Juvenile neuropsychiatric systemic lupus erythematosus: A specific clinical phenotype and proposal of a probability score.

OBJECTIVE: Juvenile systemic lupus erythematosus (j-SLE) is a rare chronic auto-immune disease involving several organs. Neuropsychiatric (NP) SLE (NPSLE) is frequent in j-SLE and associated with increased morbidity/mortality. Although NPSLE classification criteria exist, attributing NP features to j-SLE remains a major challenge. The study objective is to thoroughly describe j-NPSLE patients and assist in their diagnosis. METHODS: This is a 4-year retrospective monocentric study of j-SLE patients. NP events were attributed to j-SLE using standardised diagnostic criteria and multidisciplinary paediatric clinical expertise. Clinical features, brain magnetic resonance imaging (MRI)s and samples analysis including cerebrospinal fluid were assessed. A risk of j-NPSLE score was developed based on multivariable logistic regression analysis. RESULTS: Of 39 patients included, 44% were identified as having j-NPSLE. J-NPSLE diagnosis was established at the onset of j-SLE in 59% of patients. In addition to frequent kidney involvement (76%) and chilblains (65%), all j-NPSLE patients displayed psychiatric features: cognitive symptoms (82%), hallucinations (76%), depressed mood (35%), acute confused state (18%) and catatonia (12%). Neurological involvement was often mild and nonspecific, with headache (53%) in about half of the patients. The main features reported on brain MRI were nonspecific T2/FLAIR white matter hyperintensities (65%), and cerebral atrophy (88%). Upon immunosuppressive treatment, clinical improvement of NP features was observed in all j-NPSLE patients. The score developed to attribute j-NPSLE probability, guide further investigations and appropriate treatments is based on hallucinations, memory, sleep and renal involvement (Sensitivity: 0.95 Specificity: 0.85). Cerebrospinal fluid (CSF) neopterin assessment increases the score sensitivity and specificity. CONCLUSION: Physicians should carefully and systematically assess the presence of NP features at diagnosis and early stages of j-SLE. For j-NPSLE patients with predominant psychiatric features, a multidisciplinary collaboration, including psychiatrists, is essential for the diagnosis, management and follow-up.

Structural Brain MR Imaging Alterations in Patients with Systemic Lupus Erythematosus with and without Neuropsychiatric Events.

BACKGROUND AND PURPOSE: Systemic lupus erythematosus is a complex autoimmune disease known for its diverse clinical manifestations, including neuropsychiatric systemic lupus erythematosus, which impacts a patient's quality of life. Our aim was to explore the relationships among brain MR imaging morphometric findings, neuropsychiatric events, and laboratory values in patients with systemic lupus erythematosus, shedding light on potential volumetric biomarkers and diagnostic indicators for neuropsychiatric systemic lupus erythematosus. MATERIALS AND METHODS: Twenty-seven patients with systemic lupus erythematosus (14 with neuropsychiatric systemic lupus erythematosus, 13 with systemic lupus erythematosus), 24 women and 3 men (average age, 43 years, ranging from 21 to 62 years) were included in this cross-sectional study, along with 10 neuropsychiatric patients as controls. An MR imaging morphometric analysis, with the VolBrain online platform, to quantitatively assess brain structural features and their differences between patients with neuropsychiatric systemic lupus erythematosus and systemic lupus erythematosus, was performed. Correlations and differences between MR imaging morphometric findings and laboratory values, including disease activity scores, such as the Systemic Lupus Erythematosus Disease Activity Index and the Systemic Lupus International Collaborating Clinics Damage Index, were explored. An ordinary least squares regression analysis further explored the Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index relationship with MR imaging features. RESULTS: For neuropsychiatric systemic lupus erythematosus and non-neuropsychiatric systemic lupus erythematosus, the brain regions with the largest difference in volumetric measurements were the insular central operculum volume (P value = .003) and the occipital cortex thickness (P = .003), which were lower in neuropsychiatric systemic lupus erythematosus. The partial correlation analysis showed that the most correlated morphometric features with neuropsychiatric systemic lupus erythematosus were subcallosal area thickness asymmetry (P < .001) and temporal pole thickness asymmetry (P = .011). The ordinary least squares regression analysis yielded an R 2 of 0.725 for the Systemic Lupus Erythematosus Disease Activity Index score, with calcarine cortex volume as a significant predictor, and an R 2 of 0.715 for the Systemic Lupus International Collaborating Clinics Damage Index score, with medial postcentral gyrus volume as a significant predictor. CONCLUSIONS: The MR imaging volumetric analysis, along with the correlation study and the ordinary least squares regression analysis, revealed significant differences in brain regions and their characteristics between patients with neuropsychiatric systemic lupus erythematosus and those with systemic lupus erythematosus, as well as between patients with different Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index scores.

Effects of ropivacaine combined with morphine at 0.15 and 0.2 mg kg-1 in bitches undergoing epidural anesthesia

PURPOSE: To investigate cardiorespiratory effects and serum concentration of ropivacaine combined with morphine at different doses. METHODS: Sixteen healthy adult female dogs weighting 9.8±4.1 kg were included in the study. Twenty minutes after being premedicated with acepromazine and midazolam, the animals were randomly assigned to receive an epidural injection according to each group: RM0.15 = ropivacaine + morphine (0.15 mg kg-1) and RM0.2 = ropivacaine + morphine (0.2 mg kg-1). Variables recorded consisted of: heart rate and cardiac rhythm, respiratory rate, oxyhemoglobin saturation, inspired oxygen fraction, end-tidal carbon dioxide tension, systolic, mean and diastolic arterial pressures, serum cortisol, plasma ropivacaine and morphine. RESULTS: SAP, MAP and DAP were significantly increased at TPR in RM0.15 but returned to normal values at the end of the procedure. Arterial pH was decreased in T30 and TESu in both groups and also returned to acceptable ranges at TR. Both PaO2 and PaCO2 were increased along the duration period of the epidural blockade (T30 and TESu) and returned to acceptable values at TR. Serum cortisol was lower at TB, T30 and TR when compared to TESu. CONCLUSION: The procedures were performed safely and minimal changes in cardiovascular and respiratory variables. .

Longitudinally extensive transverse myelitis with neuro-lymphatic protein expressionin systemic lupus erithematosus patient. An autopsy case report / Mielitis transversa longitudinal extensa con expresión proteica neurolinfática en un paciente con lupus eritematoso sistémico. Reporte de una autopsia

Acute transverse myelitis (inflammation across one or more segments of spinal cord) is a rare complication of systemic lupus erythematosus (SLE) although its frequency is greater than in the general population. Even less common is longitudinal extensive transverse myelitis (LETM), (inflammation affects three or more vertebral segments). The pathogenesis of LETM is unclear and the management uncertain. We present a case of a 34-year-old woman with SLE and LETM of the whole spine, with rapid progression despite intensive treatment. Autopsy revealed a spine with liquefactive necrosis; some vessels showed fibrinoid necrosis and there were thrombi and an infiltration of lymphocytes and neutrophils in both the grey and white matter. Histological examination of brain revealed necrosis and oedema in the cortex and around the lateral ventricles. The immunohistochemistry showed CD3-positive T-lymphocytes in the wall of the spinal blood vessels, and a prominent D2-40 immunostaining, mainly localized at perivascular inflammatory regions (AU)

La mielitis aguda transversa (inflamación en uno o más segmentos de la médula espinal) es una complicación muy rara, con mayor prevalencia en los pacientes con lupus eritematoso sistémico que en la población general. Mucho menos frecuente es que esta inflamación afecte a 3 o más segmentos espinales. Su patogénesis no está bien definida y el tratamiento es incierto. Presentamos un caso de autopsia de una mujer de 34 años de edad con lupus eritematoso sistémico y mielitis transversa longitudinal extensa con un evolución clínica rápidamente desfavorable. Se encontró una médula espinal con necrosis licuefactiva, necrosis fibrinoide de la pared de los vasos, trombos en los mismos y una infiltrado inflamatorio de linfocitos y neutrófilos en la sustancia gris y blanca. En el cerebro había necrosis y edema de la corteza y alrededor de los ventrículos laterales. El estudio inmunohistoquímico mostró linfocitos T CD3 positivos en la pared de los vasos, con una fuerte expresión de D2-40 perivascular (AU)

Análisis espectral del electroencefalograma en niños con lupus eritematoso sistémico / Spectral analysis of the electroencephalogram in children with systemic lupus erythematosus

Introducción. El lupus eritematoso sistémico (LES) es una enfermedad autoinmunitaria que afecta a diferentes sistemas y órganos, incluido el sistema nervioso central (SNC). Se ha postulado que aproximadamente el 40% de las manifestaciones de lupus neuropsiquiátrico (LESNP) se desarrolla antes de que se diagnostique el LES o en el mismo momento en el que se realiza el mismo, y el 63% aparece durante el primer año después del diagnóstico. Objetivos. Evaluar la hipótesis de que el electroencefalograma (EEG) puede ser sensible al daño del SNC en niños con LES, aun sin evidencia clínica de LESNP. Pacientes y métodos. Se registró el EEG de 30 niños con diagnóstico de LES, con o sin manifestaciones de síndrome neuropsiquiátrico. Se realizó la evaluación visual y el análisis cuantitativo del EEG. Resultados. La inspección visual del EEG mostró la presencia de alteraciones en el 44,5% de los niños con LES y en el 76,9% con LESNP. La t de Student fue significativamente diferente (p 0,0055) entre ambos grupos para el análisis de las medidas espectrales de banda ancha. El análisis de banda estrecha mostró un incremento significativo en las frecuencias theta y delta en los niños con LES con respecto a los valores normativos, mientras que en los niños con LESNP las diferencias significativas se encontraron en las bandas rápidas en las regiones frontales. Conclusiones. El análisis espectral de banda estrecha podría ayudar a confirmar el diagnóstico de LESNP, mientras que las anomalías en las bandas lentas podría ser un marcador temprano de daño del SNC, aun en ausencia de síntomas de la enfermedad

Introduction. Systemic lupus erythematosus (SLE) is an autoimmune disease that affects different systems and organs, including the central nervous system (CNS). It has been suggested that about 40% of the cases of neuropsychiatric lupus (NPSLE) develop before SLE is diagnosed or at the same time it is being carried out, and 63% appear during the first year following diagnosis. Aims. The aim of this study was to check the hypothesis that the electroencephalogram (EEG) may be sensitive to the damage to the CNS in children with SLE in whom there is still no clinical evidence of NPSLE. Patients and methods. EEG recordings were performed in 30 children with a diagnosis of SLE with or without signs of a neuropsychiatric syndrome. The results of the EEG were evaluated visually and analysed quantitatively. Results. The visual inspection of the EEG showed the presence of alterations in 44.5% of the children with SLE and in 76.9% of those with NPSLE. There were significant differences in Student’s t test (p = 0.0055) between the two groups for the analysis of the broadband spectral measurements. The narrow band analysis revealed a significant increase in the theta and delta frequencies in children with SLE as compared to standard values, whereas in children with NPSLE significant differences were found in the fast bands in frontal regions. Conclusions. Spectral analysis of the narrow band could help to confirm diagnoses of NPSLE, while anomalies in the slow bands could be an early marker of damage to the CNS although there are still no symptoms of the disease