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CNS lupus

M Aringer, M Schneider
In the last few decades a number of small, often largely unrecognized steps have fundamentally changed the management of systemic lupus erythematosus (SLE). The current goal is to stop all disease activity without long-term use of more than 5 mg prednisolone per day. Remission, i.e. absence of activity in the SLE activity score of choice, is the defined target in the treat to target approach. The essential basic measures include life-long hydroxychloroquine as well as protection from sunlight (UV) and vitamin D substitution...
September 26, 2016: Der Internist
Vikram A Londhey
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder of unknown aetiology which can present with myriad clinical presentation. The neurological manifestations of SLE consist of central nervous system (CNS) and peripheral nervous system manifestations (PNS). The CNS manifestations are aseptic meningitis, cerebrovascular accidents (stroke), demyelinating disorders, headache, involuntary movements like chorea, myelopathy, acute confusional states, cognitive dysfunction, mood disorder, seizures, psychosis and cranial nerve palsies...
December 2015: Journal of the Association of Physicians of India
Vivek Joseph, Rahul Anil, Sary Aristy
A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures...
October 2016: Journal of Clinical Medicine Research
L N Moorthy, M E Baldino, V Kurra, D Puwar, A Llanos, M G E Peterson, A L Hassett, T J A Lehman
Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits...
August 5, 2016: Lupus
David A Geier, Mark R Geier
Gardasil is a quadrivalent human papillomavirus (HPV4) vaccine that was approved for use by the US Food and Drug Administration in June 2006. HPV4 vaccine is routinely recommended for administration to women in the USA who are 11-12 years old by the Advisory Committee on Immunization Practices. Previous studies suggest HPV4 vaccine administration was associated with autoimmune diseases. As a consequence, an epidemiological assessment of the vaccine adverse event reporting system database was undertaken for adverse event reports associated with vaccines administered from 2006 to 2014 to 6-39 year-old recipients with a listed US residence and a specified female gender...
July 13, 2016: Immunologic Research
M Roriz, A Lopes, I Jarrin, S Lechtman, M Polivka, J-F Bergmann, D Sène, S Mouly
INTRODUCTION: We report an unusual observation of central nervous system (CNS) lymphoma in a 60-year-old woman with systemic lupus erythematosus and fatal outcome. OBSERVATION: The patient had systemic erythematosus lupus for 7 years, treated with mycophenolate mofetil and developed lymphocytic meningitis in 2015 associated to the presence of EBV in the cerebrospinal fluid and a necrotic vermis' lesion. Diagnosis of large B-cell lymphoma was histologically confirmed from stereotaxic biopsy, shortly before she died from neurological complications...
June 27, 2016: La Revue de Médecine Interne
Maria Luisa Fiorella, Matteo Gelardi, Andrea Marzullo, Elena Sabattini, Raffaele Fiorella
Kikuchi-Fujimoto's disease (KFD) is a rare, benign condition characterized by histiocytic necrotizing lymphadenitis predominantly in the cervical region and prolonged fever. It has a higher prevalence in the Japanese and Asian populations although it may be seen in a wide geographic distribution, but rare are the cases reported in Europe. While symptoms resolve spontaneously between 2 weeks and 2 months, complications are described in: CNS, eyes, respiratory tract, kidney and liver. KFD also described an association with systemic lupus erythematosus...
June 17, 2016: European Archives of Oto-rhino-laryngology
Ananya Mahapatra, Pawan Sharma, Rajesh Sagar
Systemic Lupus Erythematosus (SLE) is a prototypic autoimmune disease of unknown etiology, which affects multiple organ systems including the central nervous system (CNS). Though not common, childhood onset SLE is a known and established entity. Neuropsychiatric symptoms are common in childhood onset SLE. Of these, psychosis and behavioural symptoms are relatively rare, and there is no consensus on the proper treatment of such cases. We report a case of 13-year-old boy, diagnosed with lupus nephritis, and presented with psychosis and behavioural symptoms...
2016: Journal of the Canadian Academy of Child and Adolescent Psychiatry
Supriya D Mahajan, Vincent M Tutino, Yonas Redae, Hui Meng, Adnan Siddiqui, Trent M Woodruff, James N Jarvis, Teresa Hennon, Stanley Schwartz, Richard J Quigg, Jessy J Alexander
Blood-brain barrier (BBB) dysfunction complicates central nervous system lupus, an important aspect of systemic lupus erythematosus. To gain insight into the underlying mechanism, vascular corrosion casts of brain were generated from the lupus mouse model, MRL/lpr mice and the MRL/MpJ congenic controls. Scanning electron microscopy of the casts showed loss of vascular endothelial cells in lupus mice compared with controls. Immunostaining revealed a significant increase in caspase 3 expression in the brain vascular endothelial cells, which suggests that apoptosis could be an important mechanism causing cell loss, and thereby loss of BBB integrity...
August 2016: Immunology
Debasish Chaudhury, Arshna Qureshi, Shiv Prasad, Ravi Kumar Meena, Simran Sharma
A 24-year-old female presented with catatonia and symptoms suggestive of Depressive Disorder. She also gave history of undocumented low grade irregular fever. The patient was worked up to rule out any organic cause or psychiatric illness. However, further investigations revealed immunological profile diagnostic of Systemic Lupus Erythematosus (SLE) with CNS involvement (CNS lupus). The diagnosis of SLE in this patient presenting with catatonia was of practical importance because catatonia as one of the manifestations of SLE or as a standalone presenting symptom is extremely rare...
May 9, 2016: Reumatología Clinica
Joseph Wai-Hin Leung, Benson Wui-Man Lau, Vera Sau-Fong Chan, Chak-Sing Lau, Kwok-Fai So
PURPOSE: Systemic Lupus Erythematosus (SLE) is an autoimmune disease which is characterised by elevated levels of autoantibodies and cytokines in the body. Via alteration of the regulation of inflammation, damage to different organ systems, including the central nervous system (CNS), was found in SLE patients. Patients diagnosed with SLE were reported to suffer from different kinds of psychiatric signs and symptoms. As neurogenesis has been suggested to be a potential key player of psychiatric symptoms and emotional behavior disturbances, this study aims to investigate whether neurogenesis is altered in an animal model of SLE...
May 2, 2016: Restorative Neurology and Neuroscience
Axel Petzold, Sui Wong, Gordon T Plant
There are a number of autoimmune disorders which can affect visual function. There are a very large number of mechanisms in the visual pathway which could potentially be the targets of autoimmune attack. In practice it is the retina and the anterior visual pathway (optic nerve and chiasm) that are recognised as being affected in autoimmune disorders. Multiple Sclerosis is one of the commonest causes of visual loss in young adults because of the frequency of attacks of optic neuritis in that condition, however the basis of the inflammation in Multiple Sclerosis and the confirmation of autoimmunity is lacking...
2016: Handbook of Clinical Neurology
Shigeru Aoki, Natsuko Kobayashi, Aya Mochimaru, Tsuneo Takahashi, Fumiki Hirahara
A sudden flare of previously stable SLE may give rise to CNS lupus. During pregnancy, seizures associated with CNS lupus can cause hypoxic-ischemic encephalopathy (HIE) in the infant.
April 2016: Clinical Case Reports
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
Jing-Jing Wang, Jian-Hua Mao
BACKGROUND: Congenital nephrotic syndrome (CNS), defined as heavy proteinuria, hypoalbuminemia, hyperlipidemia and edema presenting in the first 0-3 months of life, may be caused by congenital syphilis, toxoplasmosis, or congenital viral infections (such as cytomegalovirus). However, the majority of CNS cases are caused by monogenic defects of structural proteins that form the glomerular filtration barrier in the kidneys. Since 1998, an increasing number of genetic defects have been identified for their involvements in the pathogenesis of CNS, including NPHS1, NPHS2, WT1, PLCE1, and LAMB2...
May 2016: World Journal of Pediatrics: WJP
César Magro-Checa, Ece Ercan, Ron Wolterbeek, Bart Emmer, Nic J van der Wee, Huub A Middelkoop, Nyika D Kruyt, Itamar Ronen, Mark A van Buchem, Tom W Huizinga, Gerda M Steup-Beekman
OBJECTIVE: To assess white matter (WM) and gray matter (GM) magnetization transfer ratio histogram peak heights (MTR-HPHs) in different subsets of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) who have unremarkable findings on 3T magnetic resonance imaging of the brain and to evaluate whether these values could be used to highlight different clinically suspected underlying pathogenic processes or identify the clinical NPSLE status or whether they could be associated with a specific NPSLE syndrome...
August 2016: Arthritis & Rheumatology
Jiri Vymetal, Martina Skacelova, Andrea Smrzova, Anna Klicova, Marketa Schubertova, Pavel Horak, Josef Zadrazil
BACKGROUND AND AIM: Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides...
March 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
Kit Wu, Loucas Christodoulou, Ata Siddiqui, David D'Cruz, Thomasin Andrews
We report a 40-year-old woman with systemic lupus erythematosus (SLE) and associated inflammatory polyarthritis who presented with acute facial dystonic spasms. Her speech was also affected. An MRI brain showed bilateral symmetrical basal ganglia signal change on T2. This movement disorder was due to an acute manifestation of her lupus. Her symptoms resolved rapidly following treatment with (oral) steroids. Repeat MRI brain at 1 month showed complete resolution of the basal ganglia signal change. This is the first time that facial spasms and dystonia with corresponding MRI changes are reported as a presentation of lupus affecting the central nervous system (CNS lupus)...
December 2015: Quantitative Imaging in Medicine and Surgery
Jing Wen, Christopher Holden Chen, Ariel Stock, Jessica Doerner, Maria Gulinello, Chaim Putterman
Fn14, the sole known signaling receptor for the TNF family member TWEAK, is inducibly expressed in the central nervous system (CNS) in endothelial cells, astrocytes, microglia, and neurons. There is increasing recognition of the importance of the TWEAK/Fn14 pathway in autoimmune neurologic conditions, including experimental autoimmune encephalomyelitis and neuropsychiatric lupus. Previously, we had found that Fn14 knockout lupus-prone MRL/lpr mice display significantly attenuated neuropsychiatric manifestations...
May 2016: Brain, Behavior, and Immunity
S Jiang, T-C Lei, S-Z Xu
Patients with systemic lupus erythematosus (SLE) have an increased susceptibility to bacterial, viral, fungal and parasitic infections. Cryptococcal infection of the central nervous system (CNS) is a rare but often fatal complication of SLE. Here we describe a case of cryptococcal meningitis in a female patient with active SLE, who was successfully treated with amphotericin B. This case suggests that the clinical findings of SLE patients with cryptococcal meningitis are non-specific and misleading, and early use of amphotericin B has a good response...
December 1, 2015: West Indian Medical Journal
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