Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 28, 2017; 23(28): 5246-5252
Published online Jul 28, 2017. doi: 10.3748/wjg.v23.i28.5246
Refractory hepatic encephalopathy in a patient with hypothyroidism: Another element in ammonia metabolism
Fernando Díaz-Fontenla, Marta Castillo-Pradillo, Arantxa Díaz-Gómez, Luis Ibañez-Samaniego, Pilar Gancedo, Juan Adan Guzmán-de-Villoria, Pilar Fernández-García, Rafael Bañares-Cañizares, Rita García-Martínez
Fernando Díaz-Fontenla, Marta Castillo-Pradillo, Arantxa Díaz-Gómez, Luis Ibañez-Samaniego, Rafael Bañares-Cañizares, Rita García-Martínez, Unidad de Hepatología, Servicio de Aparato Digestivo, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
Luis Ibañez-Samaniego, Rafael Bañares-Cañizares, Rita García-Martínez, Instituto de Investigación Sanitaria Hospital Gregorio Marañón, 28007 Madrid, Spain
Pilar Gancedo, Servicio de Aparato Digestivo, Hospital Santa Bárbara, Puertollano, 13500 Ciudad Real, Spain
Juan Adan Guzmán-de-Villoria, Pilar Fernández-García, Servicio de Radiodiagnóstico, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
Juan Adan Guzmán-de-Villoria, Pilar Fernández-García, Centro de Investigación Biomédica en Red de Salud Mental, 28007 Madrid, Spain
Rafael Bañares-Cañizares, Rita García-Martínez, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas, 28007 Madrid, Spain
Rita García-Martínez, Facultad de Medicina, Universidad San Pablo CEU, 28007 Madrid, Spain
Author contributions: Díaz-Fontenla F, Castillo-Pradillo M, Díaz-Gómez A, Ibañez-Samaniego L, Fernández-García P and García-Martínez R contributed to the clinical evaluation and management of patient the and data acquisition; Guzmán-de-Villoria JA and Fernández-García P contributed to magnetic resonance data acquisition and interpretation; Bañares-Cañizares R critically reviewed the manuscript; García-Martínez R interpreted the data and drafted the manuscript; all authors contributed to this manuscript.
Institutional review board statement: This case report was exempt from the Institutional Review Board Standards at Hospital General Universitario Gregorio Marañon, Madrid, Spain.
Informed consent statement: All the procedures performed were in agreement with standard clinical practice. The patient gave written informed consent for publication.
Conflict-of-interest statement: The authors do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Rita García-Martínez, Unidad de Hepatología, Servicio de Aparato Digestivo, Hospital General Universitario Gregorio Marañón, Calle del Doctor Esquerdo 46, 28007 Madrid, Spain. rgarciamartinez3@salud.madrid.org
Telephone: +34-91-5868308 Fax: +34-34-91-4265125
Received: December 15, 2016
Peer-review started: December 17, 2016
First decision: January 10, 2017
Revised: February 21, 2017
Accepted: May 4, 2017
Article in press: May 4, 2017
Published online: July 28, 2017
Processing time: 224 Days and 14.4 Hours
Abstract

Hepatic encephalopathy (HE) remains a diagnosis of exclusion due to the lack of specific signs and symptoms. Refractory HE is an uncommon but serious condition that requires the search of hidden precipitating events (i.e., portosystemic shunt) and alternative diagnosis. Hypothyroidism shares clinical manifestations with HE and is usually considered within the differential diagnosis of HE. Here, we describe a patient with refractory HE who presented a large portosystemic shunt and post-ablative hypothyroidism. Her cognitive impairment, hyperammonaemia, electroencephalograph alterations, impaired neuropsychological performance, and magnetic resonance imaging and spectroscopy disturbances were highly suggestive of HE, paralleled the course of hypothyroidism and normalized after thyroid hormone replacement. There was no need for intervention over the portosystemic shunt. The case findings support that hypothyroidism may precipitate HE in cirrhotic patients by inducing hyperammonaemia and/or enhancing ammonia brain toxicity. This case led us to consider hypothyroidism not only in the differential diagnosis but also as a precipitating factor of HE.

Keywords: Hepatic encephalopathy; Hypothyroidism; Cognitive impairment; Cirrhosis; Portosystemic shunt; Magnetic resonance spectroscopy

Core tip: Hepatic encephalopathy (HE) remains a diagnosis of exclusion due to the lack of specific signs and symptoms. Refractory HE requires the search of hidden precipitating events and alternative diagnosis. We describe a patient with refractory HE who presented with large portosystemic shunt and post-ablative hypothyroidism. Her cognitive impairment, hyperammonaemia, electroencephalograph alterations, impaired neuropsychological performance and magnetic resonance imaging and spectroscopy disturbances suggestive of HE paralleled the course of hypothyroidism and improved after thyroid hormone replacement. The case findings support that hypothyroidism may precipitate HE in cirrhotic patients by inducing hyperammonaemia and/or enhancing ammonia brain toxicity.