Aline Tamires Lapa

Neurologic Involvement in Scleroderma en Coup de Sabre

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.

Prevalence and clinical significance of anti-ribosomal P antibody in childhood-onset systemic lupus erythematosus.

Objective: To investigate the prevalence of the anti-ribosomal P (anti-P) antibodies in childhood-onset systemic lupus erythematosus patients (cSLE), healthy controls and first degree relatives. To elucidate the association between anti-P and disease activity, laboratory and treatment features in cSLE patients. Methods: We included consecutive SLE patients with disease onset before 16 years. Controls were age- and sex-matched. SLE patients were assessed for clinical and laboratory SLE manifestations, disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and current drug exposures. Mood disorders were determined through Becks Depression and Becks Anxiety Inventory. Anti-P measured by enzyme-linked immunosorbent assay. Results: We included 50 consecutive cSLE patients (mean age of 16.82 ± 3.46 years), 35 first degree relatives (mean age of 38.73 ± 3.89 years) and 20 health control (mean age of 18.3 ± 4.97 years). Anti-P was observed in 13 (26%) cSLE patients and in no first-degree relative (p < 0.01) or control (p < 0.01). Anti-P was more frequently observed in patients with anxiety (p < 0.002). No other clinical, laboratory or treatment features, including SLEDAI and SDI scores were associated with the presence of anti-P in cSLE patients. Conclusion: Anti-P is frequently observed in cSLE patients and was associated with the presence of anxiety in this cohort of cSLE.

Reduction of Cerebral and Corpus Callosum Volumes in Childhood-Onset Systemic Lupus Erythematosus: A Volumetric Magnetic Resonance Imaging Analysis.

There have been few studies in which the prevalence of cerebral atrophy in childhood‐onset systemic lupus erythematosus (SLE) was evaluated using magnetic resonance imaging (MRI) volumetric measurements. This study was undertaken to determine the prevalence of cerebral and corpus callosum atrophy in childhood‐onset SLE and to determine the possible relationships between atrophy and clinical, laboratory, and treatment features of the disease.

Cognitive impairment in antiphospholipid syndrome: evidence from animal models

Although antiphospholipid syndrome (APS) is a multisystem prothrombotic condition, its inflammatory nature has been increasingly recognized in recent years. Stroke and transitory ischemic attacks are the neurological manifestations included in APS criteria, however many other neurological involvements have been attributed to antiphospholipid antibodies (aPL), such as seizures, transverse myelitis, and cognitive impairment. In this article we will review evidence from animal model that explain the role of aPL in cognition.

Cognitive Dysfunction and Antiphospholipid Antibodies

Antiphospholipid syndrome (APS) is a multisystem prothrombotic condition, however, in recent years, its inflammatory nature has been studied extensively. Cerebral involvement is commonly observed in APS and results in different clinical manifestations. However, most of the studies include secondary APS. In this article, we review the prevalence, clinical characteristics, and physiopathology of cognitive impairment in patients with primary APS.

Schistosoma mansoni infection: an immune complex disease presenting with polyarthritis

Schistosomiasis or bilharzia is a parasitic disease found in tropical countries. Most infections are subclinical but may progress to chronic form characterized most frequently by the presence of liver involvement and portal hypertension. We report a patient that presented chronic polyarthritis with positive rheumatoid factor. During investigation, increased liver enzymes, negative hepatitis serologies and signs of portal hypertension on an ultrasound examination raised suspicion of S. mansoni infection. We will discuss pathophysiology and clinical manifestations of S. mansoni infection with special attention to articular involvement.

Abnormality in hippocampal signal intensity predicts atrophy in patients with systemic lupus erythematosus

Objectives To quantify signal abnormalities in the hippocampus (Hsig) of patients with systemic lupus erythematosus (SLE) and to determine if Hsig predict hippocampal atrophy (HA) in SLE. Methods We included all SLE patients and healthy age- and sex-matched individuals with two magnetic resonance imaging (MRI) scans performed with a minimum of 1 year interval. All individuals underwent a standardized neuropsychological evaluation. Individual results were converted into standard scores and compared to normative data. SLE patients were additionally assessed for disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)), and the presence of antiphospholipid antibodies. MRI was performed on an Elscint 2 T scanner and T1 inversion recovery and T2 coronal images were used for analysis. Volumetric (HV) and signal quantification (Hsig) were determined by standardized protocols. Results We included 54 SLE patients (48 women; mean age 32.2 ± 10.56 years). Hsig were found at study entry in 15 (45.5%) patients. Hsig in the body and tail of non-atrophic hippocampi correlated with progression of volume loss during the follow-up period (r = 0.8, p < 0.001). The presence of Hsig in the head of atrophic hippocampi correlated with progression of HA (r = 0.73, p = 0.005) during the same period. No correlation of Hsig and disease activity or prednisone dose was observed. Conclusion HA is frequently observed in SLE patients and volume loss is progressive in a subgroup of patients. The evaluation of Hsig is an easy tool to determine patients that may have progressive hippocampal volume loss and should be followed more closely with MRI and cognitive evaluation.

S100β is associated with cognitive impairment in childhood-onset systemic lupus erythematosus patients.

Objective To investigate serologic S100β protein levels in childhood-onset SLE patients (cSLE) and to elucidate their association with disease activity and neuropsychiatric (NP) manifestations. Methods We included 71 cSLE patients (67 females; median age 18 years; range 9–37 and 53 (47 females; median age of 20 years; range 6–29) age and sex matched healthy controls. Neurological manifestations were analysed according to the American College of Rheumatology (ACR) criteria. Cognitive evaluation was performed in all participants using Wechsler Intelligence Scale for Children (WISC-III) and Wechsler Adult Intelligence Scale (WAIS), according to age, and validated in Portuguese. SLE patients were further assessed for clinical and laboratory SLE manifestations, disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and current drug exposures. Sera S100β protein levels were measured by enzyme-linked immunosorbent assay using commercial kits. Results The median S100β protein level was 116.55 pg/mL (range 1.53–468.50) in cSLE and 54.98 pg/mL (range 0.69–181.00) in healthy controls (p < 0.001). An association was observed between S100β protein and NP manifestations (p = 0.03). The S100β protein levels was associated with cognitive impairment in cSLE patients (p = 0.006). Conclusions S100β protein levels are increased in cSLE with cognitive impairment. S100β may be considered a potential biomarker that underlies central nervous system (CNS) dysfunction, especially cognitive impairment.

THU0335 Support vector machines classification of texture parameters of white matter lesions in childhood-onset systemic lupus erythematosus. Possible mechanism to distinguish between demyelination and ischemia

Background Texture analysis (TA) is a branch of image processing which seeks to reduce image information by extracting texture descriptors from the image. White matter hyperintensities (WMH) are frequently observed in childhood-onset systemic lupus erythematosus (cSLE); however the etiology is still unknown. Ischemic and demyelination have been proposed as possible etiologies. Support vector machines (SVM) are a group of supervised learning methods that can be applied to classification or regression Objectives To determine etiology of WMH in cSLE patients using texture analysis of magnetic resonance (MR) parameters basedon neural network Methods TA was applied to axial FLAIR magnetic resonance images (MRI) in 43 patients with cSLE (mean age 17.25 years (SD ± 3.57), 30 patients with multiple sclerosis (MS), 30 patients with stroke and 30 normal age and sex-matched controls. The TA approach used was based on the Gray Level Co-occurrence Matrices (GLCM). The WMH were manually segmented for each subject, classified in periventricular, subcortical, deep white matter and cortical WMH and 256 texture parameters were computed for each lesion. A SVM classifier previously developed and validated (accuracy 93%) was used to classify WMH in patients with cSLE. Nature of the classified WMH, demographic, clinical and laboratory features were included in a regression model to determine which variables could support the possible nature of WMH in clinical practice Results In cSLE, of the 125 of periventricular lesions, 59% were classified as ischemic and 41% as demyelinating. Of the 738 subcortical lesions 63% were classified as ischemic and 37% as demyelinating. Of the 64 cortical lesions, 61% were classified as ischemic and 39% as demyelinating. Of the deep white matter WMH, 72% were classified as ischemic and 28% as demyelinating in nature. In controls all lesions were classified as ischemic. Positive antiphospholipid antibodies (aPL) (OR=3.2; 95%CI 1.2-7.3) and higher total corticosteroid dose (OR=2.1; 95%CI 1.2-3.7) were variables associated with stroke, whereas cutaneous vasculitis (OR=4.2; 95%CI 2.1-7.2), anti-SM antibodies (OR=2.9; 95%CI 1.3-8.7) and disease activity (OR=3.7; 95%CI 1.3-7.8) were associated with demyelination in all cerebral regions. Conclusions In cSLE, the majority of WMH were classified as ischemic in nature; however aproximately 30% of the lesions were demyelinating. Positive aPL and higher total corticosteroid dose were variables associated with stroke, whereas cutaneous vasculitis, anti-SM antibodies and disease activity were associated with demyelination in all cerebral regions.SVM of TA is a useful method to help to determine etiology of WMH in cSLE. Disclosure of Interest A. Lapa Grant/Research support from: FAPESP 2010/13639-1, M. Bento: None Declared, L. Rittner: None Declared, H. Ruocco: None Declared, G. Castellano: None Declared, B. Damasceno: None Declared, L. Costallat: None Declared, R. Lotufo: None Declared, F. Cendes: None Declared, S. Appenzeller Grant/Research support from: FAPESP 2008/02917-0; Conselho Nacional Pesquisa Desenvolvimento-Brasil CNPq (300447/2009-4)

Watershed-based segmentation of the corpus callosum in diffusion MRI

The corpus callosum (CC) is one of the most important white matter structures of the brain, interconnecting the two cerebral hemispheres, and is related to several neurodegenerative diseases. Since segmentation is usually the first step for studies in this structure, and manual volumetric segmentation is a very time-consuming task, it is important to have a robust automatic method for CC segmentation. We propose here an approach for fully automatic 3D segmentation of the CC in the magnetic resonance diffusion tensor images. The method uses the watershed transform and is performed on the fractional anisotropy (FA) map weighted by the projection of the principal eigenvector in the left-right direction. The section of the CC in the midsagittal slice is used as seed for the volumetric segmentation. Experiments with real diffusion MRI data showed that the proposed method is able to quickly segment the CC without any user intervention, with great results when compared to manual segmentation. Since it is simple, fast and does not require parameter settings, the proposed method is well suited for clinical applications.