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Journal of Neurology Neurosurgery and Psychiatry 2002;73:90-91
© 2002 Journal of Neurology Neurosurgery and Psychiatry


LETTER

Fulminant progression of hyperammonaemic encephalopathy after treatment with valproate in a patient with ureterosigmoidostomy

S Schwarz1, D Georgiadis1, S Schwab1, F Gehlen2, E Mayatepek3, S Zoubaa4

1 Department of Neurology, University of Heidelberg, INF 400, Heidelberg, Germany
2 Department of Nephrology
3 Department of General Paediatrics, Division of Metabolic and Endocrine Diseases
4 Department of Pathology, Division of Neuropathology

Correspondence to:
Correspondence to:
Dr Stefan Schwarz, Department of Neurology, Klinikum Mannheim of the University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany;
schwarz@neuro.ma.uni-heidelberg.de

Keywords: 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 . . . [Full text of this article]




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Nephrol Dial TransplantHome page
D. J. Hampel, J. Stoll, L. Nibbe, and M. Gollasch
Hyperammonaemic encephalopathy and severe metabolic acidosis in a patient with chronic renal insufficiency years after ureterosigmoidostomy
Nephrol. Dial. Transplant., September 1, 2007; 22(9): 2713 - 2719.
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