Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease
  • Daniyal Raza
    Department of Internal Medicine, Louisiana State University Health Shreveport, Shreveport, USA
  • Natalie Bzowej
    Department of Hepatology, Ochsner Medical Center, New Orleans, USA
  • James Rini
    Department of Neurology, Ochsner Medical Center, New Orleans, USA
  • Rachel Rhinehart
    Department of Neurology, Ochsner Medical Center, New Orleans, USA
  • Hrishikesh Samant
    Department of Hepatology, Ochsner Medical Center, New Orleans, USA

Keywords

Chronic liver disease, hepatic encephalopathy, acquired hepatocerebral degeneration (AHD), liver transplant

Abstract

This case report details the complex diagnostic odyssey of a 60-year-old female grappling with chronic liver disease, initially diagnosed with hepatic encephalopathy (HE). Despite initial treatment with lactulose and rifaximin, her neurological symptoms worsened, leading to the identification of concurrent acquired hepatocerebral degeneration (AHD). This condition is characterised by cognitive decline, movement disorders and distinctive imaging abnormalities. The discussion highlights the challenges in distinguishing AHD from HE, underscoring the sophisticated diagnostic and management strategies required for such intricate cases in the realm of chronic liver disease.

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References

  • 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.
  • 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.
  • Yalcin D, Oguz-Akarsu E, Sokmen M. Acquired hepatocerebral degeneration. Neurosciences (Riyadh) 2016;21:164–167.
  • Huang FZ, Hou X, Zhou TQ, Chen S. Hepatic encephalopathy coexists with acquired chronic hepatocerebral degeneration. Neurosciences (Riyadh) 2015;20:277–279.
  • Malaquias MJ, Pinto CM, Ramos C, Ferreira S, Gandara J, Almeida A, et al. Acquired hepatocerebral degeneration and hepatic encephalopathy: one or two entities? Eur J Neurol 2020;27:2396–2404.
  • Zwingmann C, Butterworth R. An update on the role of brain glutamine synthesis and its relation to cell-specific energy metabolism in the hyperammonemic brain: further studies using NMR spectroscopy. Neurochem Int 2005;47:19–30.
  • 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.
  • Maffeo E, Montuschi A, Stura G, Giordana MT. Chronic acquired hepatocerebral degeneration, pallidal T1 MRI hyperintensity and manganese in a series of cirrhotic patients. Neuro Sci 2014;35:523–530.
  • Romeiro FG, Américo MF, Yamashiro FS, Caramori CA, Schelp AO, Santos AC, [Q4] 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.
  • Pinarbasi B, Kaymakoglu S, Matur Z, Akyuz F, Demir K, Besisik F, et al. Are acquired hepatocerebral degeneration and hepatic myelopathy reversible? J Clin Gastroenterol 2009;43:176–181.

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    Published: 2024-06-28
    Issue: 2024: LATEST ONLINE (view)

    How to cite:
    1.
    Raza D, Bzowej N, Rini J, Rhinehart R, Samant H. Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease. EJCRIM 2024;11 doi:10.12890/2024_004629.

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