Extrahepatic portosystemic shunts as an unusual but treatable cause of hyperammonemic encephalopathy in a noncirrhotic patient

Extrahepatic portosystemic shunts as an unusual but treatable cause of hyperammonemic encephalopathy in a noncirrhotic patient – a case report

Published:2022-01 Issue: Volume:15 Page:175628642210976
ISSN:1756-2864
Container-title:Therapeutic Advances in Neurological Disorders
language:en
Short-container-title:Ther Adv Neurol Disord

Author:

Kühne Escolà Jordi¹^ORCID,Theysohn Jens M.²,Li Yan²,Forsting Michael²,Capetian Philipp³,Volkmann Jens³,Lange Christian⁴,Quesada Carlos M.¹,Köhrmann Martin¹,Frank Benedikt¹^ORCID,Kleinschnitz Christoph⁵

Affiliation:

1. Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Essen, Germany

2. Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany

3. Department of Neurology, University Hospital Würzburg and Julius-Maximilian-University Würzburg, Würzburg, Germany

4. Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany

5. Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Hufelandstr. 55, 45147 Essen, Germany

Abstract

We report a case of hyperammonemic encephalopathy due to extrahepatic portosystemic shunts in a noncirrhotic patient. A 79-year-old woman suffered from episodic confusion, disorientation, dysphasia and fluctuating level of consciousness. Electroencephalography (EEG) showed encephalopathic changes and serum levels of ammonia were elevated. Further investigation revealed mesenterorenal and mesenterocaval shunts, which had possibly evolved after pancreatic surgery 5 years ago. After shunt obliteration, the symptoms completely resolved, ammonia levels dropped to the normal range and EEG findings normalized. Clinicians should be aware of this rare but treatable cause of encephalopathy in noncirrhotic patients.

Publisher

SAGE Publications

Subject

Neurology (clinical),Neurology,Pharmacology

Link

http://journals.sagepub.com/doi/pdf/10.1177/17562864221097614

Reference23 articles.

1. Cause for Confusion: Noncirrhotic Hyperammonemic Encephalopathy

2. Noncirrhotic hyperammonaemic encephalopathy

3. Hyperammonemia: What Urea-lly Need to Know: Case Report of Severe Noncirrhotic Hyperammonemic Encephalopathy and Review of the Literature

4. Hyperammonemia and encephalopathy in patients with multiple myeloma

5. Adult Onset Urea Cycle Disorder in a Patient With Presumed Hepatic Encephalopathy

Cited by 1 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. Levetiracetum/gabapentin/valproic-acid;Reactions Weekly;2022-06