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cerebral lupus

Abhinav Anand, Kruthi Malur, Juhi Kawale, Milind Y Nadkar
We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. 38 years old female who had earlier polyserositis and cerebral venous thrombosis was admitted with provisional diagnosis of SLE and developed acute abdominal pain during hospital stay. She was diagnosed as mesenteric vasculitis and initially responded to pulse methylprednisolone. However, she had relapse which subsequently responded to pulse cyclophosphamide and steroids...
July 2016: Journal of the Association of Physicians of India
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count...
October 12, 2016: Journal des Maladies Vasculaires
Stewart J Wiseman, Mark E Bastin, Charlotte L Jardine, Gayle Barclay, Iona F Hamilton, Elaine Sandeman, David Hunt, E Nicole Amft, Susan Thomson, Jill F F Belch, Stuart H Ralston, Joanna M Wardlaw
BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE) increases stroke risk, but the mechanism is uncertain. This study aimed to determine the association between SLE and features on neuroimaging of cerebral small vessel disease (SVD), a risk factor for stroke. METHODS: Consecutive patients attending a clinic for SLE were recruited. All patients underwent brain magnetic resonance imaging; had blood samples taken for markers of inflammation, endothelial dysfunction, cholesterol, and autoantibodies; and underwent cognitive and psychiatric testing...
October 4, 2016: Stroke; a Journal of Cerebral Circulation
Fei Han, Ding-Rong Zhong, Hong-Lin Hao, Wei-Ze Kong, Yi-Cheng Zhu, Hong-Zhi Guan, Li-Ying Cui
BACKGROUND: Hypertrophic pachymeningitis (HP) is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. It can be divided into cranial and spinal forms depending on the location of the lesion. HP involving 2 separate sites simultaneously is quite uncommon. CASE SUMMARY: This study presents a case of a 49-year-old woman with pathologically confirmed cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus (SLE), which is a rare etiology of HP...
September 2016: Medicine (Baltimore)
Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement...
September 9, 2016: La Presse Médicale
Yong Jae Ko, Yang Gyun Lee, Ji Woong Park, Sung Ho Ahn, Jin Myoung Kwak, Yoon-Hee Choi
Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy...
August 2016: Annals of Rehabilitation Medicine
Olcay Ünver, Gazanfer Ekinci, Büşra Işın Kutlubay, Thomas Gülten, Sağer Güneş, Nilüfer Eldeş Hacıfazlıoğlu, Dilşad Türkdoğan
AIM: We aimed to evaluate the patients who were followed up in our clinic with a diagnosis of cerebral sinovenous thrombosis in terms of age, sex, clinical findings, etiology, thrombophilic factors, imaging findings, treatment and prognosis. MATERIAL AND METHODS: The files of 11 patients who were followed up in our pediatric neurology clinic with a diagnosis of cerebral thrombosis between 1 December 2010 and 31 December 2014 were retrospectively analyzed. RESULTS: Seven of 11 patients were male (63...
June 2016: Türk Pediatri Arşivi
Jinxian Huang, Guoxiang Song, Zhihua Yin, Weizhen He, Lijun Zhang, Weihong Kong, Zhizhong Ye
This was an exploratory analysis comparing the safety and efficacy of immunoadsorption (IAS) combination therapy in severe systemic lupus erythematosus (SLE) receiving corticosteroid pulse and immunosuppressant treatment. Patients enrolled all had predominant organ involvement including proteinuria, thrombocytopenia, pericardial effusion, and cerebral involvement requiring corticosteroid pulse treatment. Fifty-two patients in study group received IAS plus corticosteroid and cyclophosphamide treatment. Fifty-two patients in non-IAS group received corticosteroid and cyclophosphamide treatment...
September 2016: Clinical Rheumatology
Satoru Teshigawara, Yoshinori Katada, Yuichi Maeda, Maiko Yoshimura, Eriko Kudo-Tanaka, Soichiro Tsuji, Yoshinori Harada, Masato Matsushita, Shiro Ohshima, Kotaro Watanabe, Takahiro Kumode, Yoshihiko Hoshida, Yukihiko Saeki
BACKGROUND: Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first case of hemophagocytic lymphohistiocytosis complicated by leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma...
2016: Journal of Medical Case Reports
Ji Young Lee, Young Seo Kim, Hyun Young Kim, Dong Woo Park, Sang-Cheol Bae, Young-Jun Lee
We describe two cases of non-aneurysmal subarachnoid hemorrhage (SAH) and multifocal stenosis of the intracranial arteries. The patients' histories together with magnetic resonance angiography, vessel wall imaging and transcranial Doppler (TCD) indicated that the SAH was due to vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Differential diagnosis of vasculitis and RCVS is important because the treatment strategies are different: immunosuppressants in vasculitis and calcium channel blockers in RCVS...
July 25, 2016: International Journal of Rheumatic Diseases
M Galli, G Finazzi, T Barbui
The Antiphospholipid Syndrome (APS) is defined by the association between antiphospholipid antibodies, i.e. anticardiolipin (aCL) and/or lupus anticoagulant (LA) antibodies, and one or more of the following clinical manifestations: arterial and venous thrombosis, recurrent abortions and thrombocytopenia (1). Among them, deep venous thromboses, pulmonary embolism and thrombosis of the cerebral arteries are the most frequent events, occurring in approximately 1/3 of the patients. Thrombosis of the placental vessels (2) is considered the cause of the obstetrical complications (recurrent spontaneous abortions, fetal deaths or fetal growth retardation) suffered by approximately 10% of the women with antiphospholipid antibodies (1), whereas a variable degree of thrombocytopenia is reported by about 20-25% of the patients (3)...
1996: Hematology (Amsterdam, Netherlands)
Hui Wang, Jason S Knight, Jeffrey B Hodgin, Jintao Wang, Chiao Guo, Kyle Kleiman, Daniel T Eitzman
Systemic lupus erythematosus (SLE) is associated with an elevated risk of vascular complications, including premature stroke. Therapies targeting leukocyte recruitment may be beneficial in reducing vascular complications associated with SLE. Lupus was induced in female wild-type (WT) and P-selectin glycoprotein ligand-1 deficient (Psgl-1(-/-)) mice with pristane. Stroke was induced following photochemical injury to the middle cerebral artery (MCA). Stroke size was increased in pristane-treated WT mice compared to non-pristane-treated WT controls...
2016: Scientific Reports
Y Yao, J B Wang, M M Xin, H Li, B Liu, L L Wang, L Q Wang, L Zhao
To investigate the cytokine profile in serum and cerebrospinal fluid (CSF) from patients with systemic lupus erythematosus (SLE) and central nervous system infection, we measured interferon-g (IFN-g), interleukin-1b (IL-1b), IL-4, IL-6, IL-8, IL-10, and IL-17 levels in serum and CSF from 50 SLE patients and 38 matched controls. In patients with active compared to quiescent disease, serum levels were higher for IL-1b (P = 0.042) and IL-17 (P = 0.041) but we found no significant correlation between IL-1b and IL-17 and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) (r = 0...
2016: Genetics and Molecular Research: GMR
Gakuro Abe, Hirotoshi Kikuchi, Yoshiyuki Arinuma, Shunsei Hirohata
OBJECTIVE: The objective of this study is to explore the characteristics of brain MRI abnormalities in acute confusional state (ACS) in neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: Thirty-six patients with ACS admitted to our institutions from 1992 to 2015 were exhaustively enrolled. Their medical charts and brain MRI scans were reviewed. RESULTS: Eighteen of 36 ACS patients had MRI abnormalities, mostly high-intensity lesions of various sizes in the cerebral white matter...
June 20, 2016: Modern Rheumatology
Kibriya Fidan, Yasar Kandur, Murat Ucar, Kivilcim Gucuyener, Oguz Soylemezoglu
Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome, composed of symptoms such as headache, seizures, visual disturbances, lethargy, confusion, stupor, focal neurologic findings and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres. PRES is associated with significant morbidity and mortality if it is not expeditiously recognized. Magnetic resonance image (MRI) represents the most sensitive imaging technique for recognizing PRES...
July 2016: Journal of Clinical Medicine Research
J M Lai, F Q Wu, Z X Zhou, Y C Yan, G X Su, S N Li, J Zhu, M Kang
OBJECTIVE: To investigate the value of brain magnetic resonance imaging (MRI) in evaluating the intracranial injuries in patients with juvenile onset systemic lupus erythematosus (SLE). METHOD: Data of brain MRI, CT, electroencephalogram (EEG), cerebrospinal fluids analysis and clinical features of the central nervous system of 44 patients from March 2007 to March 2015 with juvenile onset SLE who were not treated with glucocorticoids (Gcs) and immunosuppressive agents (Is) were retrospectively analyzed and compared...
June 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Federica Delunardo, Denise Soldati, Veronica Bellisario, Alessandra Berry, Serena Camerini, Marco Crescenzi, Cristiano Alessandri, Fabrizio Conti, Fulvia Ceccarelli, Ada Francia, Guido Valesini, Francesca Cirulli, Alberto Siracusano, Alessandra Siracusano, Cinzia Niolu, Ivo Alex Rubino, Elena Ortona, Paola Margutti
OBJECTIVE: To investigate the potential role of circulating autoantibodies specific to neuronal cell surface antigens in the pathophysiology of neuropsychiatric disorders. METHODS: We performed two different kinds of immunoscreening approaches using sera from patients with schizophrenia to identify autoantigens associated with neuropsychiatric disorders. Then, we tested the presence of autoantibodies specific to the identified autoantigen in patients with various psychiatric disorders and systemic lupus erythematosus (SLE) with neuropsychiatric manifestations...
May 23, 2016: Arthritis & Rheumatology
Julie Toubiana, Satoshi Okada, Julia Hiller, Matias Oleastro, Macarena Lagos Gomez, Juan Carlos Aldave Becerra, Marie Ouachée-Chardin, Fanny Fouyssac, Katta Mohan Girisha, Amos Etzioni, Joris Van Montfrans, Yildiz Camcioglu, Leigh Ann Kerns, Bernd Belohradsky, Stéphane Blanche, Aziz Bousfiha, Carlos Rodriguez-Gallego, Isabelle Meyts, Kai Kisand, Janine Reichenbach, Ellen D Renner, Sergio Rosenzweig, Bodo Grimbacher, Frank L van de Veerdonk, Claudia Traidl-Hoffmann, Capucine Picard, Laszlo Marodi, Tomohiro Morio, Masao Kobayashi, Desa Lilic, Joshua D Milner, Steven Holland, Jean-Laurent Casanova, Anne Puel
Since their discovery in patients with autosomal dominant (AD) chronic mucocutaneous candidiasis (CMC) in 2011, heterozygous STAT1 gain-of-function (GOF) mutations have increasingly been identified worldwide. The clinical spectrum associated with them needed to be delineated. We enrolled 274 patients from 167 kindreds originating from 40 countries from 5 continents. Demographic data, clinical features, immunological parameters, treatment, and outcome were recorded. The median age of the 274 patients was 22 years (range, 1-71 years); 98% of them had CMC, with a median age at onset of 1 year (range, 0-24 years)...
June 23, 2016: Blood
Lauren Koffman, Richard Prayson, E M Manno
BACKGROUND: The neurological manifestations of Systemic Lupus Erythematosus (SLE) are varied and incompletely described. A few case series report a benign idiopathic intracranial hypertension (IIH) related to SLE, which is responsive to immunotherapy. There are limited reports of patients with malignant cerebral edema, and diffuse white matter changes in the absence of central nervous system (CNS) vasculitis. METHODS: Case series from our tertiary care center and review of the relevant literature...
May 15, 2016: Journal of the Neurological Sciences
Akihiko Sasaki, Takuma Mikami, Hitoki Hashiguchi
A 62-year-old female complained of easy fatigue and sore throat and chest computed tomography revealed acute type A aortic dissection with pericardial effusion and chronic type B aortic dissection that was on set in 2012.She had past history of systemic lupus erythematosus (SLE) 23 years ago and received steroid therapy since then. Blood examination showed inflammation reaction and slight disseminated intravascular coagulopathy. We underwent 2 stage operation safely. In 1st stage we performed ascending graft replacement to type A aortic dissection and in 2nd stage we carried out thoracic endovascular arterial replacement to type B aortic dissection...
February 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
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