Journal Pre-proofs A PRESing Case of Visual Changes and Confusion Tanisha Ronnie, Larrisa Beyerlein, Michael Murati, Jeffrey P. Louie PII: DOI: Reference:
S0735-6757(19)30620-5 https://doi.org/10.1016/j.ajem.2019.09.006 YAJEM 158516
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American Journal of Emergency Medicine
Received Date: Accepted Date:
5 September 2019 24 September 2019
Please cite this article as: T. Ronnie, L. Beyerlein, M. Murati, J.P. Louie, A PRESing Case of Visual Changes and Confusion, American Journal of Emergency Medicine (2019), doi: https://doi.org/10.1016/j.ajem.2019.09.006
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Title: A PRESing Case of Visual Changes and Confusion Tanisha Ronnie1 BS, Larrisa Beyerlein2 MD, Michael Murati3 MD, Jeffrey P. Louie2 MD Authors Affiliation 1Tanisha Ronnie, BS University of Minnesota School of Medicine 420 Delaware Street SE, Minneapolis, Minnesota 55455
[email protected], 612-625-6678 2Larissa
Beyerlein, MD University of Minnesota, Department of Pediatrics 2450 Riverside Avenue Minneapolis, Minnesota 55454
[email protected], 612-625-6678 3Michael
Murati, MD University of Minnesota, Department of Radiology 420 Delaware Street SE Minneapolis, MN 55455
[email protected], 612-626-3345 2Jeffrey
P. Louie, MD University of Minnesota, Department of Pediatrics 2450 Riverside Avenue Minneapolis, Minnesota 55454
[email protected], 612-625-6678 Corresponding Author Jeffrey P. Louie, MD University of Minnesota, Department of Pediatrics 2450 Riverside Avenue Minneapolis, Minnesota 55454
[email protected], 612-625-6678 Word Count: 772 Keywords: PRES, posterior reversible encephalopathy syndrome; Visual disturbances; Head CT; Cerebral hypoattenuation; Headache; Hypertension; Lupus Funding Source: The author(s) received no financial support for the research, authorship, and/or publication of this article. Declaration of Interest: The authors declared no potential conflicts of interest with respect
to the research, authorship, and/or publication of this article. ABSTRACT Visual disturbances are an uncommon pediatric chief complaint. Usually, after a complete ocular exam including visual acuity, most causes are benign and not life-threatening. Children with abnormal visual complaints who have underlying medical conditions, such as SLE or other autoimmune conditions, a recipient of a transplant, renal disease, and even eclampsia require closer scrutiny. We report a 10-year-old female with a history of systemic lupus erythematosus complicated by hypertension and cardiomyopathy secondary to lupus who presented to the emergency department with a history of vision loss and headache. Head computer tomography demonstrated findings of posterior reversible encephalopathy syndrome (PRES). PRES is a clinical disease associated with cranial radiological findings of heterogenous etiologies that is often reversible. Prompt recognition and treatment are important in preventing permanent damage, long term morbidity and even death.
CASE REPORT
A 10-year-old female with a history of systemic lupus erythematosus (SLE) complicated by secondary hypertension and cardiomyopathy presented to an emergency department with acute onset of vision change that she described as “a shadow coming over her eyes” and a parental complaint that their daughter has been disoriented, confused and tripping over furniture, and
complaining of a headache. The child denied blurry vision or eye pain. By report, she had been non-compliant with prednisone for greater than a month and recently depleted her amlodipine two days prior to presentation. Vitals upon arrival included temperature 99.2oF (37.3 oC), blood pressure 154/116 mmHg, heart rate 113 bpm, and respiratory rate of 20 breaths per minute. On examination, she was not appearing toxic or in distress. Her gait was normal, but did require guidance. The patient was alert and talkative. The ocular exam was normal with pupils responding equally to light with associated symmetric extra ocular movements and a visual acuity of 20/200 bilaterally. No cranial nerve or focal neurologic abnormalities noted. Mom reports her daughter does not require corrective lenses.
During an ophthalmology consult, the patient became incontinent of urine. On reexamination, mental status was altered, left eye deviation and lip smacking was apparent, and the blood pressure was 170/100 mmHg. No response to verbal command or painful stimulus. Her blood pressure and seizure improved with hydralazine and midazolam, respectively. A head CT revealed focal hypoattenuating areas within the right occipital, bilateral posterior frontal and parietal cortex, and subcortical areas (Figure 1) consistent with PRES, posterior reversible encephalopathy syndrome.
The patient was subsequently admitted to the Pediatric Intensive Care Unit for status epilepticus, hypertensive crisis and PRES. Hypertension was refractory and ultimately required up to 6 different antihypertensives. High-dose steroids were needed to control her underlying SLE. The patient’s hospital course was complicated by lupus related thrombotic microangiopathic hemolytic anemia. Unfortunately, despite two intraventricular drains to control the intracranial
pressure (ICP), brain herniation occurred. After serial exams and following the institution’s brain death protocol confirming brain death, parents withdrew support.
PRES is a clinical radiographic syndrome that was first described in 1996 (1). PRES has been reported in patients as young as 2 and as old as 90 years of age, but the true incidence is unknown. There are multiple etiologies associated with PRES in both the adult and pediatric population including cancer, sepsis, porphyria, chronic liver failure, SLE, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, acute renal failure, and high-dose corticosteroid dosing (2,3,4,5). A number of immunosuppressant have also been linked to PRES: cyclosporine, tacrolimus, and sirolimus as well as chemotherapeutics drugs: oxaliplatin, bevacizumab, sunitinib, and gemcitabine (6,7).
In adults the most common clinical features include headache (53%), visual changes (39%), and seizures (87%), as well as altered level of consciousness, and vomiting (8,9). Studies of PRES in adult patients have noted that development of status epilepticus poses a life-threatening complication of PRES (2). MRI is often characterized by bilateral white-matter abnormalities in posterior parieto-occipital regions of both cerebral hemispheres (9). Gray matter abnormalities within the parietooccipital region have also been identified in select cases (2,5,10,11). MRI is the preferred imaging modality to diagnose PRES, but in emergent situations, CT can be used and commonly shows vasogenic edema with a hemispheric distribution (12).
Documentation of PRES in the pediatric population is limited. Within reported pediatric case series, the common presentation of symptoms includes seizures, altered mental status, headaches
and visual disturbance (3,8,13,14). In a retrospective case study, the presenting clinical symptoms included headache (73%), vision dimness (36%), seizures (91%), and altered mental status (55%). The most significant contributing factor for development of PRES was uncontrolled hypertension which was seen in over 90% of cases (15,16). MRI findings are similar to that seen in the adult population and can include vasogenic edema, hyperintensity on T2 weighted images, and slightly increased or isointense signaling on diffusion weighted images within the posterior parietal and occipital regions (2,3,13).
In this particular case, the patient presented with vision disturbances, confusion, emesis, and seizure activity that progressed to status epilepticus. Head CT imaging showed hypoattenuating areas in the parieto-occipital regions and vasogenic edema. These findings were consistent with the diagnosis of PRES. Predisposing factors included SLE, lupus nephritis, and hypertensive nephropathy. The pillars of treatment for PRES include reduction of causative factors, management of hypertension, seizure control, maintenance of hydration status and maintenance of adequate arterial oxygenation (7). If adequate supportive measures are provided the average clinical recovery time is 3.8-4.8 days in pediatric cases, and 5.3 days in adults (2,6,13). Children presenting with visual disturbances, headache, and systemic hypertension, warrant a head CT, especially in patients with complex medical conditions.
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Figure 1: Head CT with areas of hypoattenuation in the right parietal (Image 1) and left parietal lobes (Image 2).