Buscar en
Neurología (English Edition)
Toda la web
Inicio Neurología (English Edition) Aphasia in a patient with acute hepatic encephalopathy associated with multifoca...
Journal Information
Vol. 33. Issue 6.
Pages 410-412 (July - August 2018)
Vol. 33. Issue 6.
Pages 410-412 (July - August 2018)
Letter to the Editor
DOI: 10.1016/j.nrleng.2016.03.012
Open Access
Aphasia in a patient with acute hepatic encephalopathy associated with multifocal cortical brain lesions
Afasia en paciente con encefalopatía hepática aguda asociada a lesiones corticales cerebrales multifocales
Visits
...
N. Falgàsa, I. Alfarob, G. Crespob, J. Berenguerc, J.C. García-Pagánb,d, E. Muñoza,
Corresponding author
jemunoz@clinic.ub.es

Corresponding author.
a Servicio de Neurología, Hospital Clínic de Barcelona, Barcelona, Spain
b Unidad de Hepatología, Hospital Clínic de Barcelona, Barcelona, Spain
c Servicio de Radiología, Hospital Clínic de Barcelona, Barcelona, Spain
d Laboratorio de Hemodinámica Hepática, CIBERehd, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona, Hospital Clínic Barcelona, Barcelona, Spain
Article information
Full Text
Bibliography
Download PDF
Statistics
Figures (1)
Full Text
Dear Editor,

Acute hepatic encephalopathy (AHE) is typically characterised by a wide range of neuropsychiatric manifestations including behavioural, cognitive, or mood changes associated with asterixis and different degrees of alteration to the level of consciousness, which may progress to stupor or coma in some cases.1 This situation may be due to acute liver failure, cirrhosis, portal hypertension, or a transjugular intrahepatic portosystemic shunt.2,3 Brain magnetic resonance imaging (MRI) of patients with AHE may reveal signal alterations in different brain areas, caused by vasogenic and cytotoxic oedema related to the toxic effect of ammonia in the brain.4,5 These alterations may disappear after resolution of the encephalopathy,5 or may progress to cortical laminar necrosis in cases with poor outcomes.6 Presentations with focal manifestations are very infrequent in AHE, and may lead to diagnostic errors.

Clinical case. The patient was a 50-year-old man who was diagnosed in 2002 with portal vein thrombosis secondary to protein S deficiency, which was treated with a transjugular intrahepatic portosystemic shunt (TIPS) and oral anticoagulation. He was admitted to another hospital in May 2015 due to a sudden loss of consciousness which progressively and spontaneously improved over the following hours. The patient was transferred to our hospital to rule out the possibility of cerebrovascular accident, since he presented difficulties speaking after recovering a normal level of consciousness. During the neurological examination at admission, the patient was awake and presented mild asterixis in both hands; the most striking finding, however, was the presence of mixed aphasia characterised by hypofluent speech with presence of frequent phonological paraphasias in spontaneous language and difficulty understanding complex commands. Word repetition was preserved, although he showed difficulties repeating simple sentences. The neurological examination yielded otherwise normal results. The blood test revealed chronic anaemia, altered liver profile (ASAT 58IU/L, total bilirubin 3.3mg/dL) and moderately elevated levels of ammonia (91μmol/L). The peritoneal fluid analysis and a brain computed tomography showed no abnormalities. However, the brain MRI showed altered high intensity signal on the fast fluid-attenuated inversion recovery (FLAIR), T2-weighted, and diffusion-weighted imaging (DWI) sequences in the left frontal parasagittal, insular, temporal, and cingulate cortices, as well as the right insular cortex, with no abnormal diffusion restriction in the apparent diffusion coefficient (ADC) map (Fig. 1A). The MR-angiography showed permeability of the intra- and extracranial vessels. An EEG study revealed presence of a delta rhythm with bi- and triphasic waves suggestive of toxic-metabolic dysfunction, with no associated epileptiform activity. We finally diagnosed the patient with acute aphasia as a manifestation of AHE associated with multifocal cortical brain lesions, predominantly involving the left insular and temporal cortices, and in the context of a TIPS. The patient received cathartic therapy, with aphasia progressively improving and resolving several weeks later. An additional MRI scan performed 2 months later showed that the cortical lesions had completely disappeared (Fig. 1B).

Figure 1.

(A) Brain MRI revealing cortical and subcortical hyperintensity in both insular areas, the left temporal lobe, and the parasagittal frontal lobe on T2-weighted and FLAIR sequences. DWI showed cortical signal alterations in all these areas, with no abnormal diffusion restriction in the ADC map. (B) A second MRI scan performed 2 months later revealed that lesions had disappeared.

(0.38MB).

Discussion. Few cases have been described of patients with TIPS who, in the context of AHE, developed focal neurological signs associated with presence of cortical lesions in neuroimaging.7–9 Two of the patients described in the literature presented sudden loss of consciousness with posterior aphasia, with brain MRI scans showing cortical lesions with a similar location to that described in our patient.

Correlation of the level of ammonia with the type of manifestation and the clinical severity of encephalopathy is a controversial issue.10 In our case, as in that reported by Babington et al.7, blood ammonia levels increased only moderately. This suggests that the pathophysiology of these pseudo-ictal discharges may involve factors other than ammonia, such as inflammatory mechanisms or other potential toxins,1,11 the individual susceptibility of the brain to the toxic effect of ammonia, or the rate of increase of ammonia levels in the brain, favoured by TIPS.

Diagnosis of AHE in case of acute focal symptoms in patients with liver disease or TIPS is important since it may have prognostic consequences. It is well known that patients undergoing early, aggressive treatment for AHE have a better prognosis for recovery of neurological deficits and resolution of brain lesions. However, results may not be favourable, with the condition even resulting in the death of the patient, if the aetiology of these lesions is uncertain and treatment is started late.4,8

Conflicts of interest

None.

References
[1]
R. Prakash, K.D. Mullen.
Mechanisms, diagnosis and management of hepatic encephalopathy.
Nat Rev Gastroenterol Hepatol, 7 (2010), pp. 515-525
[2]
J.C. García-Pagán, K. Caca, C. Bureau, W. Laleman, B. Appenrodt, A. Luca, Early TIPS (Transjugular Intrahepatic Portosystemic Shunt) Cooperative Study Group, et al.
Early use of TIPS in patients with cirrhosis and variceal bleeding.
N Engl J Med, 362 (2010), pp. 2370-2379
[3]
O. Riggio, S. Nardelli, F. Moscucci, C. Pasquale, L. Ridola, M. Merli.
Hepatic encephalopathy after transjugular intrahepatic portosystemic shunt.
Clin Liver Dis, 16 (2012), pp. 133-146
[4]
J.M. U-King-Im, E. Yu, E. Bartlett, R. Soobrah, W. Kucharczyk.
Acute hyperammonemic encephalopathy in adults: imaging findings.
AJNR Am J Neuroradiol, 32 (2011), pp. 413-418
[5]
A.M. McKinney, B.D. Lohman, B. Sarikaya, E. Uhlmann, J. Spanbauer, T. Singewald, et al.
Acute hepatic encephalopathy: diffusion-weighted and fluid-attenuated inversion recovery findings, and correlation with plasma ammonia level and clinical outcome.
AJNR Am J Neuroradiol, 31 (2010), pp. 1471-1479
[6]
S. Toru, K. Matumura, R. Kawaguchi, T. Kobayashi, T. Irie.
Widespread cortical lesions on diffusion-weighted imaging in acute portal systemic shunt encephalopathy caused by primary biliary cirrhosis.
AJNR Am J Neuroradiol, 32 (2011), pp. E55-E56
[7]
J.R. Babington, J.H. Stahl, D.L. Coy.
Reversible cytotoxic edema in a cirrhotic patient following TIPS.
J Neuroimaging, 19 (2009), pp. 391-393
[8]
R.T. Kavitt, V.L. Yang, D.M. Jensen.
Cerebral edema and hyperammonemia after transjugular intrahepatic portosystemic shunt placement in a cirrhotic patient.
Clin Gastroenterol Hepatol, 6 (2008), pp. 1054-1056
[9]
M. Rosario, K. McMahon, P.F. Finelli.
Diffusion-weighted imaging in acute hyperammonemic encephalopathy.
Neurohospitalist, 3 (2013), pp. 125-130
[10]
A.M. McKinney, B. Sarikaya, J. Spanbauer, B.D. Lohman, E. Uhlmannn.
Acute hepatic (or hyperammonemic) encephalopathy: diffuse cortical injury and the significance of ammonia.
AJNR Am J Neuroradiol, 32 (2011), pp. E142
[11]
M. Odeh.
Pathogenesis of hepatic encephalopathy: the tumour necrosis factor-alpha theory.
Eur J Clin Investig, 37 (2007), pp. 291-304

Please cite this article as: Falgàs N, Alfaro I, Crespo G, Berenguer J, García-Pagán JC, Muñoz E. Afasia en paciente con encefalopatía hepática aguda asociada a lesiones corticales cerebrales multifocales. Neurología. 2018;33:410–412.

Copyright © 2016. Sociedad Española de Neurología
Article options
Tools
es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

es en pt
Política de cookies Cookies policy Política de cookies
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí. To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here. Utilizamos cookies próprios e de terceiros para melhorar nossos serviços e mostrar publicidade relacionada às suas preferências, analisando seus hábitos de navegação. Se continuar a navegar, consideramos que aceita o seu uso. Você pode alterar a configuração ou obter mais informações aqui.