Hypertriglyceridemia is the third most common cause of acute pancreatitis, accounting for up to 4% of cases.1, 2Severe acute pancreatitis is characterized by persistent organ failure for >48 hours and warrants intensive care unit admission because it has a mortality rate of 20%.3 Patients with severe acute pancreatitis have been noted to have higher levels of proinflammatory cytokines, including tumor necrosis factor-α, interleukin (IL)-1β, IL-6, and IL-8, likely contributing to increased disease severity.4 The prevalence of pancreatic encephalopathy syndrome has been reported to be 0.0005%-0.001%, and it has a very poor prognosis, with high mortality.3 We report a rare case of diffuse cerebral edema causing pancreatic encephalopathy in a patient who developed acute pancreatitis from hypertriglyceridemia.
A 41-year-old man with depression and epilepsy presented with abdominal pain, vomiting, and confusion for 1 week. Vital signs were significant for temperature of 38.1°C, heart rate of 123 beats per minute, blood pressure of 113/79 mm Hg, and respiratory rate of 23 breaths per minute. Physical examination results were remarkable for abdominal distension, hypoactive bowel sounds, and diffuse tenderness on palpation. The patient appeared lethargic, distressed, and suffered from episodic convulsions characterized by upper extremity muscle spasms. Laboratory studies were significant for amylase of 486 U/L and lipase of 1566 U/L. The triglyceride level was found to be elevated at 8279 mg/dL. The patient also had renal and respiratory failure requiring continuous veno-venous hemofiltration and mechanical ventilation, respectively. Computed tomography (CT) of the abdomen demonstrated acute pancreatitis. Electroencephalogram was consistent with encephalopathy. A CT scan of the head showed diffuse loss of gray–white matter differentiation and sulcal effacement in both cerebral hemispheres, suggestive of diffuse brain edema. The patient was started on an insulin drip in addition to plasmapheresis, and his triglyceride levels gradually decreased to <500 mg/dL. Repeat CT of the head showed interval improvement of diffuse cerebral edema. With decreasing levels of triglycerides, the patient’s encephalopathy resolved.
The pathophysiology behind pancreatic encephalopathy involves pancreatic enzymes, such as trypsin, and phospholipase A, leading to nerve cell toxicosis, and local encephalomalacia.5 Trypsin actives the A2 phospholipase, which converts pancreatic lecithin and cephalin into hemolytic lecithin and cephalin, which destroy membrane phospholipids of the blood–brain barrier.5 This eventually leads to diffuse cerebral edema, as seen in our patient. Other mechanisms describe the role of proinflammatory cytokines in contributing to the development of vasogenic cerebral edema.6 Patients with pancreatic encephalopathy may present with confusion, visual and auditory hallucinations, and psychomotor agitation. Occasionally, neurologic deficits and convulsions may occur. In our patient mental confusion and periodic convulsions were observed. Given the patient’s acute onset of confusion, convulsions, and electroencephalogram findings, the patient’s altered mental status can likely be attributed to encephalopathy from severe acute pancreatitis. Some cases have reported the development of posterior reversible encephalopathy syndrome in patients with acute pancreatitis.6 In posterior reversible encephalopathy syndrome, patients develop altered mental status from vasogenic edema of the posterior temporal and parieto-occipital lobes and cerebellum.6 In our case the patient developed diffuse cerebral edema, not localized to the posterior hemispheres. The severity of his altered mental status may be explained by the extent of his cerebral edema.
Clinicians need to recognize and identify patients at risk for developing pancreatic encephalopathy early in the course of acute pancreatitis, because it is critical in improving outcome. Early recognition has been shown to reduce the rates of organ failure, morbidity, and mortality.3
To read this article in its entirety please visit our website.
-Dalvir Gill, MD, Natasha Sheikh, MSc, Amish Shah, MD, Vanessa Goyes Ruiz, MD, Dana Savici, MD
This article originally appeared in the May 2017 issue of The American Journal of Medicine.
