Chronic acquired hepatocerebral degeneration: Case reports and new insights

Authors

  • Mandar S. Jog,

    1. Morton and Gloria Shulman Movement Disorders Centre and Division of Neurology, The Toronto Hospital, Western Division, Toronto, Ontario, Canada
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  • Dr. Anthony E. Lang

    Corresponding author
    1. Morton and Gloria Shulman Movement Disorders Centre and Division of Neurology, The Toronto Hospital, Western Division, Toronto, Ontario, Canada
    • Morton and Gloria Shulman Movement Disorders Centre and Division of Neurology, The Toronto Hospital, Western Division, MP11-306, 399 Bathurst Street, Toronto, Ontario M5T 2S8, Canada
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Abstract

Chronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Little has been added to the understanding of this disorder since the detailed, early clinical and pathological descriptions. The spectrum of clinical presentations can be neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical laminar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Alzheimer type II astrocytes and cytoplasmic glycogen granules are characteristic. Recent neuroradiological observations in patiensts with liver failure have shown a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the pallidum, putamen, and, rarely, mesencephalon. Using clues from a similar MR appearance in patients receiving total parenteral nutrition as well as animals given parenteral manganese, and the knowledge that manganese is cleared by the hepatobiliary system, deposition of manganese in the brain is postulated in patients with CAHD. In this review we describe three cases of CAHD with detailed clinical and radiological documentation and discuss the aforementioned pathogenetic mechanisms.

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