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SLE encephalopathy

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...
February 24, 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
Wenzhong Cao, Rui Jin
No abstract text is available yet for this article.
April 2015: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
Ziqian Wang, Yanhong Wang, Rongrong Zhu, Xinping Tian, Dong Xu, Qian Wang, Chanyuan Wu, Shangzhu Zhang, Jiuliang Zhao, Yan Zhao, Mengtao Li, Xiaofeng Zeng
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with an increased risk of death compared to general population. Although previous studies showed improvement in survival of SLE, the long-term prognosis has not been elaborated in China.This study aims to integrate the observational studies estimating current long-term survival of Chinese SLE patients and analyze the death-cause situation of SLE in China.The study is a systemic review of English and non-English articles using MEDLINE, EMBASE, CNKI, WANFANG, and SINOMED databases...
May 2015: Medicine (Baltimore)
Hiroshi Mitoma, Keya Adhikari, Daniel Aeschlimann, Partha Chattopadhyay, Marios Hadjivassiliou, Christiane S Hampe, Jérôme Honnorat, Bastien Joubert, Shinji Kakei, Jongho Lee, Mario Manto, Akiko Matsunaga, Hidehiro Mizusawa, Kazunori Nanri, Priya Shanmugarajah, Makoto Yoneda, Nobuhiro Yuki
In the last few years, a lot of publications suggested that disabling cerebellar ataxias may develop through immune-mediated mechanisms. In this consensus paper, we discuss the clinical features of the main described immune-mediated cerebellar ataxias and address their presumed pathogenesis. Immune-mediated cerebellar ataxias include cerebellar ataxia associated with anti-GAD antibodies, the cerebellar type of Hashimoto's encephalopathy, primary autoimmune cerebellar ataxia, gluten ataxia, Miller Fisher syndrome, ataxia associated with systemic lupus erythematosus, and paraneoplastic cerebellar degeneration...
April 2016: Cerebellum
Javier Merayo-Chalico, Elia Apodaca, Ana Barrera-Vargas, Jorge Alcocer-Varela, Iris Colunga-Pedraza, Alejandra González-Patiño, Antonio Arauz, Carlos Abud-Mendoza, Marco Martínez-Martínez, Diana Gómez-Martín
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a well-known but rare complication in patients (<1%) with systemic lupus erythematosus (SLE). However, current epidemiological data are quite scant. The aim of the present study was to describe potentially unrecognised risk factors. PATIENTS AND METHODS: We performed a multicentre, retrospective case-control study in Mexico between 1999 and 2014. We included a total of 168 patients who accounted for 77 episodes of PRES, as follows: SLE/PRES, 43 patients with 48 episodes; SLE without PRES, 96 patients; and PRES without SLE, 29 patients...
March 2016: Journal of Neurology, Neurosurgery, and Psychiatry
Lien Van Eyck, Lien De Somer, Diana Pombal, Simon Bornschein, Glynis Frans, Stéphanie Humblet-Baron, Leen Moens, Francis de Zegher, Xavier Bossuyt, Carine Wouters, Adrian Liston
OBJECTIVE: To identify the underlying genetic defect in a 16-year-old girl with severe early-onset and refractory systemic lupus erythematosus (SLE), IgA deficiency, and mild lower limb spasticity without neuroradiologic manifestations. METHODS: Whole-exome sequencing and extensive immunologic analysis were performed on samples from the index patient. RESULTS: We identified a de novo p.R779H IFIH1 gain-of-function mutation in a patient with severe early-onset SLE, selective IgA deficiency, and mild lower limb spasticity...
June 2015: Arthritis & Rheumatology
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