CHRONIC ACQUIRED HEPATOCEREBRAL DEGENERATION - CASE-REPORTS AND NEW INSIGHTS

Chronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combin ed presentation. Little has been added to the understanding of this di sorder since the detailed, early clinical and pathological description s. The spectrum of clinical presentations can be neuropsychiatric (apa thy, lethargy, excessive somnolence), a movement disorder (ataxia, tre mor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical lam inar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Al zheimer type II astrocytes and cytoplasmic glycogen granules are chara cteristic. Recent neuroradiological observations in patients with live r failure have shown a specific magnetic resonance (MR) imaging appear ance with a hyperintense T1 signal in the pallidum, putamen, and, rare ly, mesencephalon. Using clues from a similar MR appearance in patient s receiving total parenteral nutrition as well as animals given parent eral manganese, and the knowledge that manganese is cleared by the hep atobiliary 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.