Acquired hepatocerebral degeneration

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Presentation transcript:

Acquired hepatocerebral degeneration Teaching NeuroImages Neurology Resident and Fellow Section © 2016 American Academy of Neurology

Vignette A 42-year-old man with alcoholic cirrhosis presented to a neurology clinic with action tremor, attention/concentration deficits, and parkinsonism. Symptoms became apparent after hospitalization for alcohol withdrawal and acute alcoholic hepatitis MRI brain showed bilateral T1 hyperintense (A and B) and T2 hypointense (C and D) globus pallidus lesions Bateman et al. © 2016 American Academy of Neurology

Imaging Bateman et al. Figure: MRI Brain T1-weighted axial (A) and coronal (B) MRI demonstrating the characteristic hyperintensity primarily in the globus pallidus (asterisk)T2-weighted axial (C) and coronal (D) MRI images showing the corresponding T2 hypointensity in the globus pallidus (asterisk). Bateman et al. © 2016 American Academy of Neurology

Acquired hepatocerebral degeneration Acquired hepatocerebral degeneration is an under-diagnosed complication of cirrhotic liver disease Clinical symptoms include parkinsonism, ataxia, chorea, as well as cognitive, behavioral, and affective symptoms. Characteristic MRI changes shown above are likely secondary to manganese deposition in the basal ganglia Improvement can be seen following liver transplantation References 1. Ferrara J and Jankovic J. Acquired Hepatocerebral Degeneration. J Neurol 2009;256:320-332. 2. Fernandez-Rodriguez R, Contreras A, de Villoria JG, and Grandas F. Acquired Hepatocerebral Degeneration: Clinical Characteristics and MRI Findings. Eur J Neurol 2010;17:1463-1470. Bateman et al. © 2016 American Academy of Neurology