Skip to main content

Advertisement

SpringerLink
Log in

Acquired hepatocerebral degeneration

  • REVIEW
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Cirrhosis and its co-morbidities may cause a variety of neurological complications, the most common being bouts of toxic metabolic encephalopathy. A proportion of patients with chronic liver disease develop acquired hepatocerebral degeneration (AHD), a chronic progressive neurological syndrome characterized by parkinsonism, ataxia and other movement disorders. This article reviews the clinical spectrum, pathophysiology, neuroimaging features and differential diagnosis of AHD along with emerging treatment options.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Abe T, Kojima K, Shoji H, Tanaka N, Fujimoto K, Uchida M, Nishimura H, Hayabuchi N, Norbash AM (1998) Japanese encephalitis. J Magn Reson Imaging 8:755–761

    Article  PubMed  CAS  Google Scholar 

  2. Aggarwal A, Vaidya S, Shah S, Singh J, Desai S, Bhatt M (2006) Reversible Parkinsonism and T1W pallidal hyperintensities in acute liver failure. Mov Disord 21:1986–1990

    Article  PubMed  Google Scholar 

  3. Ahn JH, Yoo CI, Lee CR, Lee JH, Lee H, Kim CY, Park JK, Sakai T, Yoon CS, Kim Y (2003) Calcification mimicking manganese-induced increased signal intensities in T1-weighted MR images in a patient taking herbal medicine: case report. Neurotoxicology 24:835–838

    Article  PubMed  Google Scholar 

  4. American Gastroenterological Association (2001) The Burden of Gastrointestinal Diseases. at: www.gastro.org/ user-assets/Documents/burden-report. pdf

  5. Aschner M, Guilarte TR, Schneider JS, Zheng W (2007) Manganese: recent advances in understanding its transport and neurotoxicity. Toxicol Appl Pharmacol 221:131–147

    Article  PubMed  CAS  Google Scholar 

  6. Bang SJ, Choi SH, Park NH, Shin JW, Choy N, Kim SH, Kim Y (2007) High pallidal T1 signal is rarely observed in obstructive jaundice, but is frequently observed in liver cirrhosis. J Occup Health 49:268–272

    Article  PubMed  Google Scholar 

  7. Blanusa M, Varnai VM, Piasek M, Kostial K (2005) Chelators as antidotes of metal toxicity: therapeutic and experimental aspects. Curr Med Chem 12:2771–2794

    Article  PubMed  CAS  Google Scholar 

  8. Burkhard PR, Delavelle J, Du Pasquier R, Spahr L (2003) Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol 60:521–528

    Article  PubMed  Google Scholar 

  9. Callaghan N, McCoy DG (1967) Acquired chronic hepatocerebral degeneration with splenic vein occlusion. J Ir Med Assoc 60:118–120

    PubMed  CAS  Google Scholar 

  10. Charness ME (1993) Brain lesions in alcoholics. Alcohol Clin Exp Res 17:2–11

    Article  PubMed  CAS  Google Scholar 

  11. Choi Y, Park JK, Park NH, Shin JW, Yoo CI, Lee CR, Lee H, Kim HK, Kim SR, Jung TH, Park J, Yoon CS, Kim Y (2005) Whole blood and red blood cell manganese reflected signal intensities of T1-weighted magnetic resonance images better than plasma manganese in liver cirrhotics. J Occup Health 47:68–73

    Article  PubMed  CAS  Google Scholar 

  12. Chu K, Kang DW, Kim DE, Park SH, Roh JK (2002) Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia. Arch Neurol 59:448–452

    Article  PubMed  Google Scholar 

  13. Condat B, Dusoleil A, Bernardeau M, Roche A, Pelletier G, Buffet C (1999) Chronic acquired hepatocerebral degeneration: the role of manganese and treatment by endovascular occlusion of a porto-systemic shunt. Gastroenterol Clin Biol 23:268–270

    PubMed  CAS  Google Scholar 

  14. Conn HO, Rossle M, Levy L, Glocker FX (2006) Portosystemic myelopathy: spastic paraparesis after portosystemic shunting. Scand J Gastroenterol 41:619–625

    Article  PubMed  Google Scholar 

  15. Cook DG, Fahn S, Brait KA (1974) Chronic manganese intoxication. Arch Neurol 30:59–64

    PubMed  CAS  Google Scholar 

  16. Coskun A, Yikilmaz A, Kumandas S, Karahan OI, Akcakus M, Manav A (2005) Hyperintense globus pallidus on T1-weighted MR imaging in acute kernicterus: is it common or rare? Eur Radiol 15:1263–1267

    Article  PubMed  Google Scholar 

  17. Cotzias GC, Horiuchi K, Fuenzalida S, Mena I (1968) Chronic Manganese poisoning: clearance of tissue manganese concentrations with persistence of the neurological picture. Neurology 18:376–382

    PubMed  CAS  Google Scholar 

  18. Cotzias GC (1969) Metabolic modification of some neurologic disorders. JAMA 210:1255–1262

    Article  PubMed  CAS  Google Scholar 

  19. Counsell C, Warlow C (1996) Failure of presumed hepatic myelopathy to improve after liver transplantation. J Neurol Neurosurg Psychiatry 60:590

    Article  PubMed  CAS  Google Scholar 

  20. Crossgrove J, Zheng W (2004) Manganese toxicity upon overexposure. NMR Biomed 17:544–553

    Article  PubMed  CAS  Google Scholar 

  21. de Bie RM, Gladstone RM, Strafella AP, Ko JH, Lang AE (2007) Manganeseinduced Parkinsonism associated with methcathinone (Ephedrone) abuse. Arch Neurol 64:886–889

    Article  PubMed  Google Scholar 

  22. Demaerel P, Van Gool S (2008) Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology 50:85–92

    Article  PubMed  Google Scholar 

  23. Demarquay G, Setiey A, Morel Y, Trepo C, Chazot G, Broussolle E (2000) Clinical report of three patients with hereditary hemochromatosis and movement disorders. Mov Disord 15:1204–1209

    Article  PubMed  CAS  Google Scholar 

  24. Dobson AW, Erikson KM, Aschner M (2004) Manganese neurotoxicity. Ann N Y Acad Sci 1012:115–128

    Article  PubMed  CAS  Google Scholar 

  25. Drayer BP, Fram EK, Bird CR, Williams KD, Keller PJ (1989) Systemic metabolic disease and the globus pallidus: An MRI approach [abstract]. AJNR Am J Neuroradiol 10:902

    Google Scholar 

  26. Dringen R, Bishop GM, Koeppe M, Dang TN, Robinson SR (2007) The pivotal role of astrocytes in the metabolism of iron in the brain. Neurochem Res 32:1884–1890

    Article  PubMed  CAS  Google Scholar 

  27. Erikson KM, Thompson K, Aschner J, Aschner M (2007) Manganese neurotoxicity: a focus on the neonate. Pharmacol Ther 113:369–377

    Article  PubMed  CAS  Google Scholar 

  28. Fahn S (2007) The Tardive Syndromes: Phenomenology, Concepts on Pathophysiology and Treatment, and Other Neruoleptic-Induced Syndromes. In: Fahn S, Jankovic J (eds) The Principles and Practice of Movement Disorders. 1st ed. Philadelphia, PA: Churchill Livingstone, pp 479–518

  29. Ferrara J, Gupta D, Foster E, Garman K, Stacy M (2008) Extraocular muscle dystonia due to acquired (non-Wilsonian) hepatocerebral degeneration. Mov Disord 23:875–878

    Article  PubMed  Google Scholar 

  30. Finlayson MH, Superville B (1981) Distribution of cerebral lesions in acquired hepatocerebral degeneration. Brain 104:79–95

    Article  PubMed  CAS  Google Scholar 

  31. Finley JW, Davis CD (1999) Manganese deficiency and toxicity: are high or low dietary amounts of manganese cause for concern? Biofactors 10:15–24

    Article  PubMed  CAS  Google Scholar 

  32. Fitsanakis VA, Piccola G, Marreilha dos Santos AP, Aschner JL, Aschner M (2007) Putative proteins involved in manganese transport across the bloodbrain barrier. Hum Exp Toxicol 26:295–302

    Article  PubMed  CAS  Google Scholar 

  33. Fukuzawa T, Matsutani S, Maruyama H, Akiike T, Saisho H, Hattori T (2006) Magnetic resonance images of the globus pallidus in patients with idiopathic portal hypertension: a quantitative analysis of the relationship between signal intensity and the grade of portosystemic shunt. J Gastroenterol Hepatol 21:902–907

    Article  PubMed  Google Scholar 

  34. Galluzzi P, Rufa A, Balestri P, Cerase A, Federico A (2001) MR brain imaging of fucosidosis type I. AJNR Am J Neuroradiol 22:777–780

    PubMed  CAS  Google Scholar 

  35. Gershanik O (2004) Drug-induced dyskinesias. In: Jankovic J, Tolosa E (eds) Parkinson’s Disease and Movement Disorders. 4rd ed. Baltimore, MD: Lippincott Williams & Wilkins, pp 365–379

  36. Graham DI, Adams JH, Caird FI, Lawson JW (1970) Acquired hepatocerebral degeneration: report of an atypical case. J Neurol Neurosurg Psychiatry 33:656–662

    Article  PubMed  CAS  Google Scholar 

  37. Grossman RI, Yousem DM (2003) Neurodegenerative diseases and hydrocephalus. In: Neuroradiology: The Requisites. 2nd ed. St. Louis, Mosby, pp 391–396

  38. Guilarte TR, Chen MK, McGlothan JL, Verina T, Wong DF, Zhou Y, Alexander M, Rohde CA, Syversen T, Decamp E, Koser AJ, Fritz S, Gonczi H, Anderson DW, Schneider JS (2006) Nigrostriatal dopamine system dysfunction and subtle motor deficits in manganeseexposed non-human primates. Exp Neurol 202:381–390

    Article  PubMed  CAS  Google Scholar 

  39. Gunter TE, Gavin CE, Aschner M, Gunter KK (2006) Speciation of manganese in cells and mitochondria: a search for the proximal cause of manganese neurotoxicity. Neurotoxicology 27:765–776

    Article  PubMed  CAS  Google Scholar 

  40. Hanner JS, Li KC, Davis GL (1988) Acquired hepatocerebral degeneration: MR similarity with Wilson disease. J Comput Assist Tomogr 12:1076–1077

    Article  PubMed  CAS  Google Scholar 

  41. Hauser RA, Zesiewicz TA, Rosemurgy AS, Martinez C, Olanow CW (1994) Manganese intoxication and chronic liver failure. Ann Neurol 36:871–875

    Article  PubMed  CAS  Google Scholar 

  42. Hazell AS, Normandin L, Norenberg MD, Kennedy G, Yi JH (2006) Alzheimer type II astrocytic changes following sub-acute exposure to manganese and its prevention by antioxidant treatment. Neurosci Lett 396:167–171

    Article  PubMed  CAS  Google Scholar 

  43. Herrero Hernandez E, Valentini MC, Discalzi G (2002) T1-weighted hyperintensity in basal ganglia at brain magnetic resonance imaging: are different pathologies sharing a common mechanism? Neurotoxicology 23:669–674

    Article  PubMed  Google Scholar 

  44. Huang BY, Castillo M (2008) Hypoxicischemic brain injury: imaging findings from birth to adulthood. Radiographics 28:417–439

    Article  PubMed  Google Scholar 

  45. Huang CC, Chu NS, Lu CS, Chen RS, Calne DB (1998) Long-term progression in chronic manganism: ten years of follow-up. Neurology 50:698–700

    PubMed  CAS  Google Scholar 

  46. Huang CC, Chu NS, Lu CS, Chen RS, Schulzer M, Calne DB (2007) The natural history of neurological manganism over 18 years. Parkinsonism Relat Disord 13:143–145

    Article  PubMed  Google Scholar 

  47. Huang CC, Chu NS, Lu CS, Wang JD, Tsai JL, Tzeng JL, Wolters EC, Calne DB (1989) Chronic manganese intoxication. Arch Neurol 46:1104–1106

    PubMed  CAS  Google Scholar 

  48. Huang CC, Lu CS, Chu NS, Hochberg F, Lilienfeld D, Olanow W, Calne DB (1993) Progression after chronic manganese exposure. Neurology 43:1479–1483

    PubMed  CAS  Google Scholar 

  49. Ihara H (1998) High signal intensity of globus pallidus on T1-weighted MRI in liver cirrhosis patients: clinical and pathological study. Nippon Igaku Hoshasen Gakkai Zasshi 58:479–488

    PubMed  CAS  Google Scholar 

  50. Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, Suzuki K, Lynch DR, Suzuki N, Hata T, Dalmau J (2008) Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology 70:504–511

    Article  PubMed  CAS  Google Scholar 

  51. Ikeda S, Yamaguchi Y, Sera Y, Ohshiro H, Uchino S, Yamashita Y, Ogawa M (2000) Manganese deposition in the globus pallidus in patients with biliary atresia. Transplantation 69:2339–2343

    Article  PubMed  CAS  Google Scholar 

  52. Inose T (1952) Hepatocerebral degeneration, a special type. J neuropath Exp Neurol 11:401–408

    Article  PubMed  CAS  Google Scholar 

  53. Inoue E, Hori S, Narumi Y, Fujita M, Kuriyama K, Kadota T, Kuroda C (1991) Portal-systemic encephalopathy: presence of basal ganglia lesions with high signal intensity on MR images. Radiology 179:551–555

    PubMed  CAS  Google Scholar 

  54. Jankovic J (2005) Searching for a relationship between manganese and welding and Parkinson’s disease. Neurology 64:2021–2028

    Article  PubMed  CAS  Google Scholar 

  55. Jog MS, Lang AE (1995) Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 10:714–722

    Article  PubMed  CAS  Google Scholar 

  56. Jover R, Compañy L, Gutiérrez A, Lorente M, Zapater P, Poveda MJ, Such J, Pascual S, Palazón JM, Carnicer F, Ferrandis F, Pérez-Mateo M (2005) Clinical significance of extrapyramidal signs in patients with cirrhosis. J Hepatol 42:659–665

    Article  PubMed  Google Scholar 

  57. Kalia K, Jiang W, Zheng W (2008) Manganese accumulates primarily in nuclei of cultured brain cells. Neurotoxicology 29:466–470

    Article  PubMed  CAS  Google Scholar 

  58. Kalita J, Misra UK (2000) Comparison of CT scan and MRI findings in the diagnosis of Japanese encephalitis. J Neurol Sci 174:3–8

    Article  PubMed  CAS  Google Scholar 

  59. Katsuragi T, Iseki E, Kosaka K, Koyano S, Iwabuchi K (1999) Cerebrospinal fluid manganese concentrations in patients with symmetric pallidal hyperintensities on T1 weighted MRI. J Neurol Neurosurg Psychiatry 66:551–552

    Article  PubMed  CAS  Google Scholar 

  60. Kenney C, Hunter C, Jankovic J (2007) Long-term tolerability of tetrabenazine in the treatment of hyperkinetic movement disorders. Mov Disord 22:193–197

    Article  PubMed  Google Scholar 

  61. Kenney C, Jankovic J (2006) Tetrabenazine in the treatment of hyperkinetic movement disorders. Expert Rev Neurother 6:7–17

    Article  PubMed  CAS  Google Scholar 

  62. Kessler KR, Wunderlich G, Hefter H, Seitz RJ (2003) Secondary progressive chronic manganism associated with markedly decreased striatal D2 receptor density. Mov Disord 18:217–218

    Article  PubMed  Google Scholar 

  63. Khanna S, Gopalan S (2007) Role of branched-chain amino acids in liver disease: the evidence for and against. Curr Opin Clin Nutr Metab Care 10:297–303

    Article  PubMed  CAS  Google Scholar 

  64. Kim J, Kim JM, Kim YK, Shin JW, Choi SH, Kim SE, Kim Y (2007)Dopamine transporter SPECT of a liver cirrhotic with atypical parkinsonism. Ind Health 45:497–500

    Article  PubMed  Google Scholar 

  65. Kim Y, Park JK, Choi Y, Yoo CI, Lee CR, Lee H, Lee JH, Kim SR, Jeong TH, Yoon CS, Park JH (2005) Blood manganese concentration is elevated in iron deficiency anemia patients, whereas globus pallidus signal intensity is minimally affected. Neurotoxicology 26:107–111

    Article  PubMed  CAS  Google Scholar 

  66. Kleinschmidt-DeMasters BK, Filley CM, Rojiani AM (2006) Overlapping features of extrapontine myelinolysis and acquired chronic (non-Wilsonian) hepatocerebral degeneration. Acta Neuropathol (Berl) 112:605–616

    Article  PubMed  CAS  Google Scholar 

  67. Klos KJ, Ahlskog JE, Josephs KA, Fealey RD, Cowl CT, Kumar N (2005) Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: probable manganese neurotoxicity. Arch Neurol 62:1385–1390

    Article  PubMed  Google Scholar 

  68. Klos KJ, Ahlskog JE, Kumar N, Cambern S, Butz J, Burritt M, Fealey RD, Cowl CT, Parisi JE, Josephs KA (2006) Brain metal concentrations in chronic liver failure patients with pallidal T1 MRI hyperintensity. Neurology 67:1984–1989

    Article  PubMed  CAS  Google Scholar 

  69. Krieger D, Krieger S, Jansen O, Gass P, Theilmann L, Lichtnecker H (1995) Manganese and chronic encephalopathy. Lancet 346:270–274

    Article  PubMed  CAS  Google Scholar 

  70. Krieger S, Jauss M, Jansen O, Stiehl A, Sauer P, Geissler M, Theilmann L, Krieger D (1997) MRI findings in chronic hepatic encephalopathy depend on portosystemic shunt: results of a controlled prospective clinical investigation. J Hepatol 27:121–126

    Article  PubMed  CAS  Google Scholar 

  71. Krieger S, Jauss M, Jansen O, Theilmann L, Geissler M, Krieger D (1996) Neuropsychiatric profile and hyperintense globus pallidus on T1-weighted magnetic resonance images in liver cirrhosis. Gastroenterology 111:147–155

    Article  PubMed  CAS  Google Scholar 

  72. Kril JJ, Butterworth RF (1997) Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease. Hepatology 26:837–841

    Article  PubMed  CAS  Google Scholar 

  73. Kulisevsky J, Pujol J, Balanzó J, Junqué C, Deus J, Capdevilla A, Villanueva C (1992) Pallidal hyperintensity on magnetic resonance imaging in cirrhotic patients: clinical correlations. Hepatology 16:1382–1388

    Article  PubMed  CAS  Google Scholar 

  74. Kumar S, Fowler M, Gonzalez-Toledo E, Jaffe SL (2006) Central pontine myelinolysis, an update. Neurol Res 28:360–366

    Article  PubMed  Google Scholar 

  75. Kurian MA, Morgan NV, MacPherson L, Foster K, Peake D, Gupta R, Philip SG, Hendriksz C, Morton JE, Kingston HM, Rosser EM, Wassmer E, Gissen P, Maher ER (2008) Phenotypic spectrum of neurodegeneration associated with mutations in the PLA2G6 gene (PLAN). Neurology 70:1623–1629

    Article  PubMed  CAS  Google Scholar 

  76. Kurlan R, Shoulson I (1988) Differential diagnosis of facial chorea. Adv Neurol 49:225–237

    PubMed  CAS  Google Scholar 

  77. Lazeyras F, Spahr L, DuPasquier R, Delavelle J, Burkhard P, Hadengue A, Hochstrasser D, Mentha G, Giostra E, Terrier F, Vingerhoets F (2002) Persistence of mild parkinsonism 4 months after liver transplantation in patients with preoperative minimal hepatic encephalopathy: a study on neuroradiological and blood manganese changes. Transpl Int 15:188–195

    Article  PubMed  Google Scholar 

  78. Lee J, Lacomis D, Comu S, Jacobsohn J, Kanal E (1998) Acquired hepatocerebral degeneration: MR and pathologic findings. AJNR Am J Neuroradiol 19:485–487

    PubMed  CAS  Google Scholar 

  79. Levy LM, Yang A, Kumar AJ, Hennigar R, Rothstein J, Bryan RN (1989) The brain and hepatic encephalopathy MR abnormalities (abstract). AJNR Am J Neuroradiol 10:904

    Google Scholar 

  80. Lewis MB, MacQuillan G, Bamford JM, Howdle PD (2000) Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome. Neurology 54:1011

    PubMed  CAS  Google Scholar 

  81. Lo CP, Chen SY, Lee KW, Chen WL, Chen CY, Hsueh CJ, Huang GS (2007) Brain injury after acute carbon monoxide poisoning: early and late complications. AJR Am J Roentgenol 189:W205–W211

    Article  PubMed  Google Scholar 

  82. Lu CS, Huang CC, Chu NS, Caine DB (1994) Levodopa failure in chronic manganism. Neurology 44:1600–1602

    PubMed  CAS  Google Scholar 

  83. Lunzer M, James IM, Weinman J, Sherlock S (1974) Treatment of chronic hepatic encephalopathy with levodopa. Gut 15:555–561

    Article  PubMed  CAS  Google Scholar 

  84. Maeda H, Sato M, Yoshikawa A, Kimura M, Sonomura T, Terada M, Kishi K (1997) Brain MR imaging in patients with hepatic cirrhosis: relationship between high intensity signal in basal ganglia on T1-weighted images and elemental concentrations in brain. Neuroradiology 39:546–550

    Article  PubMed  CAS  Google Scholar 

  85. Matsumoto S, Mori H, Yoshioka K, Kiyosue H, Komatsu E (1997) Effects of portal-systemic shunt embolization on the basal ganglia: MRI. Neuroradiology 39:326–328

    Article  PubMed  CAS  Google Scholar 

  86. Mattarozzi K, Stracciari A, Vignatelli L, D’Alessandro R, Morelli MC, Guarino M (2004) Minimal hepatic encephalopathy: longitudinal effects of liver transplantation. Arch Neurol 61:242–247

    Article  PubMed  Google Scholar 

  87. McNeill A, Birchall D, Hayflick SJ, Gregory A, Schenk JF, Zimmerman EA, Shang H, Miyajima H, Chinnery PF (2008) T2 and FSE MRI distinguishes four subtypes of neurodegeneration with brain iron accumulation. Neurology 70:1614–1619

    Article  PubMed  CAS  Google Scholar 

  88. Meltzer CC, Wells SW, Becher MW, Flanigan KM, Oyler GA, Lee RR (1998) AIDS-related MR hyperintensity of the basal ganglia. AJNR Am J Neuroradiol 19:83–89

    PubMed  CAS  Google Scholar 

  89. Mena I, Court J, Fuenzalida S, Papavasiliou PS, Cotzias GC (1970) Modification of chronic manganese poisoning. Treatment with L-dopa or 5-0H tryptophane. N Engl J Med 282:5–10

    Article  PubMed  CAS  Google Scholar 

  90. Mena I, Horiuchi K, Burke K, Cotzias GC (1969) Chronic manganese poisoning. Individual susceptibility and absorption of iron. Neurology 19:1000–1006

    PubMed  CAS  Google Scholar 

  91. Mena I, Marin O, Fuenzalida S, Cotzias GC (1967) Chronic manganese poisoning: clinical picture and manganese turnover. Neurology 17:128–136

    PubMed  CAS  Google Scholar 

  92. Mentzel HJ, Seidel J, Fitzek C, Eichhorn A, Vogt S, Reichenbach JR, Zintl F, Kaiser WA (2005) Pediatric brain MRI in neurofibromatosis type I. Eur Radiol 15:814–822

    Article  PubMed  Google Scholar 

  93. Mizoguchi N, Nishimura Y, Ono H, Sakura N (2001) Manganese elevations in blood of children with congenital portosystemic shunts. Eur J Pediatr 160:247–250

    Article  PubMed  CAS  Google Scholar 

  94. Morgan MY, Jakobovits AW, James IM, Sherlock S (1980) Successful use of bromocriptine in the treatment of chronic hepatic encephalopathy. Gastroenterology 78:663–670

    PubMed  CAS  Google Scholar 

  95. Morgan MY (1998) Cerebral magnetic resonance imaging in patients with chronic liver disease. Metab Brain Dis 13:273–290

    Article  PubMed  CAS  Google Scholar 

  96. Naegele T, Grodd W, Viebahn R, Seeger U, Klose U, Seitz D, Kaiser S, Mader I, Mayer J, Lauchart W, Gregor M, Voigt K (2000) MR imaging and (1)H spectroscopy of brain metabolites in hepatic encephalopathy: time-course of renormalization after liver transplantation. Radiology 216:683–691

    PubMed  CAS  Google Scholar 

  97. Nagatomo S, Umehara F, Hanada K, Nobuhara Y, Takenaga S, Arimura K, Osame M (1999) Manganese intoxication during total parenteral nutrition: report of two cases and review of the literature. Neurol Sci 162:102–105

    Article  CAS  Google Scholar 

  98. Nardone R, Buratti T, Oliviero A, Lochmann A, Tezzon F (2006) Corticospinal involvement in patients with a portosystemic shunt due to liver cirrhosis: a MEP study. J Neurol 253:81–85

    Article  PubMed  Google Scholar 

  99. Newland MC, Ceckler TL, Kordower JH, Weiss B (1989) Visualizing manganese in the primate basal ganglia with magnetic resonance imaging. Exp Neurol 106:251–258

    Article  PubMed  CAS  Google Scholar 

  100. Nolte W, Wiltfang J, Schindler CG, Unterberg K, Finkenstaedt M, Niedmann PD, Hartmann H, Ramadori G (1998) Bright basal ganglia in T1- weighted magnetic resonance images are frequent in patients with portal vein thrombosis without liver cirrhosis and not suggestive of hepatic encephalopathy. J Hepatol 29:443–449

    Article  PubMed  CAS  Google Scholar 

  101. Normandin L, Hazell AS (2002) Manganese neurotoxicity: an update of pathophysiologic mechanisms. Metab Brain Dis 17:375–387

    Article  PubMed  CAS  Google Scholar 

  102. Oh SH, Lee KY, Im JH, Lee MS (2002) Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. J Neurol Sci 200:57–62

    Article  PubMed  Google Scholar 

  103. Olanow CW, Good PF, Shinotoh H, Hewitt KA, Vingerhoets F, Snow BJ, Beal MF, Calne DB, Perl DP (1996) Manganese intoxication in the rhesus monkey: a clinical, imaging, pathologic, and biochemical study. Neurology 46:492–498

    PubMed  CAS  Google Scholar 

  104. Oner AY, Cansu A, Akpek S, Serdaroglu A (2007) Fucosidosis: MRI and MRS findings. Pediatr Radiol 37:1050–1052

    Article  PubMed  Google Scholar 

  105. Page RA, Davie CA, MacManus D, Miszkiel KA, Walshe JM, Miller DH, Lees AJ, Schapira AH (2004) Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease. Neurology 63:638–643

    PubMed  CAS  Google Scholar 

  106. Pal PK, Samii A, Calne DB (1999) Manganese neurotoxicity: a review of clinical features, imaging and pathology. Neurotoxicology 20:227–238

    PubMed  CAS  Google Scholar 

  107. Papapetropoulos S, Singer C (2005) Management of the extrapyramidal syndrome in chronic acquired hepatocerebral degeneration (CAHD). Mov Disord 20:1088–1089

    Article  PubMed  Google Scholar 

  108. Papapetropoulos S, Tzakis A, Sengun C, Reddy C, Boukas K, Zitser J, Singer C (2008) Case of pediatric acquired chronic hepatocerebral degeneration. Pediatr Neurol 38:67–70

    Article  PubMed  Google Scholar 

  109. Park HK, Kim SM, Choi CG, Lee MC, Chung SJ (2008) Effect of trientine on manganese intoxication in a patient with acquired hepatocerebral degeneration. Mov Disord 23:768–770

    Article  PubMed  Google Scholar 

  110. Parkes JD, Murray-Lyon IM, Williams R (1970) Neuropsychiatric and electro- encephalographic changes after transplantation of the liver. Q J Med 39:515–527

    PubMed  CAS  Google Scholar 

  111. Pennington JA, Schoen SA (1996) Contributions of food groups to estimated intakes of nutritional elements: results from the FDA total diet studies, 1982–1991. Int J Vitam Nutr Res 66:342–349

    PubMed  CAS  Google Scholar 

  112. Pennington JA, Young BE, Wilson DB, Johnson RD, Vanderveen JE (1986) Mineral content of foods and total diets: the Selected Minerals in Foods Survey, 1982 to 1984. J Am Diet Assoc 86:876–891

    PubMed  CAS  Google Scholar 

  113. Perl DP, Olanow CW (2007) The neuropathology of manganese-induced Parkinsonism. J Neuropathol Exp Neurol 66:675–682

    Article  PubMed  CAS  Google Scholar 

  114. Pinarbasi B, Kaymakoglu S, Matur Z, Akyuz F, Demir K, Besisik F, Ozdil S, Boztas G, Cakaloglu Y, Mungan Z, Okten A (2008) Are acquired hepatocerebral degeneration and hepatic myelopathy reversible? J Clin Gastroenterol Aug 11 [Epub ahead of print]

  115. Pomier-Layrargues G, Spahr L, Butterworth RF (1995) Increased manganese concentrations in pallidum of cirrhotic patients. Lancet 345:735

    Article  PubMed  CAS  Google Scholar 

  116. Pomier-Layrargues GP, Shapcott D, Spahr L, Butterworth RF (1995) Accumulation of manganese and copper in pallidum of cirrhotic patients: role in the pathogenesis of hepatic encephalopathy? Metab Brain Dis 10:353–356

    Article  Google Scholar 

  117. Pomier-Layrargues GP (2001) Movement dysfunction and hepatic encephalopathy. Metab Brain Dis 16:27–35

    Article  Google Scholar 

  118. Powell EE, Pender MP, Chalk JB, Parkin PJ, Strong R, Lynch S, Kerlin P, Cooksley WG, Cheng W, Powell LW (1990) Improvement in chronic hepatocerebral degeneration following liver transplantation. Gastroenterology 98:1079–1082

    PubMed  CAS  Google Scholar 

  119. Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ (2004) Magnetic resonance imaging presentation of (intra) cranial disease associated with Langerhans cell histiocytosis. Am J Neuroradiol 25:880–891

    PubMed  Google Scholar 

  120. Provenzale JM, Barboriak DP, Sims K (1995) Neuroradiologic findings in fucosidosis, a rare lysosomal storage disease. AJNR Am J Neuroradiol 16 (4 Suppl):809–813

    PubMed  CAS  Google Scholar 

  121. Pujol A, Pujol J, Graus F, Rimola A, Peri J, Mercader JM, García-Pagan JC, Bosch J, Rodés J, Tolosa E (1993) Hyperintense globus pallidus on T1- weighted MRI in cirrhotic patients is associated with severity of liver failure. Neurology 43:65–69

    PubMed  CAS  Google Scholar 

  122. Read AE, Sherlock S, Laidlaw J, Walker JG (1967) The neuro-psychiatric syndromes associated with chronic liver disease and an extensive portal-systemic collateral circulation. Q J Med 36:135–150

    Google Scholar 

  123. Rodier J (1955) Manganese poisoning in Moroccan miners. Br J Indust Med 12:21–35

    CAS  Google Scholar 

  124. Rose C, Butterworth RF, Zayed J, Normandin L, Todd K, Michalak A, Spahr L, Huet PM, Pomier-Layrargues G (1999) Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction. Gastroenterology 117:640–644

    Article  PubMed  CAS  Google Scholar 

  125. Rosenstock HA, Simons DG, Meyer JS (1971) Chronic manganism: neurologic and laboratory studies during treatment with levodopa. JAMA 217:1354–1358

    Article  PubMed  CAS  Google Scholar 

  126. Russo N, Edwards M, Andrews T, O’Brien M, Bhatia KP (2004) Hereditary haemochromatosis is unlikely to cause movement disorders – a critical review. J Neurol 251:849–852

    Article  PubMed  Google Scholar 

  127. Saporta MA, André C, Bahia PR, Chimelli L, Pais PH, Silva TS, Correia AH, Nobre AB (2004) Acquired hepatocerebral degeneration without overt liver disease. Neurology 63:1981–1982

    PubMed  CAS  Google Scholar 

  128. Sarmiento de la Iglesia MM, Lecumberri Cortés G, Lecumberri Cortés I, Oleaga Zufiria L, Isusi Fontan M, Grande Icaran D (2006) Intracranial calcifications on MRI. Radiologia 48:19–26

    Article  Google Scholar 

  129. Schmidt S, Gudinchet F, Meagher- Villemure K, Maeder P (2001) Brain involvement in haemolytic-uraemic syndrome: MRI features of coagulative necrosis. Neuroradiology 43:581–585

    Article  PubMed  CAS  Google Scholar 

  130. Sener RN (2003) Diffusion MR Imaging Changes Associated with Wilson Disease. AJNR Am. J. Neuroradiol 24:965–967

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  132. Shinotoh H, Snow BJ, Chu NS, Huang CC, Lu CS, Lee C, Takahashi H, Calne DB (1997) Presynaptic and postsynaptic striatal dopaminergic function in patients with manganese intoxication: a positron emission tomography study. Neurology 48:1053–1056

    PubMed  CAS  Google Scholar 

  133. Shinotoh H, Snow BJ, Hewitt KA, Pate BD, Doudet D, Nugent R, Perl DP, Olanow W, Calne DB (1995) MRI and PET studies of manganese-intoxicated monkeys. Neurology 45:1199–1204

    PubMed  CAS  Google Scholar 

  134. Shulman LM, Minagar A, Weiner WJ (2003) Reversal of parkinsonism following liver transplantation. Neurology 60:519

    PubMed  Google Scholar 

  135. Silverdale MA, Schneider SA, Bhatia KP, Lang AE (2008) The spectrum of orolingual tremor – A proposed classification system. Mov Disord 23:159–167

    Article  PubMed  Google Scholar 

  136. Sinha S, Taly AB, Prashanth LK, Ravishankar S, Arunodaya GR, Vasudev MK (2007) Sequential MRI changes in Wilson’s disease with de-coppering therapy: a study of 50 patients. Br J Radiol 80:744–749

    Article  PubMed  CAS  Google Scholar 

  137. Soffer D, Sherman Y, Tur-Kaspa R, Eid A (1995) Acquired hepatocerebral degeneration in a liver transplant recipient. Acta Neuropathol (Berl) 90:107–111

    Article  PubMed  CAS  Google Scholar 

  138. Spahr L, Butterworth RF, Fontaine S, Bui L, Therrien G, Milette PC, Lebrun LH, Zayed J, Leblanc A, Pomier- Layrargues G (1996) Increased blood manganese in cirrhotic patients: relationship to pallidal magnetic resonance signal hyperintensity and neurological symptoms. Hepatology 24:1116–1120

    Article  PubMed  CAS  Google Scholar 

  139. Spahr L, Vingerhoets F, Lazeyras F, Delavelle J, DuPasquier R, Giostra E, Mentha G, Terrier F, Hadengue A (2000) Magnetic resonance imaging and proton spectroscopic alterations correlate with parkinsonian signs in patients with cirrhosis. Gastroenterology 119:774–781

    Article  PubMed  CAS  Google Scholar 

  140. Steinborn M, Leiz S, Rüdisser K, Griebel M, Harder T, Hahn H (2004) CT and MRI in haemolytic uraemic syndrome with central nervous system involvement: distribution of lesions and prognostic value of imaging findings. Pediatr Radiol 34:805–810

    Article  PubMed  Google Scholar 

  141. Stepens A, Logina I, Liguts V, Aldins P, Eksteina I, Platkājis A, Mārtinsone I, Tērauds E, Rozentāle B, Donaghy M (2008) Parkinsonian syndrome in methcathinone users and the role of manganese. N Engl J Med 358:1009–1017

    Article  PubMed  CAS  Google Scholar 

  142. Stracciari A, Guarino M, Pazzaglia P, Marchesini G, Pisi P (2001) Acquired hepatocerebral degeneration: full recovery after liver transplantation. J Neurol Neurosurg Psychiatry 70:136–137

    Article  PubMed  CAS  Google Scholar 

  143. Stracciari A, Mattarozzi K, D’Alessandro R, Baldin E, Guarino M (2008) Cognitive functioning in chronic acquired hepatocerebral degeneration. Metab Brain Dis 23:155–160

    Article  PubMed  Google Scholar 

  144. Sussman EB, Sternberg SS (1975) Hereditary hemorrhagic telangiectasia. A case with hepatocellular carcinoma and acquired hepatocerebral degeneration. Arch Pathol 99:95–100

    PubMed  CAS  Google Scholar 

  145. Taguchi Y, Takashima S, Hirade S, Inoue H (1999) Disappearance of globus pallidus hyperintensity in a patient with portal-systemic encephalopathy after occlusion of the shunt vessel. Rinsho Shinkeigaku 39:565–569

    PubMed  CAS  Google Scholar 

  146. Taylor-Robinson SD, Oatridge A, Hajnal J, Burroughs A, McIntyre N, Desouza N (1995) MR imaging of the basal ganglia in chronic liver disease: correlation of T1-weighted and magnetisation transfer contrast measurements with liver dysfunction and neuropsychiatric status. Metab Brain Dis 10:175–188

    Article  PubMed  CAS  Google Scholar 

  147. Thobois S, Giraud P, Debat P, Gouttard M, Maurizi A, Perret-Liaudet A, Kopp N, Broussolle E (2002) Orofacial dyskinesias in a patient with primary biliary cirrhosis: a clinicopathological case report and review. Mov Disord 17:415–419

    Article  PubMed  Google Scholar 

  148. Toghill P, Johnston A, Smith J (1967) Choreoathetosis in portosystemic encephalopathy. J Neurol Neurosurg Psychiatry 30:358–363

    Article  PubMed  CAS  Google Scholar 

  149. Triulzi F, Parazzini C, Righini A (2006) Patterns of damage in the mature neonatal brain. Pediatr Radiol 36:608–620

    Article  PubMed  Google Scholar 

  150. Troisi R, Debruyne J, de Hemptinne B (1999) Improvement of hepatic myelopathy after liver transplantation. N Engl J Med 340:151

    Article  PubMed  CAS  Google Scholar 

  151. Ueki Y, Isozaki E, Miyazaki Y, Koide R, Shimizu T, Yagi K, Hirai S (2002) Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy. Acta Neurol Scand 106:113–116

    Article  PubMed  CAS  Google Scholar 

  152. United Network for Organ Sharing. Data. (Accessed September 1, 2007) at: www.unos.org/data/

  153. vanWoerkem W (1914) 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 7: 41–51

  154. Victor M, Adams RD, Cole M (1965) The acquired (non-Wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 44:345–396

    Article  PubMed  CAS  Google Scholar 

  155. Walshe JM (2007) Cause of death in Wilson disease. Mov Disord 22:2216–2220

    Article  PubMed  Google Scholar 

  156. Weissenborn K, Ehrenheim C, Hori A, Kubicka S, Manns MP (1995) Pallidal lesions in patients with liver cirrhosis: clinical and MRI evaluation. Metab Brain Dis 10:219–231

    Article  PubMed  CAS  Google Scholar 

  157. Weissenborn K, Tietge UJ, Bokemeyer M, Mohammadi B, Bode U, Manns MP, Caselitz M (2003) Liver transplantation improves hepatic myelopathy: evidence by three cases. Gastroenterology 124:346–351

    Article  PubMed  Google Scholar 

  158. Wijdicks EF, Wiesner RH (2003) Acquired (non-Wilsonian) hepatocerebral degeneration: complex management decisions. Liver Transpl 9:993–994

    Article  PubMed  Google Scholar 

  159. Wilson S (1912) Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 34:295–509

    Article  Google Scholar 

  160. Wnorowski M, Prosch H, Prayer D, Janssen G, Gadner H, Grois N (2008) Pattern and course of neurodegeneration in langerhans cell histiocytosis. J Pediatr 153:127–132

    Article  PubMed  Google Scholar 

  161. Wolters EC, Huang CC, Clark C, Peppard RF, Okada J, Chu NS, Adam MJ, Ruth TJ, Li D, Calne DB (1989) Positron emission tomography in manganese intoxication. Ann Neurol 26:647–651

    Article  PubMed  CAS  Google Scholar 

  162. Wolz M, Junghanns S, Löhle M, von Kummer R, Storch A (2008) Dystonia associated with hyperintense basal ganglia lesions on T1-weighted brain MRI. Mov Disord 23:1618–1619

    Article  PubMed  Google Scholar 

  163. Yamada M, Ohno S, Okayasu I, Okeda R, Hatakeyama S, Watanabe H, Ushio K, Tsukagoshi H (1986) Chronic manganese poisoning: a neuropathological study with determination of manganese distribution in the brain. Acta Neuropathol (Berl) 70:273–278

    Article  PubMed  CAS  Google Scholar 

  164. Yoshikawa K, Matsumoto M, Hamanaka M, Nakagawa M (2003) A case of manganese induced parkinsonism in hereditary haemorrhagic telangiectasia. J Neurol Neurosurg Psychiatry 74:1312–1314

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dept. of Neurology, Baylor College of Medicine, 6550 Fannin, Suite 1801, Houston, Texas, 77030, USA

    J. Ferrara MD & J. Jankovic MD

Authors
  1. J. Ferrara MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J. Jankovic MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. Jankovic MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ferrara, J., Jankovic, J. Acquired hepatocerebral degeneration. J Neurol 256, 320–332 (2009). https://doi.org/10.1007/s00415-009-0144-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-009-0144-7

Key words

Search

Navigation