• valproate
• carnitine
• hyperammonaemia
• encephalopathy
• ureterosigmoidostomy

In the absence of liver disease, hyperammonaemia is often not considered in the differential diagnosis of encephalopathy and, therefore, the diagnosis of hyperammonaemic encephalopathy may be delayed. We report a case of fulminant progression of hyperammonaemic encephalopathy after valproate treatment in a patient with ureterosigmoidostomy.

A 31 year old patient was admitted because of confusion and agitation. Ureterosigmoidostomy for congenital bladder exstrophy was performed when he was 9 years old. Over the past years, he was repeatedly hospitalised with episodes of abnormal behaviour, which were blamed on his abuse of various illegal drugs. At this admission, he was disoriented, with lapses into somnolence as well as agitation. Apart from that, his general and neurological status was unremarkable. C reactive protein was 80.5 mg/l, leucocytes 16160/μl, and blood urea 12.6 mmol/l. Venous ammonia concentration was mildly increased (63 μmol/l). Arterial blood gas analysis showed respiratory alkalosis. All other laboratory analyses including toxicological screening, microbiological tests, cerebrospinal fluid analysis, and cranial computed tomography were unremarkable. Plasma amino acids, organic acids, and orotic acid in urine were normal. After treatment with fluids and amoxicillin …