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Cerebellar Parieto-occipital Posterior Reversible Encephalopathy Syndrome and Cerebral Metamorphopsia Associated with Asymptomatic Atrial Septum Vegetation and Renal Disease: Case Report

Challenging differential diagnosis, Unusual or unexpected effect of treatment, Clinical situation which can not be reproduced for ethical reasons, Rare co-existance of disease or pathology

Arash Ghaffari-Rafi, Anthony C. Netzel, Madeline Prat, Daniel T. Miles

United Kingdom Queen Square Institute of Neurology, University College London, London, United Kingdom

Am J Case Rep 2020; 21:e923441

DOI: 10.12659/AJCR.923441

Available online: 2020-05-28

Published: 2020-06-09


#923441

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a poorly characterized and enigmatic syndrome. Despite consistently presenting with nervous system vasogenic edema, this malady has been associated with variable triggers, neurological symptoms, and natural history.
CASE REPORT: The report presents a 25-year old African American female who presented with altered mental status and bilateral cortical blindness. Neuroimaging identified vasogenic edema in the cerebellum, parietal lobe, and occipital lobe. Her PRES was associated with a hypertensive emergency, renal failure, and an atrial septum vegetation (culture-negative endocarditis). All 3 contributing etiologies were addressed, upon which the patient began to recover. During recovery, the patient experienced cerebral metamorphopsia, visualizing her entire environment in the form of a cartoon. After 2 weeks of treatment she recovered to baseline state of heath, with vasogenic edema resolved on follow-up neuroimaging.
CONCLUSIONS: This case presents a rarely catalogued phenomena during PRES recovery, cerebral metamorphopsia, along with a new potential association (culture negative atrial septum endocarditis). The report also highlights how PRES recovery patients (with cortical blindness) should be explicitly assessed for cerebral metamorphopsia and Charles Bonnet syndrome - which may distress patients. Lastly, the atypical presentation of cerebellar vasogenic edema in our patient validates existing literature that PRES does not have a uniform picture and is not well served by its current name or proposed diagnostic criteria. Therefore, renaming the disorder to reversible vasogenic edema syndrome and derestricting the diagnostic criteria, may prevent clinicians from being discouraged when faced with diagnosing PRES in the face of atypical findings.

Keywords: Endocarditis, Subacute Bacterial, Posterior Leukoencephalopathy Syndrome, Vision Disorders



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