Abstract
The term “acquired hepatocerebral degeneration” (AHD) was coined to describe clinical entity distinct from genetically defined Wilson’s disease. AHD is chronic neurological disorder, characterized by extrapyramidal and neuropsychiatric symptoms accompanied with advanced liver disease with portosystemic shunts. In majority of AHD cases, extrapyramidal symptoms appear in the presence of known liver disease. Here we present a patient with subacute onset of bilateral, asymmetric, hypokinetic-rigid syndrome and ataxia as initial presentation of liver cirrhosis. Manganese toxicity have major role in AHD pathogenesis. Failure of liver detoxification and presence of portosystemic shunting enables neurotoxic substance of manganese to avoid hepatic metabolism and to enter and accumulate in central nervous system. Predilection brain regions for manganese deposits are globus pallidum (GP) and substantia nigra (SN). Characteristic MRI findings of bilateral, symmetrical hyperintensities of GP and SN on T1-weighted sequences supported the diagnosis of AHD in our patient. In addition, increased T2 signal in dendate nuclei seen in our patient is rare radiological finding. So far, no consensus guidelines regarding medical treatment of AHD exist. We initiated low-dose levodopa treatment, but failed to provide beneficial effect. In conclusion, AHD is distinct clinical entity that should be included in differential diagnosis of both typical and atypical parkinsonian syndromes. Furthermore, our case highlights the importance of performing MRI in patients with atypical parkinsonism.
References
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
Butterworth RF (2012) Parkinsonism in cirrhosis: Pathogenesis and current therapeutic options. Metab Brain Dis 28:261–7
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 23:268–70
Fernández-Rodriguez R, Contreras A, De Villoria JG, Grandas F (2010) Acquired hepatocerebral degeneration: Clinical characteristics and MRI findings. Eur J Neurol 17:1463–70
Ferrara J, Jankovic J (2009) Acquired hepatocerebral degeneration. J Neurol 256:320–332
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 manganese-exposed non-human primates. Exp Neurol 202:381–90
Noone ML, Kumar VG, Ummer K, Achambat L, Salam KA (2008) Cirrhosis presenting as parkinsonism. Ann Indian Acad Neurol 11:179–81
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 23:768–70
Park SA, Heo K (2004) Prominent cerebellar symptoms with unusual magnetic resonance imaging findings in acquired hepatocerebral degeneration. Arch Neurol 61:1458–60
Pomier-Layrargues G, Spahr L, Butterworth RF (1995) Increased manganese concentrations in pallidum of cirrhotic patients. Lancet 345:735
Shulman LM, Minagar A, Weiner WJ (2003) Reversal of parkinsonism following liver transplantation. Neurology 60:519
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
Tuschl K, Clayton PT, Gospe SM Jr, Gulab S, Ibrahim S, Singhi P, Aulakh R, Ribeiro RT, Barsottini OG, Zaki MS, Del Rosario ML, Dyack S, Price V, Rideout A, Gordon K, Wevers RA, Chong WK, Mills PB (2012) Syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia caused by mutations in SLC30A10, a manganese transporter in man. Am J Hum Genet 90:457–66
van Woerkom W (1914) La cirrhose hépatique avec altérations dans les centres nerveux évoluant chez des sujets d’âge moyen. Nouvelle iconographie de la salpétriére. Clinique des Maladies du Systéme Nerveux 7:41–51
Victor M, Adams R, Cole M (1965) The acquired (non-wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 44:345–396
Acknowledgments
Supported from Croatian Ministry of Science, Education and Sports
Conflict of interest
The authors declare that they have no conflict of interest
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Miletić, V., Ozretić, D. & Relja, M. Parkinsonian syndrome and ataxia as a presenting finding of acquired hepatocerebral degeneration. Metab Brain Dis 29, 207–209 (2014). https://doi.org/10.1007/s11011-013-9478-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11011-013-9478-z