Acquired hepatocerebral degeneration: a case report

Listik, Clarice; Machado-Porto, Gislaine Cristina Lopes; Oliveira, Maira Okada de; Porto, Fábio Henrique de Gobbi

doi:10.1590/S1980-57642012DN06010010

ABSTRACT

Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism, ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liver disease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline. Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatments proposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.

Key words:
acquired hepatocerebral degeneration; hepatic encephalopathy; liver disease

RESUMO

Degeneração hepatocerebral adquirida é uma síndrome neurológica subdiagnosticado caracterizada por parkinsonismo, ataxia ou outros distúrbios do movimento e por sintomas neuropsiquiátricos e cognitivos. Ocorre em pacientes com doença hepática crônica, especialmente aqueles que desenvolvem shunts porto-sistêmicos e frequentemente não é reconhecido como uma causa de declínio cognitivo. Recentemente, sua patogênese tem sido associada ao acúmulo de manganês nos gânglios da base e alguns tratamentos foram propostos. O objetivo do artigo foi relatar um caso e discutir algumas descobertas nesta doença.

Palavras-chave:
degeneração hepatocerebral adquirida; encefalopatia hepatica; hepatopatia

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REFERENCES

Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 1995;10:714-722.
Ferrara J, Jankovic J. Acquired hepatocerebral degeneration. J Neurol 2009;256:320-332.
Butterworth RF, Spahr L, Fontaine S, Layrargues GP. Manganese toxicity, dopaminergic dysfunction and hepatic encephalopathy. Metab Brain Dis 1995;10:259-267.
Layrargues GP. Movement dysfunction and hepatic encephalopathy. Metab Brain Dis 2001;16:27-35.
Hauser RA, Zesiewicz TA, Rosemurgy AS, Martinez C, Olanow CW. Manganese intoxication and chronic liver failure. Ann Neurol 1994;36: 871-875.
Child CG, Turcotte JG. Surgery and portal hypertension. In: Child CG, editor. The liver and portal hypertension. Philadelphia, Saunders; 1964: 50-64.
Pugh RNH, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the esophagus in bleeding oesophageal varices. Br J Surg 1973;60:648-652.
Brucki SMD, Nitrini R, Caramelli P, Bertolucci PHF, Okamoto IH. Sugestões para o uso do mini-exame do estado mental no Brasil. Arq Neuropsiquiatr 2003;61:777-781.
Folstein MF, Folstein SE, McHugh PR. Mini-Mental State: a practical method for grading the cognitive state of patients for clinician. J Psychiatr Res 1975;12:189-198.
Mattis S. Dementia Rating Scale. Professional Manual. Florida: Psychological Assessment Resource, Inc; 1988.
Porto CS, Fichman HC, Caramelli P, Bahia VS, Nitrini R. Brazilian version of the Mattis Dementia Rating Scale: Diagnosis of mild dementia in Alzheimer's disease. Arq Neuropsiquiatr 2003;61:339-345.
Spreen O, Strauss E. A Compendium of Neuropsychological Tests. Administration, Norms, and Comentary. Second Edition. Oxford University Press; 1998.
Wechsler D. Wechsler Memory Scale. Manual The Psychological Corporation Harcourt Brace Jovanovich; 1987.
Diniz LFM, Cruz MF, Torres VM, Consenza, RM . Teste de aprendizagem auditivo verbal de Rey: normas para uma população brasileira. Rev Bras Neurol 2000;36:79-83.
Raven JC, Raven J, Courth JH. Manual matrizes progressivas coloridas. São Paulo: Casa do Psicólogo; 1988.
Krieger S, Jauss M, Jansen O, et al. MRI findings in chronic hepatic encephalopathy depend on portosystemic shunt: Results of a controlled prospective clinical investigation. J Hepatol 1997;27:121-126.
Pinarbasi BS, Kalymakoglu Z, Matur F, et al. Are Acquired Hepatocerebral Degeneration and Hepatic Myelopathy Reversible? J Clin Gastroenterol 2009;43:176-181.
Van Woerkem W. La cirrhose hepatique avec alterations dans les centres nerveux evoluant chez des sujets d'age moyen. Nouvelle Iconographie de la Salpetriere, Clinique des Maladies du Systeme Nerveux 1914;7:41-45.
Victor M, Adams RD, Cole M. The acquired (non-Wilsonian) type of chronic hepatocerebral. Medicine 1965;44:345-396.
Bellentani S, Tiribelli C, Saccoccio G, et al. Prevalence of chronic liver disease in the general population of northern Italy: the Dionysos Study. Hepatology 1994;20:1442-1449.
Barros MB, Francisco PM, Zanchetta LM, César CL. [Trends in social and demographic inequalities in the prevalence of chronic diseases in Brazil. PNAD: 2003-2008].Cien Saude Colet 2011;16:3755-3768.
Burkhard PR, Delavelle J, Du Pasquier R, Spahr L. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol 2003;60:521-528.
Fernández-Rodriguez R, Contreras A, De Villoria JG, Grandas F. Acquired hepatocerebral degeneration: clinical characteristics and MRI findings. Eur J Neurol 2010;17:1463-1470.
Stracciari A, Mattarozzi K, D'Alessandro R, Baldin E, Guarino M. Cognitive functioning in chronic acquired hepatocerebral degeneration. Metab Brain Dis 2008;23:155-160.
Pinarbasi B, Kaymakoglu S, Matur Z, et al. Are acquired hepatocerebral degeneration and hepatic myelopathy reversible? J Clin Gastroenterol 2009;43:1761-1781.
Stracciari A, Guarino M, Pazzaglia P, Marchesini G, Pisi P. Acquired hepatocerebral degeneration: full recovery after liver transplantation. J Neurol Neurosurg Psychiatry 2001;70:136-137.
Papapetropoulos S, Tzakis A, Sengun C, et al. Case of pediatric acquired chronic hepatocerebral degeneration. Pediatr Neurol 2008;38:67-70.
Huang CC, Chu NS, Lu CS, Chen RS, Schulzer M, Calne DB. The natural history of neurological manganism over 18 years. Parkinsonism Relat Disord 2007;13:143-145.
Layrargues GP. Movement dysfunction and hepatic encephalopathy. Metab Brain Dis 2001;16:27-35.
Rose C, Butterworth RF, Zayed J, et al. Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction. Gastroenterology 1999;117:640-604.
Rovira A, Alonso J, Cordoba J. MR Imaging findings in hepatic encephalopathy. Am J Neuroradiol 2008;29:1612-1621.
Spahr L, Butterworth RF, Fontaine S, et al. Increased Blood Manganese in Cirrhotic Patients: Relationship to Pallidal Magnetic Resonance Signal Hyperintensity and Neurological Symptoms. Hepatology 1996;24:1116-1120.
Naegele T, Grodd W, Viebahn R, et al. MR Imaging and 1H spectroscopy of brain metabolites in hepatic encephalopathy: time-course of renormalization after liver transplantation. Radiology 2000;216:683-691.
Matsusue E, Kinoshita T, Ohama E, Ogawa T. Cerebral cortical and white matter lesions in chronic hepatic encephalopathy: MR-pathologic correlations. Am J Neuroradiol 2005;26:347-351.
Servin-Abad L, Tzakis A, Schiff ER, Regev A. Acquired hepatocerebral degeneration in a patient with HCV cirrhosis: complete resolution with subsequent recurrence after liver transplantation. Liver Transpl 2006; 12:1161-1165.
Ueki Y, Isozaki E, Miyazaki Y, et al. Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy. Acta Neurol Scand 2002;106:113-116.
Romeiro FG, Américo MF, Yamashiro FS, et al. Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease. Arq Neuropsiquiatr 2011;69:496-501.
Chen WX, Wang P, Yan SX, Li YM, Yu CH, Jiang LL. Acquired hepatocerebral degeneration: a case report. World J Gastroenterol 2005; 11:764-766.
Park HK, Kim SM, Choi CG, Lee MC, Chung SJ. Effect of trientine on manganese intoxication in a patient with acquired hepatocerebral degeneration. Mov Disord 2008;23:768-770.

Publication Dates

Publication in this collection
Jan-Mar 2012

History

Received
20 Dec 2011
Accepted
06 Feb 2012

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 1995;10:714-722.

[2] Ferrara J, Jankovic J. Acquired hepatocerebral degeneration. J Neurol 2009;256:320-332.

[3] Butterworth RF, Spahr L, Fontaine S, Layrargues GP. Manganese toxicity, dopaminergic dysfunction and hepatic encephalopathy. Metab Brain Dis 1995;10:259-267.

[4] Layrargues GP. Movement dysfunction and hepatic encephalopathy. Metab Brain Dis 2001;16:27-35.

[5] Hauser RA, Zesiewicz TA, Rosemurgy AS, Martinez C, Olanow CW. Manganese intoxication and chronic liver failure. Ann Neurol 1994;36: 871-875.

[6] Child CG, Turcotte JG. Surgery and portal hypertension. In: Child CG, editor. The liver and portal hypertension. Philadelphia, Saunders; 1964: 50-64.

[7] Pugh RNH, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the esophagus in bleeding oesophageal varices. Br J Surg 1973;60:648-652.

[8] Brucki SMD, Nitrini R, Caramelli P, Bertolucci PHF, Okamoto IH. Sugestões para o uso do mini-exame do estado mental no Brasil. Arq Neuropsiquiatr 2003;61:777-781.

[9] Folstein MF, Folstein SE, McHugh PR. Mini-Mental State: a practical method for grading the cognitive state of patients for clinician. J Psychiatr Res 1975;12:189-198.

[10] Mattis S. Dementia Rating Scale. Professional Manual. Florida: Psychological Assessment Resource, Inc; 1988.

[11] Porto CS, Fichman HC, Caramelli P, Bahia VS, Nitrini R. Brazilian version of the Mattis Dementia Rating Scale: Diagnosis of mild dementia in Alzheimer's disease. Arq Neuropsiquiatr 2003;61:339-345.

[12] Spreen O, Strauss E. A Compendium of Neuropsychological Tests. Administration, Norms, and Comentary. Second Edition. Oxford University Press; 1998.

[13] Wechsler D. Wechsler Memory Scale. Manual The Psychological Corporation Harcourt Brace Jovanovich; 1987.

[14] Diniz LFM, Cruz MF, Torres VM, Consenza, RM . Teste de aprendizagem auditivo verbal de Rey: normas para uma população brasileira. Rev Bras Neurol 2000;36:79-83.

[15] Raven JC, Raven J, Courth JH. Manual matrizes progressivas coloridas. São Paulo: Casa do Psicólogo; 1988.

[16] Krieger S, Jauss M, Jansen O, et al. MRI findings in chronic hepatic encephalopathy depend on portosystemic shunt: Results of a controlled prospective clinical investigation. J Hepatol 1997;27:121-126.

[17] Pinarbasi BS, Kalymakoglu Z, Matur F, et al. Are Acquired Hepatocerebral Degeneration and Hepatic Myelopathy Reversible? J Clin Gastroenterol 2009;43:176-181.

[18] Van Woerkem W. La cirrhose hepatique avec alterations dans les centres nerveux evoluant chez des sujets d'age moyen. Nouvelle Iconographie de la Salpetriere, Clinique des Maladies du Systeme Nerveux 1914;7:41-45.

[19] Victor M, Adams RD, Cole M. The acquired (non-Wilsonian) type of chronic hepatocerebral. Medicine 1965;44:345-396.

[20] Bellentani S, Tiribelli C, Saccoccio G, et al. Prevalence of chronic liver disease in the general population of northern Italy: the Dionysos Study. Hepatology 1994;20:1442-1449.

[21] Barros MB, Francisco PM, Zanchetta LM, César CL. [Trends in social and demographic inequalities in the prevalence of chronic diseases in Brazil. PNAD: 2003-2008].Cien Saude Colet 2011;16:3755-3768.

[22] Burkhard PR, Delavelle J, Du Pasquier R, Spahr L. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol 2003;60:521-528.

[23] Fernández-Rodriguez R, Contreras A, De Villoria JG, Grandas F. Acquired hepatocerebral degeneration: clinical characteristics and MRI findings. Eur J Neurol 2010;17:1463-1470.

[24] Stracciari A, Mattarozzi K, D'Alessandro R, Baldin E, Guarino M. Cognitive functioning in chronic acquired hepatocerebral degeneration. Metab Brain Dis 2008;23:155-160.

[25] Pinarbasi B, Kaymakoglu S, Matur Z, et al. Are acquired hepatocerebral degeneration and hepatic myelopathy reversible? J Clin Gastroenterol 2009;43:1761-1781.

[26] Stracciari A, Guarino M, Pazzaglia P, Marchesini G, Pisi P. Acquired hepatocerebral degeneration: full recovery after liver transplantation. J Neurol Neurosurg Psychiatry 2001;70:136-137.

[27] Papapetropoulos S, Tzakis A, Sengun C, et al. Case of pediatric acquired chronic hepatocerebral degeneration. Pediatr Neurol 2008;38:67-70.

[28] Huang CC, Chu NS, Lu CS, Chen RS, Schulzer M, Calne DB. The natural history of neurological manganism over 18 years. Parkinsonism Relat Disord 2007;13:143-145.

[29] Layrargues GP. Movement dysfunction and hepatic encephalopathy. Metab Brain Dis 2001;16:27-35.

[30] Rose C, Butterworth RF, Zayed J, et al. Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction. Gastroenterology 1999;117:640-604.

[31] Rovira A, Alonso J, Cordoba J. MR Imaging findings in hepatic encephalopathy. Am J Neuroradiol 2008;29:1612-1621.

[32] Spahr L, Butterworth RF, Fontaine S, et al. Increased Blood Manganese in Cirrhotic Patients: Relationship to Pallidal Magnetic Resonance Signal Hyperintensity and Neurological Symptoms. Hepatology 1996;24:1116-1120.

[33] Naegele T, Grodd W, Viebahn R, et al. MR Imaging and 1H spectroscopy of brain metabolites in hepatic encephalopathy: time-course of renormalization after liver transplantation. Radiology 2000;216:683-691.

[34] Matsusue E, Kinoshita T, Ohama E, Ogawa T. Cerebral cortical and white matter lesions in chronic hepatic encephalopathy: MR-pathologic correlations. Am J Neuroradiol 2005;26:347-351.

[35] Servin-Abad L, Tzakis A, Schiff ER, Regev A. Acquired hepatocerebral degeneration in a patient with HCV cirrhosis: complete resolution with subsequent recurrence after liver transplantation. Liver Transpl 2006; 12:1161-1165.

[36] Ueki Y, Isozaki E, Miyazaki Y, et al. Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy. Acta Neurol Scand 2002;106:113-116.

[37] Romeiro FG, Américo MF, Yamashiro FS, et al. Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease. Arq Neuropsiquiatr 2011;69:496-501.

[38] Chen WX, Wang P, Yan SX, Li YM, Yu CH, Jiang LL. Acquired hepatocerebral degeneration: a case report. World J Gastroenterol 2005; 11:764-766.

[39] Park HK, Kim SM, Choi CG, Lee MC, Chung SJ. Effect of trientine on manganese intoxication in a patient with acquired hepatocerebral degeneration. Mov Disord 2008;23:768-770.

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