Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI): A case report

Khokhar, Harsh Vardhan; Choudhary, Pradeep; Saxena, Sangeeta; Arif, Mohamed

doi:10.4103/0972-2327.165456

Cited by 5 publications

(4 citation statements)

References 9 publications

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“…In contrast to the general prevalence of IgAV (male 2:1 female), neurological involvement in pediatric patients was nearly equally contributed, with 13 girls and 13 boys affected, respectively. Two cases of adult males ( 19 , 35 ) (22 and 42 years of age) and one female patient were found ( 36 ). Bérubé et al, however, reported a 1.5:1 preponderance of male patients ( 38 ).…”

Section: Resultsmentioning

confidence: 98%

“…In total, we identified 28 cases with IgAV and reversible encephalopathy ( 6 – 10 , 17 – 36 ). Twenty six cases were associated with seizures ( 6 – 10 , 17 – 19 , 21 – 31 , 33 , 34 , 36 ), and thereof 14 cases fulfilled all clinico-radiological criteria of PRES ( 6 , 7 , 9 , 10 , 17 – 25 ). Lava et al indicated the existence of seven other cases, which, however, were not available in English or did not fulfill the radiologic diagnostic criteria of PRES and were therefore not considered a confirmed PRES diagnosis in this summary ( 37 ).…”

Section: Resultsmentioning

confidence: 99%

“…In total, we identified 28 cases with IgAV and reversible encephalopathy (6)(7)(8)(9)(10)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36). Twenty six cases were associated with seizures (6-10, 17-19, 21-31, 33, 34, 36), and thereof 14 cases fulfilled all clinico-radiological criteria of PRES (6, 7, 9, 10, 17-25).…”

Section: Seizures and Pres In Igavmentioning

confidence: 99%

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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: Resultsmentioning

confidence: 98%

Section: Resultsmentioning

confidence: 99%

Section: Seizures and Pres In Igavmentioning

confidence: 99%

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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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“…Although the parieto-occipital lobes are most commonly involved in PRES (98%), frequencies ranging from 10%-68% have been reported for particular regions of the brain [ 2 ]. Different studies have also proposed the introduction of the term posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) in a case series [ 4 ], with additional cases published elsewhere [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Posterior Reversible Encephalopathy Syndrome (PRES) and the Uncommon Sequela: Mesial Temporal Sclerosis

Mengstu,

Chakko,

Salisbury

et al. 2024

Cureus

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Posterior reversible encephalopathy syndrome is often linked to conditions like hypertension and is characterized by reversible brain edema. The development of mesial temporal sclerosis as a consequence of posterior reversible encephalopathy syndrome is an uncommon clinical outcome. We report a 48-year-old female who initially presented with severe iron deficiency anemia, hypertension, and septic tenosynovitis requiring surgical drainage with subsequent development of posterior reversible encephalopathy syndrome accompanied by endocarditis. Although there was a question of one seizure episode during one of her hospital days, the patient experienced multiple seizure episodes three months after she left the hospital. Subsequent MRI demonstrated atrophy of the left mesial temporal lobe suggesting mesial temporal sclerosis. The temporal development of mesial temporal sclerosis in a patient with posterior reversible encephalopathy syndrome highlights mesial temporal sclerosis as a potential long-term consequence of posterior reversible encephalopathy syndrome, and the need for imaging surveillance in patients diagnosed with posterior reversible encephalopathy syndrome.

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Posterior reversible encephalopathy syndrome in Henoch–Schönlein purpura

et al. 2016

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Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI): A case report

Abstract: Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) is a recently described entity with a handful of cases reported in literature. We describe a case of PRES in setting of Henoch-Schönlein purpura (HSP) with involvement of brain stem and spinal cord.

Cited by 5 publications

References 9 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

Posterior Reversible Encephalopathy Syndrome (PRES) and the Uncommon Sequela: Mesial Temporal Sclerosis

Posterior reversible encephalopathy syndrome in Henoch–Schönlein purpura

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