Seizure as an Initial Presentation for Posterior Reversible Encephalopathy Syndrome in Undiagnosed Systemic Lupus Erythematosus and Lupus Nephritis: A Case Report

Hartman, Ethan N; Tuna, Kubra; Monsour, Elio; Komanduri, Karthikram; Tarasiuk-Rusek, Aneta

doi:10.7759/cureus.10195

Cited by 7 publications

(14 citation statements)

References 17 publications

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“…From a pediatric perspective, it is noteworthy that almost all patients (28/29) with neurologic manifestations presented with (25/27) or developed (2/27) diffuse to colicky abdominal pain. Similar cases have been described in young adults for the initial presentation of, e.g., acute intermittent porphyria or systemic lupus erythematosus complicated by PRES ( 52 , 53 ). In all three entities, abdominal pain was one of the first signs of the systemic manifestation of the underlying disease.…”

Section: Discussionsupporting

confidence: 66%

Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

et al. 2021

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Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated.Methods: We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations.Results: We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment.Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.

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Section: Discussionsupporting

confidence: 66%

Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

et al. 2021

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“…The second theory is a reflexe hypopefusion of the brain secondary to endothelial dysfunction and exacerbated by vasoconstriction, spasm and/or auto-immune activation, therefore resulting in vascular hyperpermeability and vasogenic edema. All these conditions may be coinciding, creating a summative or multiplicative effect on the course of the disease [11] .…”

Section: Discussionmentioning

confidence: 99%

Fatal outcome of posterior reversible encephalopathy syndrome (PRES) in a lupus nephropathy patient: A case report

Afilal

Nasri

Bendaoud

et al. 2022

Radiology Case Reports

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“…Acute hypertensive crisis is another common cause of PRES [ 5 ]. Although SLE is one of the rare causes of PRES, both these mechanisms can be responsible in SLE as these patients can have endothelial dysfunction as well as raised blood pressure secondary to lupus nephritis [ 6 ]. PRES is a rare neurological entity in SLE and must be entertained as a differential in patients presenting with neurological signs and symptoms.…”

Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus Induced Central Variant of Posterior Reversible Encephalopathy Syndrome: A Rare Association

Jesrani

Gupta

Arya

et al. 2021

Cureus

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Involvement of the posterior cerebral structures is well established in patients with posterior reversible encephalopathy syndrome (PRES). In striking contrast to this, a subset of patients may have an atypical presentation characterized by the involvement of central structures. This is usually seen in the backdrop of systemic lupus erythematosus (SLE), a rare but known cause of PRES. Timely diagnosis with judicious use of radiological modalities and therapeutic intervention is key for successful recovery; however, the prognosis of PRES in patients with SLE is not very promising. We are describing an extremely rare case of a patient diagnosed with a central variant of PRES with previously undiagnosed SLE.

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Seizure as an Initial Presentation for Posterior Reversible Encephalopathy Syndrome in Undiagnosed Systemic Lupus Erythematosus and Lupus Nephritis: A Case Report

Cited by 7 publications

References 17 publications

Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

Fatal outcome of posterior reversible encephalopathy syndrome (PRES) in a lupus nephropathy patient: A case report

Systemic Lupus Erythematosus Induced Central Variant of Posterior Reversible Encephalopathy Syndrome: A Rare Association

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