Encephalopathy and Fatty Degeneration of the Viscera: A Clinicopathologic Analysis of 40 Cases

Abstract

Forty cases of encephalopathy and fatty degeneration of the viscera in Thai children confirmed at autopsy or by biopsy were characterized by the abrupt onset of coma or convulsions, fever, respiratory distress, vomiting, and death within 72 hr. Abnormal laboratory findings included: elevated serum transaminases and free fatty acids; prolonged prothrombin times; decreased carbon dioxide content, cholesterol, and triglyceride values. Spinal fluid was clear with low glucose. Autopsies revealed cerebral edema and neuronal degeneration; fatty degeneration of the liver, kidneys, and heart; and generalized lymphocytosis. Fatty metamorphosis of hepatocytes was generalized but was most pronounced in portal areas (the increased lipids were identified as triglycerides, diglycerides, and fatty acids); renal involvement was most severe in the proximal tubules; cardiac changes were worse in atrial and conduction system fibers. It is postulated that the syndrome results from acute toxic hepatitis occurring in patients with subclinical hepatic disease. Circumstantial evidence suggests that anatoxin B₁ may be an etiologic agent in the disease in Thailand.