Reversible Posterior Leukoencephalopathy in Connective Tissue Diseases

Min, Liliane; Zwerling, Jessica; Ocava, Lenore; Chen, I.-Hweii Amy; Putterman, Chaim

doi:10.1016/j.semarthrit.2006.01.003

Cited by 68 publications

(70 citation statements)

References 18 publications

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“…The exact pathophysiological mechanism of PRES is still unclear [12]. Three hypotheses have been proposed till now, which include 1) cerebral vasoconstriction causing subsequent infarcts in the brain, 2) failure of cerebral auto-regulation with vasogenic edema, and 3) endothelial damage with blood-brain barrier disruption further leading to fluid and protein transudation in the brain [12][13][14]. The distinct imaging patterns in PRES are represented in Table 2 [15].…”

Section: Discussionmentioning

confidence: 99%

Posterior Reversible Encephalopathy Syndrome: Case Report

Sodalagunta¹,

Sudulagunta

Kumbhat

et al. 2016

J Neurol Res

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Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome characterized by headache, seizures, altered mental status, and visual loss, as well as white matter vasogenic edema affecting the posterior occipital and parietal lobes of the brain predominantly. This clinical syndrome is increasingly recognized due to improvement and availability of brain imaging, specifically magnetic resonance imaging (MRI). A 35-year-old female with history of unsafe abortion and massive blood transfusion 10 days ago was brought to the emergency room with three episodes of generalized tonic clonic seizures, urinary incontinence and altered sensorium since 3 hours. Brain MRI showed bilateral occipital, parietal, frontal cortex and subcortical white matter T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities, suggestive of PRES. Patient improved after management with intravenous fluids, antibiotics, antiepileptics and monitoring of blood pressure. If recognized and treated early, the clinical syndrome commonly resolves within a week. PRES can be a major problem in rapid and massive blood transfusion. High index of suspicion and prompt treatment can reduce morbidity and mortality, and pave the path for early recovery.

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Section: Discussionmentioning

confidence: 99%

Posterior Reversible Encephalopathy Syndrome: Case Report

Sodalagunta¹,

Sudulagunta

Kumbhat

et al. 2016

J Neurol Res

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“…It can occur as a result of conditions such as eclampsia, hypertensive encephalopathy, and intense immunosuppression [168]. Patients with SLE and systemic vasculitis s have been reported to present with PRES [169], and a distinction between 2 subtypes of PRES in SLE, inflammatory and hypertensive, has been proposed [170].…”

Section: Posterior Reversible Encephalopathy Syndromementioning

confidence: 99%

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example antitumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

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“…Urine full report revealed proteinuria, 4-6 red cells, few pus cells and granular casts. HaemoReversible posterior leukoencephalopathy syndrome in a patient with systemic lupus erythematosus and lupus nephritis H D Samarasinghe 1 , K P Wanigasooriya 1 , S B Gunatilake 1 Journal of the Ceylon College of Physicians, 2012, 43, 56-57 globin was 8.5 g/dl, with normocromic normocytic red cells and platelet count was 80 x 10 9 /L. She was diagnosed to have an acute SLE flair up with CNS and renal involvement.…”

Section: Case Reportmentioning

confidence: 99%

“…According to a study done in USA during the period from 1967 to 2005, RPLE has been identified in 13 patients with connective tissue disease. In separate case reports, 9 SLE patients, 2 Wegener's granulomatosis (WG) patients, and 1 patient with SLE and systemic sclerosis presented with RPLS 1 . In most cases mentioned above, complete resolution of neurological symptoms occured within 2 weeks of presentation, along with improvement or resolution of imaging abnormalities.…”

Section: Introductionmentioning

confidence: 99%