT findings correlate well with the clinical course of disease.Objective:Neuropsychiatric systemic lupus erythematous(NPSLE)is a serious complication of systemic lupus erythematosus(SLE),and there is no specific serum biomarker for the diagnosis and follow-up of NPSLE.Neuron specific enolase(NSE)is an important neuronal glycolytic enzyme.The serum concentration of NSE in patients with multi infarct dementia,multiple sclerosis and depression was decreased.The aim of the study was to evaluate serum NSE levels in SLE patients with and without nervous system involvement,and in healthy controls,and to assess its clinical diagnostic value in NPSLE patients.Methods:A cross-sectional study was conducted.This study comprised 39 SLE patients and 11 controls,and SLE patients were sub-divided into NPSLE group(n=25)and non-NPSLE group(n=12)accroding to strict NPSLE criteria.NSE serum levels were measured with electrochemiluminescence assay.Results:The clinical manifestations of NPSLE patients included headache(12 patients),convulsions(3patients),cognitive reduce(3 patients),acute consciousness disorder(2 patients),anxiety(2 patients),cerebral vascular disease(1 patient),mental diorder(1 patient)and demyelination(1 patient).Serum levels of NSE significantly decreased in patients with NPSLE compared to the non-NPSLE patients(11.8±3.0ng/ml vs.15.2±3.7ng/ml,P<0.01),and the control group(11.8±3.0ng/ml vs.17.9±2.6ng/ml,P<0.01).Conclusions:Decreased serum concentration of NSE may reflect declined metabolism of the nervous tissue in patient with NPSLE.Peripheral neuronal specific enolase may be a useful marker for NPSLE.ObjectiveThe clinical manifestations of SLE with PRES and NPSLE are overlap.Both of them can manifested as seizures,headache,confusional state.It is difficult for doctor to differential diagnosis.So we want to study the clinical profile of the posterior reversible encephalopathy syndrome(PRES)in children with systemic lupus erythematosus(SLE).MethodsWe reviewed cases of RPLS with SLE including both admitted to our department and searched from the literature to analysis the lupus characteristics,PRES features,laboratory abnormalities,treatment and outcomes.ResultsWe reviewed 15 cases of PRES in children with SLE including our 4 cases(14children were girl).The minimum age was 8 years.15 children had seizures;10 had headache;7 had vomiting;9 had loss of consciousness and 7 had vision loss.Hypertension was seen in all 15 patients;12 had nephritis.All patients had brain magnetic resonance imaging(MRI)findings consistent with PRES.Antihypertensives and antiepileptics were main treatment.Glucocorticoid and immunosuppressive were used in 9 patients with active lupus,but they were withheld or reduced if the child was not accompanied by active lupus.All patients improved without neurological deficit.Repeat MRI were done in 9 patients and showed complete resolution of abnormalities.ConclusionsPRES in children with SLE presented with seizures,headache,vomiting,loss of consciousness and vision loss.SLE patients with lupus nephritis,hypertension and use of immunosuppressive could precede the occurrence of PRES.Brain MRI is important for diagnosis of PRES.