title: Long-term clinical and radiological improvement of chronic acquired hepatocerebral degeneration after obliteration of portosystemic shunt: Report of a case.
abstract: Neurological manifestations are common in patients with decompensated cirrhosis. The majority of these patients show hepatic encephalopathy or chronic acquired (non-Wilsonian) hepatocerebral degeneration (CAHD). They characteristically present with dysarthria, ataxia, involuntary movements, and altered mental status. Neuroradiological examination in patients with hepatic encephalopathy often shows abnormal signals in multiple regions of the brain, such as the pallidum, putamen, caudate nucleus, hemispheric white matter, and ventral midbrain. The pathogenesis of hepatic encephalopathy and CAHD is poorly understood and the response to conventional therapies is often poor. We report a male patient with cirrhosis of unknown cause, who developed slowly progressive cerebellar truncal and limb ataxia and slurred speech. Magnetic resonance imaging (MRI) showed focal T2 hyperintensity in bilateral dentate nuclei and middle cerebellar peduncles (MCPs). After treatment by obliteration of the portosystemic shunt, clinical manifestations and MRI abnormalities were dramatically improved. He was followed for six years until he died of uncontrollable bleeding due to hepatocellular carcinoma. At the last examination 9 months before death, he showed no apparent aggravation of neurological symptoms, and no abnormal signal intensities in the MCPs and supratentorial compartment. The clinical course and changes of brain MRI findings of this case are extremely rare, suggesting that obliteration of the portosystemic shunt may be effective for CAHD over long term.
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan. Electronic address: hisahara@sapmed.ac.jp.
authors.forename: Shin
authors.initials: S
authors.lastname: Hisahara
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsushita
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan; Department of Neurology, Yokohama Rosai Hospital, Yokohama 222-0036, Japan.
authors.forename: Mizuki
authors.initials: M
authors.lastname: Kitamura
authors.affiliation: Department of Gastroenterology, Sapporo Kyoritsu Hospital, Sapporo 007-0848, Japan.
authors.forename: Shinichi
authors.initials: S
authors.lastname: Mezawa
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan; Department of Neurology, Hokuyukai Neurological Hospital, Sapporo, 063-0802, Japan.
authors.forename: Michio
authors.initials: M
authors.lastname: Nonaka
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan; Department of Occupational Therapy, School of Health Science, Sapporo Medical University, Sapporo 060-8556, Japan.
authors.forename: Tomihiro
authors.initials: T
authors.lastname: Imai
authors.affiliation: Department of Neurology, School of Medicine, Sapporo Medical University, Sapporo 060-8556, Japan.
authors.forename: Shun
authors.initials: S
authors.lastname: Shimohama
mesh_terms: D000368:Aged; D006501:Hepatic Encephalopathy / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008103:Liver Cirrhosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D008297:Male; D011859:Radiography; D012082:Renal Veins / Q000601:surgery*; D013162:Splenic Vein / Q000601:surgery*; D016896:Treatment Outcome
keywords: Cerebellar ataxia; Chronic acquired hepatocerebral degeneration; Liver disease; MRI; Neuroradiology; Portosystemic shunt