Clinical Study
Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome?

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Abstract

Posterior reversible encephalopathy syndrome (PRES) has a distinctive clinical presentation and typical neuroimaging findings. However, data on its clinical course and recurrence are scarce. This study aims to investigate its clinical profile and factors that predict recurrence. We included patients diagnosed with PRES between 2005 and 2010 and collected data on demographics, presenting symptoms, co-morbidities, risk factors, clinical parameters, MRI findings, complications and recurrence. Patients were categorized into two groups: PRES due to primary hypertension and PRES due to secondary causes. Correlation with presenting symptoms, radiological features, and recurrence were analyzed. PRES was identified in 28 patients. Fourteen (50%) had primary hypertension. Secondary causes included immunosuppression-related (39%), preeclampsia/eclampsia (7%), and marijuana-intake-related (4%) causes. Patients presented with altered mental status (79%), headache (75%), seizure (68%), visual disturbance (39%) and hemiparesis (21%). On MRI 93% had the typical parietal–occipital involvement. The frontal lobe was affected in 64%, cerebellum in 29%, brainstem in 21%, and basal ganglia in 11%. About 36% had cortical involvement; 21% had diffusion-restricted lesions. Non-aneurysmal subarachnoid haemorrhage was found in 18% of patients and intracerebral hemorrhage in 14% of patients. No significant difference existed in presenting symptoms and the MRI distribution of vasogenic edema between the primary hypertension group and the secondary causes group. Recurrence occurred in four patients (14.3%, 95% confidence interval 4.2–33.7) and was significantly associated (p = 0.05) with primary hypertension as the etiology. Intensive monitoring and treatment of hypertension is recommended for reducing morbidity.

Introduction

Posterior reversible encephalopathy syndrome (PRES) was first described by Hinchey et al. in 1996.1 It is characterized by the presence of headache, encephalopathy, visual disturbance and seizures. Severe hypertension,2 pre-eclampsia or eclampsia,3, 4 use of immunosuppressive drugs,5, 6 sepsis,7 and history of renal and autoimmune diseases8, 9, 10 have been identified as possible predisposing factors. Occasionally, the clinical presentation may be non-specific. MRI is required for diagnosis, which typically demonstrates focal vasogenic edema over the “posterior” parieto-occipital white matter. However, it is not uncommon for the edema to also involve the cortex, frontal lobes, brainstem and basal ganglia.11

Although PRES has been described for more than 15 years, its natural history and pathogenesis remain largely unknown. Case reports have been published on its radiological features, but systemic studies on its clinical history and prognosis are scarce. The 15 patients described by Hinchey et al. had completely reversible clinical signs, and among the eight patients with follow up neuroimaging, six had full resolution of abnormalities.1 However, subsequent single case reports have suggested the possibility of PRES recurrence despite apparent symptom resolution.12, 13 To our knowledge, systemic data on this aspect is lacking, especially factors that may predict recurrence.

A better understanding of the clinical history and recurrence of PRES is essential, as early recognition and prompt intensive management may alter the outcome and prognosis of this life-threatening condition, minimizing secondary complications such as intracranial haemorrhage, status epilepticus and cerebral infarction, which all lead to significant morbidity and mortality.

Our study aims to investigate the clinical profile and associated radiological features of PRES, with particular emphasis on its recurrence.

Section snippets

Methods

We retrospectively identified all patients with PRES diagnosed clinically at Royal Melbourne Hospital (RMH) between 1st October 2005 and 30th September 2010. The patient database was generated from the RMH discharge notes database and the RMH radiology information system (Karisma System, Kestral Computing, Melbourne, Vic., Australia) through keyword search in the final diagnosis for “posterior reversible encephalopathy syndrome”, “PRES”, “reversible posterior leukoencephalopathy syndrome”. All

Clinical profile

We identified PRES in 28 patients (22 women and six men, female-to-male ratio: 3.7:1) during 5 years. The median age at presentation was 48 years old, with an IQR of 21.3 (minimum to maximum, 18–73 years). The median age was 52.5 years for patients with primary hypertension, and 38.5 years for patients with secondary causes. The etiologies of PRES in these patients are listed in Table 1. The median age difference was statistically significant between the two groups (p = 0.04). No gender predominance

Discussion

PRES has been described as a syndrome with distinctive clinical profile, associated with typical neuroimaging findings. Our study demonstrated a spectrum of clinical symptoms consistent with data from the previous literature, showing similar patterns of disease presentation.1, 14, 15 Most of our patients had typical posterior vasogenic edema on MRI. However, the previously classified “atypical” areas, including the cortex, brainstem, cerebellum and basal ganglia, were not infrequently affected

Conflicts of interest/disclosures

The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

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