A Case of PRESS Syndrome Presenting With Nausea, Vomiting, and Abdominal Pain

Abstract

A 68 year-old, Caucasian female presented to the emergency department with 4 day history of nausea, vomiting and generalised abdominal pain associated with delusions, confusion, agitation, and stupor. She had no history of illicit drug use, alcohol abuse, recent medication change, hypoglycemia, falls or psychiatric illness. On examination, she had a Blood pressure 206/145 with a pulse of 84 and normal respiration. She was able to ambulate with an unsteady gait and upper extremity apraxia.The rest of her examination was otherwise normal. EEG failed to show features suggesting any underlying seizure activity. CT scan of the brain was negative for hemorrhages. MRI of the brain demonstrated volume loss of periventricular white matter disease and abnormal symmetric patchy foci of increased signal in cortex, subcortical white matter of the posterior frontal and occipital lobes. MRA was normal. The patient responded well to conservative measures including anti-emetics and anti-hypertensive therapy. Her agitation, aggression, confusion and disorientation and gastrointestinal symptoms resolved. Based on clinical course and radiological findings, it was concluded that this patient had Posterior Reversible Encephalopathy Syndrome (PRES) also known as Reversible Posterior Leukoencephalopathy Syndrome (RPLS). PRES is a radiologically diagnosed condition. Imaging shows posterior brain hyperintensities which translates to vasogenic edema, frequently seen in the white matter junctions. Most common locations of involvement are the occipital lobes, parietal lobes, and posterior frontal lobes. Clinically, it is associated with accelerated/malignant hypertension, eclampsia, renal disease, seizures, autoimmune disorders, and immunosuppressive therapy, but these conditions do not demonstrate causality. Pathophysiology of the syndrome is uncertain, although hypotheses of endothelial dysfunction, autonomic dysregulation, and cerebral ischemia have been postulated to be at the root of the problem. Prevalence is high in women. Clinicians most often see patients manifesting with mental status changes, constant headaches, seizures, and vision impairment. Gastrointestinal symptoms usually resolves with conservative measures. Diagnosis relies on a combination of clinical presentation and imaging, but emphasis is on neurological imaging. Treatment of the condition starts with early detection and diagnosis, which requires early recognition of the constellation of symptoms, associated conditions and MRI scanning of the brain. Removal of the offensive agent(s) prevents further progression of PRES and reverses the vasogenic edema present in the brain, which in turn, avoids permanent neurological impairment.