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Pigtail probe coiled in distal ileum due to migration, conditioning severe retrograde small bowel dilation.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Arantxa Díaz Gómez, Raquel Díaz Ruiz, Óscar Nogales, Beatriz de la Casa, Carmen Fernández Alvárez, Cecilia González Asanza" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Arantxa" "apellidos" => "Díaz Gómez" ] 1 => array:2 [ "nombre" => "Raquel" "apellidos" => "Díaz Ruiz" ] 2 => array:2 [ "nombre" => "Óscar" "apellidos" => "Nogales" ] 3 => array:2 [ "nombre" => "Beatriz" "apellidos" => "de la Casa" ] 4 => array:2 [ "nombre" => "Carmen" "apellidos" => "Fernández Alvárez" ] 5 => array:2 [ "nombre" => "Cecilia" "apellidos" => "González Asanza" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0210570517300523?idApp=UINPBA00004N" "url" => "/02105705/0000004100000003/v2_201806280404/S0210570517300523/v2_201806280404/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S0210570517300766" "issn" => "02105705" "doi" => "10.1016/j.gastrohep.2017.03.003" "estado" => "S300" "fechaPublicacion" => "2018-03-01" "aid" => "1124" "copyright" => "Elsevier España, S.L.U." 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Cámara-Lemarroy, Ingrid Estrada-Bellmann" "autores" => array:5 [ 0 => array:3 [ "nombre" => "Roberto" "apellidos" => "Monreal-Robles" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "Guillermo" "apellidos" => "Delgado-García" "email" => array:2 [ 0 => "grdelgadog@gmail.com" 1 => "guillermo.delgadogr@uanl.edu.mx" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "Erik" "apellidos" => "García-Valadez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "Carlos R." "apellidos" => "Cámara-Lemarroy" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 4 => array:3 [ "nombre" => "Ingrid" "apellidos" => "Estrada-Bellmann" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Gastroenterología, Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, N.L., Mexico" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Medicina Interna, Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, N.L., Mexico" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, N.L., Mexico" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Mioclono que respondió al tratamiento antiamonio como presentación inicial de la degeneración hepatocerebral adquirida" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 975 "Ancho" => 975 "Tamanyo" => 66137 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial T1-weighted brain magnetic resonance imaging. Symmetrical hyperintensities in lentiform nuclei (arrows).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acquired hepatocerebral degeneration (AHD) is an often debilitating neurological disorder characterized by a variety of movement disorders in the setting of chronic liver disease (CLD) and portosystemic shunt. AHD was first described by van Woerkem in 1914.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Cirrhosis-related parkinsonism is the core manifestation in AHD and has been described to have a prevalence of 4.2%.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Reports of myoclonus in patients with AHD are scarce<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> and they always present along with other clinical manifestations, most often parkinsonism and cerebellar signs. Liver transplantation is presently the only therapeutic option that has been shown to ameliorate and even reverse neurological deterioration.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> Here, we describe the case of a 57-year-old woman with an unusual presentation of AHD who responded well to treatment with anti-ammonia therapy.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 57-year-old woman was admitted to the emergency department because of altered mental status and abnormal upper-limbs movements. She had a history of primary biliary cirrhosis and reported no alcohol consumption. Viral markers for HIV, hepatitis B and C were all negative. She also had a history of esophageal varices and had a previous diagnosis of splenorenal shunt, which was detected by a contrast-enhanced abdominal computed tomography scan. Her regular medication included propranolol and ursodeoxycholic acid. In the past she has had several episodes of acute hepatic encephalopathy (AHE); these episodes have been successfully treated, in the outpatient setting, with oral L-ornithine-L-aspartate (LOLA) and oral lactulose.</p><p id="par0015" class="elsevierStylePara elsevierViewall">On admission our patient had an altered mental status. On physical exam, there was no jaundice, telangiectases, ascites, superficial collateral abdominal veins or hepatosplenomegaly. She had sudden, brief, shock-like jerks of her upper limbs consistent with myoclonus. Hyperreflexia was also found in the same limbs, and Babinski sign was elicited bilaterally. Kayser–Fleischer rings were not seen on ophthalmologic examination. She initially received lactose enemas as treatment for AHE, with improvement in her mental status. However, there was no change in her myoclonus. A brain magnetic resonance imaging (MRI) scan revealed bilateral symmetrical hyperintensities in lentiform nuclei on T1-weighted images (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). She had a mild thrombocytopenia (148<span class="elsevierStyleHsp" style=""></span>K/mm<span class="elsevierStyleSup">3</span>) but neither anemia nor leukocytosis was found. She was euglycemic, and renal function was normal. Serum electrolytes and coagulation tests were non-contributory. She was not hypoalbuminemic but had a mild hyperbilirubinemia (total: 2.2<span class="elsevierStyleHsp" style=""></span>mg/dL; conjugated: 0.6<span class="elsevierStyleHsp" style=""></span>mg/dL); other liver function tests were as follow: serum glutamic–oxaloacetic transaminase 88<span class="elsevierStyleHsp" style=""></span>U/L (10–42<span class="elsevierStyleHsp" style=""></span>U/L), serum glutamate-pyruvate transaminase 42<span class="elsevierStyleHsp" style=""></span>U/L (10–42<span class="elsevierStyleHsp" style=""></span>U/L), alkaline phosphatase 134<span class="elsevierStyleHsp" style=""></span>U/L (38–126<span class="elsevierStyleHsp" style=""></span>U/L).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Based on MRI findings, we diagnosed AHD and ammonia-lowering therapy was continued to treat her myoclonus, since liver transplantation was not a practical possibility in our setting. The patient showed a mild improvement during her hospital stay. When her mental status normalized, after one week of treatment, she was discharged on oral lactulose (20<span class="elsevierStyleHsp" style=""></span>g thrice daily), rifaximin (400<span class="elsevierStyleHsp" style=""></span>mg twice daily) and LOLA (6<span class="elsevierStyleHsp" style=""></span>g thrice daily). She was then evaluated weekly for tolerability and adherence. One month later she presented to the office with complete resolution of her myoclonus, and we decided to continue the same treatment. After 18 months of follow up, the patient continues with ammonia-lowering therapy and has not had any recurrence of movement disorders or encephalopathy.</p><p id="par0025" class="elsevierStylePara elsevierViewall">CLD may be associated with a wide variety of motor and neuropsychiatric manifestations as a result of the diversion of portal blood flow to the systemic circulation, presumably through toxic effects of chemical substances that cross the blood–brain barrier. AHD is a chronic encephalopathy characterized by cognitive impairment, parkinsonism, and other movement disorders besides myoclonus, including ataxia, chorea, and dystonia.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> Pathophysiological mechanisms are poorly understood and many factors are probably involved. Ammonia and manganese are both candidate substances for chronic neurological dysfunction in liver diseases, and more recently it has been demonstrated that there is a pathologically decreased striatal dopamine D2 receptor availability and decreased dopamine transporter availability in the pathogenesis of cirrhosis-related parkinsonism.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> Brain MRI typically showed high intensity signal in the lenticular nuclei on T1-weighted images, which differentiates this entity from AHE.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Although chronic AHD can present with almost any kind of movement disorder, myoclonus is an extremely rare symptom that has been very seldom reported.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> At least one other case has been described in the literature, involving a patient with Budd-Chiari syndrome and T1 hyperintensities in pallidum and substantia nigra.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> In CLD and portosystemic shunt, ammonia toxicity and manganese accumulation in the mitochondria of glial cells in the pallidum and other basal ganglia structures may lead to disruption of energy metabolism and the characteristic imaging findings.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,7,10</span></a> In general, dopaminergic treatment is ineffective, manganese chelation unavailable, and anti-ammonia therapy often without any substantial benefit, leaving liver transplantation as the most reasonable alternative.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2–5,7</span></a> In our case, anti-ammonia treatment proved successful, suggesting that this therapy should be attempted in settings where liver transplantation is not possible.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 975 "Ancho" => 975 "Tamanyo" => 66137 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial T1-weighted brain magnetic resonance imaging. 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Year/Month | Html | Total | |
---|---|---|---|
2024 November | 2 | 0 | 2 |
2024 October | 33 | 6 | 39 |
2024 September | 26 | 6 | 32 |
2024 August | 23 | 3 | 26 |
2024 July | 33 | 5 | 38 |
2024 June | 18 | 2 | 20 |
2024 May | 28 | 4 | 32 |
2024 April | 19 | 4 | 23 |
2024 March | 46 | 7 | 53 |
2024 February | 43 | 7 | 50 |
2024 January | 68 | 5 | 73 |
2023 December | 60 | 10 | 70 |
2023 November | 56 | 3 | 59 |
2023 October | 80 | 9 | 89 |
2023 September | 39 | 2 | 41 |
2023 August | 47 | 3 | 50 |
2023 July | 88 | 3 | 91 |
2023 June | 49 | 2 | 51 |
2023 May | 73 | 8 | 81 |
2023 April | 64 | 8 | 72 |
2023 March | 60 | 2 | 62 |
2023 February | 51 | 5 | 56 |
2023 January | 39 | 7 | 46 |
2022 December | 50 | 10 | 60 |
2022 November | 46 | 9 | 55 |
2022 October | 39 | 15 | 54 |
2022 September | 44 | 14 | 58 |
2022 August | 41 | 21 | 62 |
2022 July | 44 | 16 | 60 |
2022 June | 72 | 13 | 85 |
2022 May | 55 | 10 | 65 |
2022 April | 54 | 19 | 73 |
2022 March | 54 | 13 | 67 |
2022 February | 43 | 14 | 57 |
2022 January | 60 | 14 | 74 |
2021 December | 36 | 13 | 49 |
2021 November | 35 | 9 | 44 |
2021 October | 54 | 12 | 66 |
2021 September | 26 | 12 | 38 |
2021 August | 30 | 8 | 38 |
2021 July | 44 | 12 | 56 |
2021 June | 25 | 11 | 36 |
2021 May | 25 | 8 | 33 |
2021 April | 46 | 13 | 59 |
2021 March | 20 | 8 | 28 |
2021 February | 25 | 11 | 36 |
2021 January | 15 | 19 | 34 |
2020 December | 12 | 8 | 20 |
2020 November | 13 | 4 | 17 |
2020 October | 17 | 6 | 23 |
2020 September | 24 | 12 | 36 |
2020 August | 15 | 8 | 23 |
2020 July | 13 | 5 | 18 |
2020 June | 14 | 12 | 26 |
2020 May | 20 | 13 | 33 |
2020 April | 7 | 6 | 13 |
2020 March | 15 | 13 | 28 |
2019 May | 1 | 0 | 1 |
2018 August | 2 | 0 | 2 |
2018 July | 1 | 0 | 1 |
2018 June | 0 | 4 | 4 |
2018 April | 6 | 8 | 14 |
2018 March | 4 | 6 | 10 |
2018 February | 6 | 8 | 14 |
2017 June | 0 | 1 | 1 |