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Neuropsychiatric lupus

G V Novak, B C Molinari, J C Ferreira, A P Sakamoto, M T Terreri, R M R Pereira, C Saad-Magalhães, N E Aikawa, L M Campos, C A Len, S Appenzeller, V P Ferriani, M F Silva, S K Oliveira, A G Islabão, F R Sztajnbok, L B Paim, C M Barbosa, M C Santos, B E Bica, E G Sena, A J Moraes, A M Rolim, P F Spelling, I M Scheibel, A S Cavalcanti, E N Matos, T C Robazzi, L J Guimarães, F P Santos, C T Silva, E Bonfá, C A Silva
Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months)...
January 1, 2018: Lupus
Mohammad-Amin Khajezadeh, Gholamreza Zamani, Bobak Moazzami, Zahra Nagahi, Mahdie Mousavi-Torshizi, Vahid Ziaee
Objective: Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by multisystem involvement, including the nervous system. In the present study, we aimed to assess neuropsychiatric manifestations in juvenile-onset systemic lupus erythematosus (JSLE) in Iran. Methods: One hundred and forty-six pediatric onset patients with SLE who had registered in our pediatric rheumatology database were evaluated prospectively and cross sectionally within 2013-2015...
2018: Neurology Research International
Maryam Sahebari, Zahra Rezaieyazdi, Mandana Khodashahi, Bita Abbasi, Fazlollah Ayatollahi
Objectives: Systemic lupus erythematosus (SLE) is an autoimmune disease with a wide range of clinical manifestations. Cognitive dysfunction is one of the manifestations that could present prior to the emergence of any other neuropsychiatric involvements in SLE. Cognitive dysfunction is a subtle condition occurring with a high frequency. However, there is no data on the correlation of cognitive dysfunction with central nervous system (CNS) imaging findings, in particular single-photon emission computed tomography scan (SPECT) and functional MRI...
2018: Asia Oceania Journal of Nuclear Medicine & Biology
Yuichi Ishikawa, Tadamichi Kasuya, Junichi Ishikawa, Michio Fujiwara, Yasuhiko Kita
Progressive multifocal leukoencephalopathy (PML) is a central nervous system infection caused by John Cunningham (JC) virus reactivation in an immunocompromised patient. PML has various neurologic symptoms and has very poor prognosis. A 36-year-old man developed transverse myelitis and had a psychiatric disorder at the age of 26. He was diagnosed with systemic lupus erythematosus (SLE) and neuropsychiatric SLE (NPSLE), on the basis of leukopenia and presence of anti-DNA and anti-nuclear antibodies. Treatment with glucocorticoid (GC) was started, and remission was introduced...
2018: Therapeutics and Clinical Risk Management
Magdalena Szmyrka, Anna Pokryszko-Dragan, Krzysztof Słotwiński, Ewa Gruszka, Lucyna Korman, Ryszard Podemski, Piotr Wiland
BACKGROUND: Cognitive impairment (CI) is a frequent problem in lupus patients, regardless of their overt neuropsychiatric (NP) involvement. OBJECTIVES: The aim of our study was to test cognitive abilities in systemic lupus erythematosus (SLE) patients by means of neuropsychological testing and event-related potentials (ERPs), and to search for their cognitive abilities correlations with a wide range of auto-antibodies. MATERIAL AND METHODS: A total of 37 SLE patients were subjected to a battery of neuropsychological tests, recommended by the American College of Rheumatology (ACR), and to ERPs...
July 2, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
H H Kwon, S Y Bang, S Won, Y Park, J H Yi, Y B Joo, H S Lee, S C Bae
Objectives Avascular necrosis (AVN) is one of the most common causes of organ damage in patients with systemic lupus erythematosus (SLE) and often causes serious physical disability. The aims of this study were to investigate clinical risk factors associated with symptomatic AVN and to analyze their synergistic effects in a large SLE cohort in Korea. Methods Patients with SLE were enrolled and followed from 1998 to 2014 in the Hanyang BAE Lupus cohort, and damage was measured annually according to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)...
January 1, 2018: Lupus
A Kalinowska-Lyszczarz, M A Pawlak, A Pietrzak, K Pawlak-Bus, P Leszczynski, M Puszczewicz, W Paprzycki, W Kozubski, S Michalak
Differentiation of systemic lupus erythematosus (SLE) from multiple sclerosis (MS) can be challenging, especially when neuropsychiatric (NP) symptoms are accompanied by white matter lesions in the brain. Given the lack of discriminative power of currently applied tools for their differentiation, there is an unmet need for other measures that can aid in distinguishing between the two autoimmune disorders. In this study we aimed at exploring whether brain atrophy measures could serve as markers differentiating MS and SLE...
January 1, 2018: Lupus
Enrico Maria Zardi, Chiara Giorgi, Domenico Maria Zardi
Neuropsychiatric systemic lupus erythematosus is a diagnostic challenge due to the multifarious neurological and psychiatric manifestations that define it but, when suspected, diagnostic imaging can give a fundamental help. The advancements and variety of neuroimaging techniques allow us to perform more and more accurate evaluations of structure, perfusion and metabolism of the brain and to detect cerebral and spinal lesions. Moreover, vascular districts of the neck and the brain as well as the electrical brain and peripheral muscle activity may be accurately investigated, thus giving us a wide panoramic view...
June 25, 2018: Postgraduate Medicine
Robert Szulawski, Peter J Kourlas, Marc Antonchak
BACKGROUND Systemic lupus erythematosus (SLE) has myriad manifestations that can affect any organ system in the body. Macrophage activation syndrome (MAS) is a disease of uncontrolled lymphocyte and macrophage proliferation and activation, which has various triggers, including autoimmune disorder, viral infection, and malignancy. We report here on MAS as a complication of adult SLE, a rare association in the literature, in a patient with an unknown past medical history. CASE REPORT A 38-year-old male patient presented with severe muscle weakness, diffuse abdominal cramps with vomiting and incontinence of stool, confusion, cough, and sweating increasing in severity for about 1 week...
June 22, 2018: American Journal of Case Reports
John G Hanly, Elizabeth Kozora, Steven Beyea, Julius Birnbaum
The American College of Rheumatology (ACR) case definitions for 19 neuropsychiatric syndromes constitute a comprehensive classification of nervous system events in SLE patients. However, additional strategies are needed to determine whether a neurological syndrome is attributable to SLE versus a competing co-morbidity. Cognitive function is a clinical surrogate of overall brain health with applications in both diagnosis and determination of clinical outcomes. Ischemic and inflammatory mechanisms are both key components of the immunopathogenesis of NPSLE, including abnormalities of the blood-brain-barrier and autoantibody mediated production of pro-inflammatory cytokines...
June 21, 2018: Arthritis & Rheumatology
Yun Zhang, Dongmei Wang, Wei Wei, Xuejun Zeng
BACKGROUND: Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation, particularly in the liver and brain. Systemic lupus erythematosus (SLE) is a multi-system disorder that can manifest in any system. Cases with concomitant WD and SLE, unrelated to treatment with penicillamine, have been rarely reported. CASE PRESENTATION: We report a case of a young woman who had typical neuropsychiatric symptoms and laboratory tests results of WD...
June 15, 2018: BMC Neurology
Jessica Fernandes Vivaldo, Jaqueline Cristina de Amorim, Paulo Rogério Julio, Rodrigo Joel de Oliveira, Simone Appenzeller
Systemic lupus erythematosus (SLE) patients have frequently neuropsychiatric manifestations. From the first description of coma in 1875, a variety of manifestations has been described to occur in SLE. However, the lack of standardization reduced the comparability of published studies. In 1999, the American College of Rheumatology published guidelines to define neuropsychiatric nomenclature in SLE. This was the first step toward uniform diagnostic criteria. Several studies have been published since then applying the ACR criteria and frequencies of different manifestations can now be compared between cohorts...
2018: Frontiers in Medicine
Zbigniew Zdrojewski
Recognizing the antiphospholipid syndrome (APS) in patients with lupus (SLE) can be difficult and therefore underestimated. Detection of antiphospholipid antibodies (aPL) in each patient with SLE should be done as a rule. Introduction of the new classification criteria of this syndrome will certainly improve the recognition of APS in the future. The Sapporo APS classification criteria (1998) were replaced by the Sydney criteria in 2006. Based on the most recent criteria, classification with APS requires one clinical and one laboratory manifestation...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Kazuto Tsukita, Hirofumi Miyake, Takashi Kageyama, Toshihiko Suenaga
A 49-year-old woman was admitted to our hospital with recurrent episodes of paresthesia attacks evolving in 5 to 15 minutes from the left hand to the left leg through the left trunk. Neurological examination revealed cortical sensory disturbance in her left hand. Although contrast-enhanced T1 -weighted MRI findings were unremarkable, contrast-enhanced fluid-attenuated inversion recovery (FLAIR) MRI revealed abnormal leptomeningeal enhancement over the sulcus of the parietal lobe, including the sulcus around the postcentral gyrus...
June 27, 2018: Rinshō Shinkeigaku, Clinical Neurology
Matthew J Blitz, Adiel Fleischer
Pregnancy in women with systemic lupus erythematosus (SLE) is associated with an increased risk of adverse maternal and fetal outcomes. Here, we present a case of severe maternal morbidity in a 23-year-old primigravida with SLE and secondary Sjögren's syndrome who experienced a life-threatening multisystem flare at 17 weeks of gestational age. She presented to the emergency department complaining of cough with hemoptysis and shortness of breath. She developed hypoxic respiratory failure and was admitted to the intensive care unit...
2018: Case Reports in Obstetrics and Gynecology
Shunsei Hirohata, Yuko Sakuma, Yu Matsueda, Yoshiyuki Arinuma, Tamiko Yanagida
OBJECTIVES: The present study was carried out to elucidate the roles of serum autoantibodies in the development of blood-brain barrier (BBB) damages in neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: Paired serum and CSF samples were obtained from 101 SLE patients when they presented active neuropsychiatric manifestations (69 patients with diffuse psychiatric/neuropsychological syndromes [diffuse NPSLE] and 32 patients with neurologic syndromes or peripheral neuropathy [focal NPSLE])...
May 24, 2018: Clinical and Experimental Rheumatology
G A Mahmoud, A A Shahin, H S Zayed, A Moghazy, B M Eissa
Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002-2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90...
January 1, 2018: Lupus
David Dongkyung Kim, Charles Ho, Rebecca King, Sarah A Morrow
No abstract text is available yet for this article.
May 22, 2018: Neurology
Yuya Fujita, Shoichi Fukui, Midori Ishida, Yushiro Endo, Sosuke Tsuji, Ayuko Takatani, Takashi Igawa, Toshimasa Shimizu, Masataka Umeda, Remi Sumiyoshi, Ayako Nishino, Tomohiro Koga, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
A 70-year-old Japanese woman presented to our hospital with gait disturbance and cognitive dysfunction. Since she had arthritis, leukocytopenia, thrombocytopenia, hypocomplementemia, and anti-nuclear and anti-double-stranded DNA antibodies, she was diagnosed with systemic lupus erythematosus (SLE). T2-weighted magnetic resonance imaging revealed bilateral hyperintensities in the putamen. Based on her cognitive impairment, muscle rigidity, and high levels of interleukin-6 in the cerebrospinal fluid, we believed she had developed a complication of a neuropsychiatric disease and administered corticosteroids and intravenous cyclophosphamide therapy...
May 18, 2018: Internal Medicine
Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, Hidenori Takahashi, Saori Abe, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond...
May 18, 2018: Internal Medicine
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