title: Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy.
abstract: We report two patients with chronic acquired hepatocerebral degeneration (CAHD) who showed neurological and radiological improvement after the administration of branched-chain amino acids (BAA). The first patient with chronic hepatitis C presented with progressive parkinsonism for 7 months, whereas the second patient with liver cirrhosis presented with progressive ataxia for 15 months. T1-weighted magnetic resonance imaging (MRI) showed symmetric high intensity signals in the lenticular nuclei in both patients. In the first patient, single photon emission computed tomography (SPECT) disclosed a marked decrease in cerebral blood flow in the parieto-occipital regions. In the second patient, T2-weighted MRI demonstrated symmetric high intensity signals in the deep cerebral and cerebellar white matter. After the administration of BAA, their neurological signs and radiological abnormalities markedly improved in both patients. CAHD might be a reversible and treatable disorder where aromatic amino acids are deeply involved in its pathogenesis.
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu, Tokyo, Japan. yueki@tmnh.fuchu.tokyo.jp
authors.forename: Y
authors.initials: Y
authors.lastname: Ueki
authors.forename: E
authors.initials: E
authors.lastname: Isozaki
authors.forename: Y
authors.initials: Y
authors.lastname: Miyazaki
authors.forename: R
authors.initials: R
authors.lastname: Koide
authors.forename: T
authors.initials: T
authors.lastname: Shimizu
authors.forename: K
authors.initials: K
authors.lastname: Yagi
authors.forename: S
authors.initials: S
authors.lastname: Hirai
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000597:Amino Acids, Branched-Chain / Q000627:therapeutic use*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D000597:Amino Acids, Branched-Chain