title: Evaluation of magnetic resonance imaging abnormalities in juvenile onset neuropsychiatric systemic lupus erythematosus.
abstract: The aim of this study was to describe the abnormalities identified with conventional MRI in children with neuropsychiatric systemic lupus erythematosus (NPSLE). This was single-centre (Great Ormond Street Hospital, London) retrospective case series of patients with juvenile NPSLE seen in 2003-2013. Brain MR images of the first episode of active NPSLE were reviewed. All patients fulfilled the 1999 ACR case definitions for NPSLE syndromes. Presenting neuropsychiatric manifestations, immunological findings and treatment are reported. Results are expressed as median and ranges or percentages. Fisher's exact test was used to identify clinical predictors of abnormal MRI. A total of 27 patients (22 females), median age 11 years (4-15), were identified. Presenting clinical symptoms included the following: headaches (85.1 %), mood disorder/depression (62.9 %), seizures (22.2 %), acute psychosis (18.5 %), cognitive dysfunction (14.8 %), movement disorder (14.8 %), acute confusional state (14.8 %), aseptic meningitis (7.4 %), demyelinating syndrome (3.7 %), myelopathy (3.7 %), dysautonomia (3.7 %) and cranial neuropathy (3.7 %). The principal MR findings were as follows: (1) absence of MRI abnormalities despite signs and symptoms of active NPSLE (59 %); (2) basilar artery territory infarction (3 %); (3) focal white matter hyperintensities on T2-weighted imaging (33 %); (4) cortical grey matter lesions (3 %); and (5) brain atrophy (18.5 %). The presence of an anxiety disorder strongly associated with abnormal MRI findings (p = 0.008). In over half the children with NPSLE, no conventional MRI abnormalities were observed; white matter hyperintensities were the most commonly described abnormalities. Improved MR techniques coupled with other alternative diagnostic imaging modalities may improve the detection rate of brain involvement in juvenile NPSLE.
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Al-Obaidi
authors.affiliation: Radiology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: D
authors.initials: D
authors.lastname: Saunders
authors.affiliation: Radiology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: S
authors.initials: S
authors.lastname: Brown
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: L
authors.initials: L
authors.lastname: Ramsden
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: N
authors.initials: N
authors.lastname: Martin
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: E
authors.initials: E
authors.lastname: Moraitis
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: C A
authors.initials: CA
authors.lastname: Pilkington
authors.affiliation: Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Institute of Child Health, London, WC1E1HN, UK.
authors.forename: P A
authors.initials: PA
authors.lastname: Brogan
authors.affiliation: Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Institute of Child Health, London, WC1E1HN, UK. d.eleftheriou@ucl.ac.uk.
authors.forename: D
authors.initials: D
authors.lastname: Eleftheriou
mesh_terms: D000293:Adolescent; D001284:Atrophy / Q000000981:diagnostic imaging / Q000473:pathology; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006261:Headache / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D020945:Lupus Vasculitis, Central Nervous System / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D019964:Mood Disorders / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies
keywords: Juvenile SLE; Magnetic resonance imaging; Neuropsychiatric SLE