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SLE encephalopathy

Lei Zhang, Jian Xu
Posterior reversible encephalopathy syndrome (PRES) is a rare clinical entity associated with systemic lupus erythematosus which characterized by seizure, headache, and altered mental status. The pathophysiology involves subcortical vasogenic edema secondary to hypertension and endothelial damage. PRES is reversible with withdrawal of the offending agent, strict blood pressure control, and treating the underlying disease. We report present here a patient with lupus nephritis who developed PRES following mycophenolate administration...
2018: American Journal of Clinical and Experimental Immunology
Yong-Jun Mei, Peng Wang, Chao Jiang, Tao Wang, Lin-Jie Chen, Zhi-Jun Li, Hai-Feng Pan
The purpose of this study is to investigate the clinical and serological associations of anti-ribosomal P0 protein antibodies (anti-Rib-P0) in patients with systemic lupus erythematosus (SLE). The sera of 470 patients with SLE and 124 patients with primary Sjogren's Syndrome (pSS) were collected. Line immunoassay (LIA) was used to detect anti-Rib-P0 and other related antibodies. A complete laboratory evaluation and clinical examination were also performed in each SLE patient. The prevalence of anti-Rib-P0 in SLE patients was significantly higher than that in pSS patients (35...
November 18, 2017: Clinical Rheumatology
Usanee Damrongpipatkul, Kanokporn Oranratanachai, Nuntana Kasitanon, Salita Wuttiplakorn, Worawit Louthrenoo
Posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE) has been recognized increasingly. This study aimed to determine the prevalence, clinical features, brain imaging findings, outcomes, and associated factors of PRES in Thai SLE patients. SLE patients with PRES were identified from the lupus cohort of Chiang Mai University. Controls were SLE patients with a hospital number close to and actually had SLE diagnosis within 5 years of the case (case:control ratio = 1:4)...
November 4, 2017: Clinical Rheumatology
Dominika D Raciborska, Alastair John Noyce, Dev Pyne, Benjamin P Turner
Although neurological manifestations of systemic lupus erythematosus (SLE) are well recognised, myelopathy complicating SLE is rare. A 35-year-old woman presented with non-specific symptoms and a respiratory tract infection but had serological evidence of SLE. She subsequently deteriorated rapidly, developing a catastrophic spinal cord syndrome. Her initial MRI was normal; but after 1 month, her encephalopathy having progressed, repeat imaging showed characteristic myelitic changes. She responded only slowly to a combination of cyclophosphamide and corticosteroids...
November 2, 2017: Practical Neurology
Tatjana Zekić, Mirjana Stanić Benić, Ronald Antulov, Igor Antončić, Srđan Novak
The cyclophosphamide as a predisposing factor for Posterior Reversible Encephalopathy Syndrome (PRES) and therapeutic option for systemic lupus erythematosus (SLE) is still confusing. The first and only case of PRES, probably induced by cyclophosphamide, in Croatia followed by the findings of 36 SLE patients diagnosed with PRES after treatment with cyclophosphamide worldwide are described. An 18-year-old Caucasian female patient with a 1-year history of SLE was admitted to the hospital due to lupus nephritis and acute arthritis...
December 2017: Rheumatology International
M T L Nyo, D Magazi, M M T M Ally
We report a young woman with systemic lupus erythematosus (SLE) and an acute cerebellar ataxia. A history of poor appetite and vomiting preceded the inco-ordination. Ataxia in SLE has been well described, but is nevertheless uncommon. The clinical triad of mild confusion, ataxia and ophthalmoplegia also raised the possibility of Wernicke's encephalopathy (WE). The diagnosis of WE was further supported by the magnetic resonance imaging features. Owing to overlapping causal factors, this case illustrates the complexity of diagnosing and managing neuropsychiatric syndromes in a patient with SLE...
March 29, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
Kashif Jafri, Hengameh Zahed, Katherine D Wysham, Sarah Patterson, Amber L Nolan, Matthew D Bucknor, R Krishna Chaganti
Weakness, seizures, and encephalopathy have a broad differential diagnosis in patients with systemic lupus erythematosus (SLE). We present a case of a 26-year-old female with a recent diagnosis of SLE who experienced a clinical deterioration with quadriparesis, seizures, and encephalopathy. Her quadriparesis was found to be secondary to biopsy-proven hydroxychloroquine-induced myopathy with concomitant inflammatory myopathy. Her seizures and encephalopathy were suspected to be multifactorial in the setting of sepsis and critical illness with possible contributions from neuropsychiatric manifestations of SLE and macrophage activation syndrome...
June 2017: Clinical Rheumatology
Rose M Ayoob, Andrew L Schwaderer
The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed...
2016: International Journal of Nephrology
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
A Castro, J C Romeu, R Geraldes, J A Pereira da Silva
Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET).Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies...
January 2017: Lupus
S Appenzeller, F Reis, L T Lavras Costallat, J Adams Pérez, C F Saldanha, O A Monticielo
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that involves many organs and systems. Nervous system involvement in SLE encompasses neurological and psychiatric disorders, and remains a diagnostic and therapeutic challenge. Wernicke's encephalopathy (WE) is a neurological disorder that occurs as a consequence of thiamine deficiency, and its clinical presentation resembles the neuropsychiatric events attributed to SLE (NPSLE). Differentiation between these two entities is crucial because their treatment differs greatly and can change prognosis...
July 14, 2016: Lupus
V Dvorakova, H Kolarova, M Magner, M Tesarova, H Hansikova, J Zeman, T Honzik
BACKGROUND: Mitochondrial myopathy, Encephalopathy, Lactic Acidosis and Stroke-like episodes syndrome (MELAS) is a common mitochondrial disorder with varying multisystemic clinical manifestation. We present a comprehensive clinical picture of 50 Czech m.3243A>G carriers with emphasis on the sequence of symptoms in symptomatic patients. RESULTS: Symptoms developed in 33 patients (66%) and 17 carriers remained unaffected (34%). The age of onset varied from 1month to 47years of age, with juvenile presentation occurring in 53% of patients...
August 2016: Molecular Genetics and Metabolism
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
Amina Nasri, Ines Bedoui, Ridha Mrissa, Anis Riahi, Hager Derbali, Jamel Zaouali, Meriem Messelmani, Malek Mansour
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. Systemic lupus erythematosus (SLE), beginning in 15-20% in childhood, is considered as a potential underlying etiology of PRES. In children, status epilepticus (SE) rarely complicates PRES, and exceptionally occurs in SLE. METHODS: We report on an illustrative case of PRES complicating pediatric lupus revealed by recurrent SE, and we further review through a Pubmed search the previously reported cases of pediatric SLE, PRES and SE...
October 2016: Brain & Development
S Hima Mani, S M Shivaprasad, L Umesh
Systemic lupus erythematosus (SLE) is associated with various neurologic or psychiatric abnormalities and Posterior Reversible Leuco Encephalopathy Syndrome (PRES) is very rare neurological manifestation in SLE. PRES is associated with various clinical manifestations, like, seizures, visual loss, headaches, vomiting altered mental status and rarely focal neurological deficits. Other predisposing condition associated with PRES is eclampsia, accelerated hypertension, uraemia, transplantation, autoimmune diseases and/or use of immunosuppressive drugs...
January 2016: Journal of Clinical and Diagnostic Research: JCDR
Mohamed A El-Naggari, Dana Al-Nabhani, Ibtisam El-Nour, Alaa El-Manzalawy, Anas-Alwogud A Abdelmogheth
Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a combination of clinical and radiological features. Clinical symptoms include headaches, confusion, seizures, disturbed vision or an altered level of consciousness. Classic magnetic resonance imaging (MRI) findings indicate subcortical and cortical oedema, affecting mainly the posterior cerebral region. We report two paediatric cases of PRES with underlying renal diseases presenting at the Sultan Qaboos University Hospital in Muscat, Oman, in April 2010 and August 2011...
August 2015: Sultan Qaboos University Medical Journal
Amritha Budhoo, Girish M Mody
Our aim was to compare our South African cohort of systemic lupus erythematosus (SLE) and posterior reversible encephalopathy syndrome (PRES) with other published series. We reviewed the records of 10 patients with SLE and PRES seen over a 10-year period and their demographic data, clinical manifestations, laboratory tests, imaging findings, and outcome were recorded. We identified 10 females who included six Indians, three mixed ethnicity, and one African Black. Three patients had PRES at the onset of SLE...
December 2015: Clinical Rheumatology
Gustavo R Marín
Posterior reversible encephalopathy is a rare disease in children. Clinical manifestations include headache, seizures, visual disturbances and altered consciousness associated with typical magnetic resonance images of the nervous system. The syndrome usually manifests in patients with eclampsia, solid organ transplantation, haematologic, renal and autoimmune diseases among other less common causes and it is often triggered after a hypertensive crisis or use of immunosuppressive drugs. Less common pathogenic factors as blood transfusion, use of immunoglobulins or an underlying infection can be associated...
October 2015: Archivos Argentinos de Pediatría
Wu Yun, Zhang Weihua, Ren Xiaotun, Li Jiuwei, Yang Xinying, Lyu Junlan, Ding Changhong, Chen Chunhong, Ren Haitao, Cui Liying, Fang Fang
OBJECTIVE: To analyze the clinical characteristics of pediatric neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD). METHOD: A retrospective analysis was performed evaluating clinical and laboratory characteristics of ten NMO and NMOSD children who were seen in our hospital from December 2010 to May 2014. Median age at onset was 8.9 years (range 0.8-13.8 years). Seven cases were female and three were male. Median disease duration was 1...
April 2015: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
M Fredi, M Bianchi, L Andreoli, G Greco, I Olivieri, S Orcesi, E Fazzi, C Cereda, A Tincani
An impaired expression of interferon-α regulated genes has been reported in patients with either systemic lupus erythematosus (SLE) or Aicardi-Goutières syndrome (AGS), a rare monogenic encephalopathy with onset in infancy. One of mutations causing AGS is located in the TREX1 gene on chromosome 3. Heterozygous mutations in TREX1 were reported in SLE patients. TREX1 is a DNA exonuclease with specificity for ssDNA. An impairment of its activity may result in the accumulation of nucleid acid. A recent study described a significant association between a haplotype including several common single nucleotide polymorphisms (SNPs) of TREX1 and neurological manifestations in European SLE patients...
June 30, 2015: Reumatismo
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