Neurologic manifestations of pediatric systemic lupus erythematosus

Steinlin, Maja; Blasér, Susan; Gilday, David L.; Eddy, Allison A.; Logan, William J.; Laxer, Ronald M.; Silverman, Earl D.

doi:10.1016/0887-8994(95)00110-2

Cited by 143 publications

(81 citation statements)

References 22 publications

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“…Ravelli et al volvement, particularly central nervous system disease, is a leading cause of morbidity and mortality in juvenileonset SLE and is frequently observed either at onset or diagnosis (38,39). Our finding suggests that patients with juvenile-onset SLE who experience early NPS manifestations deserve close monitoring and careful treatment because they may be at highest risk for the subsequent development of permanent organ damage.…”

Section: Discussionmentioning

confidence: 55%

Assessment of damage in juvenile‐onset systemic lupus erythematosus: A multicenter cohort study

Ravelli¹,

Duarte-Salazár²,

Buratti³

et al. 2003

Arthritis & Rheumatism

154

131

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Objective. To investigate the prevalence of cumulative organ damage in patients with juvenile-onset systemic lupus erythematosus (SLE) and its association with demographic and clinical variables, medication use, and quality of life. Methods. The occurrence of organ system damage, as measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), was determined for 387 patients consecutively enrolled in pediatric rheumatology centers from Europe, the US, Mexico, and Japan. Risk factors for damage included demographic variables; clinical manifestations at diagnosis; previous corticosteroid, immunosuppressive, and antimalarial therapies; disease activity; and quality of life. Results. Overall, 195 (50.5%) patients had damage within a mean of 5.7 years after disease onset. Renal (21.8%) and neuropsychiatric (15.8%) system involvement were observed most frequently, followed by musculoskeletal (11.7%), ocular (10.9%) and skin (9.6%) system involvement, with a mean SDI score of 1.1. In multivariate models, the occurrence of neuropsychiatric manifestations at diagnosis, a longer disease duration, and a greater number of intravenous cyclophosphamide pulses showed the strongest association with the presence of damage. Conclusion. We found evidence of cumulative organ damage, as measured by the SDI, in half of the patients with juvenile-onset SLE. Damage was significantly more likely in patients who had experienced neuropsychiatric manifestations at diagnosis, had a longer disease duration, and had received more intravenous pulses of cyclophosphamide.

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Section: Discussionmentioning

confidence: 55%

Assessment of damage in juvenile‐onset systemic lupus erythematosus: A multicenter cohort study

Ravelli¹,

Duarte-Salazár²,

Buratti³

et al. 2003

Arthritis & Rheumatism

154

131

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“…In a study on 19 children and adolescents with migraine, SPECT showed hypoperfusion areas in 3/6 patients with classic migraine and in 1/3 patients with hemiplegic migraine (15). Steinlin et al (6) studied nine JSLE children with headache; four of them were submitted to SPECT and abnormalities were found in only one. In our study, we observed SPECT changes in two patients reporting headache.…”

Section: Discussionmentioning

confidence: 99%

Use of single photon emission computed tomography and magnetic resonance to evaluate central nervous system involvement in patients with juvenile systemic lupus erythematosus

Prismich

Hilário

Len

et al. 2002

Braz J Med Biol Res

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The objective of the present study was to identify the single photon emission computed tomography (SPECT) and magnetic resonance (MR) findings in juvenile systemic lupus erythematosus (JSLE) patients with CNS involvement and to try to correlate them with neurological clinical history data and neurological clinical examination. Nineteen patients with JSLE (16 girls and 3 boys, mean age at onset 9.2 years) were submitted to neurological examination, electroencephalography, cerebrospinal fluid analysis, SPECT and MR. All the evaluations were made separately within a period of 15 days. SPECT and MR findings were analyzed independently by two radiologists. Electroencephalography and cerebrospinal fluid analysis revealed no relevant alterations. Ten of 19 patients (53%) presented neurological abnormalities including present or past neurological clinical history (8/19, 42%), abnormal neurological clinical examination (5/19, 26%), and abnormal SPECT or MR (8/19, 42% and 3/19, 16%, respectively). The most common changes in SPECT were cerebral hypoperfusion and heterogeneous distribution of blood flow. The most common abnormalities in MR were leukomalacia and diffuse alterations of white matter. There was a correlation between SPECT and MR (P<0.05). We conclude that SPECT and MR are complementary and useful exams in the evaluation of neurological involvement of lupus.

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“…22,26,27 Psychosis, however, appears more common in children than adults with NPSLE, affecting between 7.5-12%. 2,[28][29][30] A recent study of 500 adult SLE patients followed for 30 years, in contrast, reported a 2% prevalence of psychosis.…”

Section: Differences Between Childhood and Adult Systemic Lupus Erythmentioning

confidence: 99%

“…132,133 Additionally there is an interesting discrepancy of demonstrated focal hypoperfusion defects in context of diffuse or multifocal neurological manifestations, such as psychosis. 26,134 Again, such studies are limited by small sample sizes.…”

Section: Neuroimagingmentioning

confidence: 99%

Pathogenesis of neurocognitive and neuropsychiatric manifestations in childhood-onset lupus: an overview

2014

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This review explores current understanding of neuropsychiatric systemic lupus erythematosus (NPSLE) of childhood onset, in particular neurocognitive impairment. As yet, fewer studies have focused on childhood onset NPSLE compared to adult onset NPSLE and diagnosis still involves the 1999 American College of Rheumatology case definitions of neuropsychiatric syndromes, which were developed for adults. Although a validated core set of neuropsychometric tests exist for childhood onset NPSLE, these still have limitations and possible biomarkers and newer neuroimaging modalities remain mostly experimental. Important differences exist between childhood and adult onset SLE and specifically with NPSLE, outlined in this review. Normal adolescent brain development also involves significant differences from adults, particularly in executive function and social cognition. These issues may impact on the pathogenesis of NPSLE during this vulnerable period and also influence their management options.

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Neurologic manifestations of pediatric systemic lupus erythematosus

Cited by 143 publications

References 22 publications

Assessment of damage in juvenile‐onset systemic lupus erythematosus: A multicenter cohort study

Assessment of damage in juvenile‐onset systemic lupus erythematosus: A multicenter cohort study

Use of single photon emission computed tomography and magnetic resonance to evaluate central nervous system involvement in patients with juvenile systemic lupus erythematosus

Pathogenesis of neurocognitive and neuropsychiatric manifestations in childhood-onset lupus: an overview

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