Abstract
Background: Accurate diagnosis and timely treatment for posterior reversible encephalopathy syndrome (PRES) with atypical regions are very important in clinical practice. However, until now, little has been known about the clinical and MRI manifestations of this disease. Therefore, the aim of this study is to investigate the clinical and MRI features of PRES to promote clinical management and deepen our understanding of this disease. Materials and Methods: Data from six PRES patients with atypical regions were collected from our hospital. Data from another 550 cases were obtained by searching the PubMed, EMBASE and Web of Science databases with the keywords "posterior reversible encephalopathy syndrome" "PRES" "reversible posterior leukoencephalopathy" "RPLS" "hypertensive encephalopathy" "hyperperfusion encephalopathy" or "reversible posterior cerebral edema encephalopathy." The clinical and MRI features of these 556 cases were analyzed together. Results: A total of 305 patients were female, and 248 were male, with a median age of 34 years. The information on sex and age of three patients was not available. The most common symptom was headache (282/556, 50.7%), followed by altered mental status (243/556, 43.7%), seizures (233/556, 41.9%), visual disturbances (194/556, 34.9%), nausea/vomiting (130/556, 23.4%), and focal neurological deficits (101/556, 18.2%). Hypertension (425/556, 76.4%), renal diseases (152/556, 27.3%), immunosuppressant drugs (79/556, 14.2%), and chemotherapy/chemoradiotherapy (59/556, 10.6%) were the major predisposing factors. The atypical regions of the lesions were the cerebellum (331/556, 59.5%), basal ganglia (135/556, 24.3%), periventricular/deep white matter (125/556, 22.5%), pons (124/556, 22.3%), brainstem (115/556, 20.7%), thalamus (114/556, 20.5%), midbrain (48/556, 8.6%), spinal cord (33/556, 5.9%), and medulla (29/556, 5.2%). Additionally, the following typical regions were observed: occipital (278/556, 50.0%), parietal (234/556, 42.1%), frontal (150/556, 27.0%), and temporal (124/556, 22.3%) lobes. The major treatments were antihypertensives (358/515, 69.5%), antiepileptics/sedation (126/515, 24.5%), discontinuation/switching agents (67/515, 13.0%), and steroids (54/515, 10.5%). The median time of the clinical state improved and abnormal neuroimaging resolved is 2-3 weeks after appropriate treatment. Conclusion: The common symptoms of PRES with atypical regions include headaches, altered mental status, seizures, visual disturbances, nausea or vomiting, and focal neurological deficits. The frequent predisposing factors include hypertension, renal diseases, immunosuppressant drugs and chemotherapy/chemoradiotherapy. MRI features are mainly characterized by vasogenic edema in central zones always accompanied by typical regions. Most cases can be reversed in 2-3 weeks when promptly recognized and properly treated.