Reye’s like Syndrome in an Adult; a Forgotten Entity

We report a case of 25 years old Caucasian lady who was admitted with history of use of non-steroidal anti-inflammatory drug for a viral like illness, repeated vomiting and hypokalemia. Her endoscopy showed pyloric stenosis for which she had surgery. 2 weeks after her surgery she became confused and had generalised tonic-clonic seizures. Her blood tests showed deranged liver function tests. Her lumbar puncture, autoimmune screen and viral PCR were negative. Her imaging was normal and her EEG showed slowing of the background activity consistent with diffuse encephalopathy. Her ammonia level was noted to be persistently high and there was a gradual deterioration of liver functions. Because of hyperammonia she was extensively investigated for inborn error of metabolism. Her tests were normal apart from high levels of glutamine. She had a liver ultrasound which showed fatty infiltration. A presumptive diagnosis of Reye's-like syndrome was made in view of her past history of viral like illness, analgesic overuse, persistent vomiting, hyperammonia, hepatic dysfunction, fatty liver and encephalopathy with low CSF white cell count. She was managed on intensive care with neuroprotective ventilation, haemofiltration to clear the ammonia and nutritional support. She eventually improved 2 months from the onset of symptoms. Reye's syndrome (RS) is an acute illness characterized by hepatic encephalopathy and fatty degeneration of the liver. It occurs in children but also has been reported in adults. It often occurs after viral infections treated with acetylsalicylic acid and NSAID. This case report alerts the Emergency staff and internists to the possibility of Reye's like syndrome in an adult person. The diagnosis of Reye's syndrome is not straightforward. It requires high index of suspicion and is usually a diagnosis of exclusion.