Clinical StudyIs hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome?
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.
References (21)
The role of the endothelium in severe sepsis and multiple organ dysfunction syndrome
Blood
(2003)- et al.
Induction of apoptosis in mouse brain capillary endothelial cells by cyclosporin A and tacrolimus
Life Sci
(2000) - et al.
A reversible posterior leukoencephalopathy syndrome
N Engl J Med
(1996) - et al.
Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases
AJR Am J Roentgenol
(1992) - et al.
Cerebral vasospasm and eclampsia
Stroke
(1988) - et al.
Reversible posterior encephalopathy syndrome followed by MR angiography-documented cerebral vasospasm in preeclampsia-eclampsia: report of 2 cases
Cerebrovasc Dis
(2008) Cyclosporine-related central nervous system toxicity in cardiac transplantation
N Engl J Med
(1990)- et al.
Cyclosporine neurotoxicity and its relationship to hypertensive encephalopathy: CT and MR findings in 16 cases
AJR Am J Roentgenol
(1995) - et al.
Posterior reversible encephalopathy syndrome in infection, sepsis, and shock
AJNR Am J Neuroradiol
(2006) - et al.
Posterior reversible encephalopathy syndrome–an underrecognized manifestation of systemic lupus erythematosus
J Rheumatol
(2006)