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A study on clinical and radiological features and outcome in patients with posterior reversible encephalopathy syndrome (PRES)

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Abstract

Posterior reversible encephalopathy syndrome (PRES) is characterized clinically by headaches, seizures, vomiting, nausea, visual abnormalities, and altered mental function and is often (but not invariably) accompanied by parieto-occipital imaging features. The aim of this study is to describe the clinical and radiological features and outcome following PRES in a paediatric cohort. From a retrospectively identified cohort, case records were studied to confirm a diagnosis of PRES. Neuroimaging was reviewed again to assign to recently described radiological subtypes parieto-occipital pattern, holohemispheric watershed pattern, dominant superior frontal sulcus pattern, and asymmetrical or partial expression of the three primary patterns (A/P). Patient outcome was measured by the modified Rankin scale (mRS) scores. Nine boys and three girls with mean age of 12 were identified. Hypertensive episodes (n = 11), tacrolimus toxicity (n = 4), and autoimmunity (n = 1) were identified as potential risk factors/etiologies. Their median mRS at the peak of illness was 2 (range 2–5); three children required intensive care support. After mean follow-up of 35 months (median 37 months; range 3–60 months), all patients improved significantly with mean mRS of 1 (median 1; range 0–1). Conclusion: PRES is easily recognizable by the clinical and radiological features. Although severe at presentation, the outcome from this condition is favorable.

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Abbreviations

AI:

Autoimmune

A/P:

Atypical or partial expression

CNI:

Calcineurin Inhibitors

CKD:

Chronic kidney disease

DWI:

Diffusion-weighted imaging

EEG:

Electroencephalogram

ESRF:

End stage renal failure

FLAIR:

Fluid-attenuated inversion recovery

HSP:

Henoch-Scholein purpura

HbSS:

Sickle cell anemia

HH:

Holohemispheric

HT:

Hypertension

MRI:

Magnetic resonance imaging

mRS:

Modified Rankin scale

Non T:

Non-transplant

PO:

Parieto-occipital

PRES:

Posterior reversible encephalopathy syndrome

SFS:

Superior frontal sulcus

SLE:

Systemic lupus erythematosus

TT:

Tacrolimus toxicity

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Acknowledgments

The authors MDS and ML acknowledges financial support from the Department of Health via the National Institute for Health Research (NIHR) comprehensive Biomedical Research Centre award to Guy’s and St Thomas’ NHS Foundation Trust in partnership with King’s College London and King’s College Hospital NHS Foundation Trust.

Disclosure

ML receives research grants from Action Medical Research and MS Society, receives research support grants from the London Clinical Research Network and Evelina Appeal, has received consultation fees from CSL Behring, received travel grants from Merck Serono, and awarded educational grants to organize meetings by Novartis, Biogen Idec, Merck Serono, and Bayer.

Author information

Authors and Affiliations

  1. Children’s Neurosciences, Evelina Children’s Hospital at Guys and St Thomas’ NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK

    Rahul R. Singh & Ming Lim

  2. Department of Paediatric Nephrology, Evelina Children’s Hospital at Guys and St Thomas’ NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK

    Nurdan Ozyilmaz, Simon Waller & Manish D. Sinha

  3. Department of Neuroradiology, Evelina Children’s Hospital at Guys and St Thomas’ NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK

    Jean-Marie U-King-Im & Ata Siddiqui

Authors
  1. Rahul R. Singh
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  2. Nurdan Ozyilmaz
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  3. Simon Waller
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  4. Jean-Marie U-King-Im
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  5. Ming Lim
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  6. Ata Siddiqui
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  7. Manish D. Sinha
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Corresponding authors

Correspondence to Rahul R. Singh or Manish D. Sinha.

Additional information

Communicated by Peter de Winter

PRES in children

NO MS helped collecting data and preparing initial draft. NO, SW, and MS reviewed the data and helped in preparing the initial draft, and MS helped in subsequent reviews. AS and JU reviewed all the images independently. AS, JU, RS, and ML reviewed all the images to reach to a consensus. ML, MS, and RS assigned mRS scoring and in preparing the final draft and submission.

What is known about this condition and what is new?

PRES is a reversible condition, and there has been no report of quantitative measurement of outcome in this group of children at the time of illness and on complete recovery. We report that irrespective of the lesion load and distribution of lesion, the outcome on modified Rankin scale (mRS) is good.

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Singh, R.R., Ozyilmaz, N., Waller, S. et al. A study on clinical and radiological features and outcome in patients with posterior reversible encephalopathy syndrome (PRES). Eur J Pediatr 173, 1225–1231 (2014). https://doi.org/10.1007/s00431-014-2301-y

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  • DOI: https://doi.org/10.1007/s00431-014-2301-y

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