Are acquired hepatocerebral degeneration and hepatic myelopathy reversible?
BACKGROUND
Acquired hepatocerebral degeneration (AHD) and hepatic myelopathy (HM) are rare complications of chronic liver disease and are usually resistant to medical therapy.


METHODS
The clinical and laboratory findings of 14 male and 2 female patients with AHD or HM were evaluated.


RESULTS
The prevalence of AHD and HM was 2% inpatient case series in the last 10 years. The median age of the patients (5 Child's B and 11 Child's C) was 48.7 years (28 to 66 y), and the mean known duration of the liver disease was 75 months (24 to 194 mo). The median time of onset of neurologic findings after diagnosis of the liver disease was 14.5 months. Eight patients who had marked spastic paraparesis or tetraparesis were included in the HM group and all others had AHD group. Sixty-nine percent of the patients had a spontaneous or surgical portosystemic shunts, and the remaining dense retroperitoneal collaterals. During the follow-up period of median 29 months (4 to 72 mo), 12 patients died while waiting for liver transplantation, and these patients suffered from the several complications of chronic liver disease more than the living patients. A marked improvement was observed in 2 of the patients (1 with AHD and the other with HM) at 6 and 8 months after the liver transplantation, respectively.


CONCLUSIONS
Our data suggest that liver transplantation had an important effect on the improvement in these patients.
Pinarbasi
Binnur
B
Department of Gastroenterohepatology, Medical Faculty, Istanbul University, Istanbul, Turkey. binnurcapa@yahoo.com
Kaymakoglu
Sabahattin
S
Matur
Zeliha
Z
Akyuz
Filiz
F
Demir
Kadir
K
Besisik
Fatih
F
Ozdil
Sadakat
S
Boztas
Gungor
G
Cakaloglu
Yilmaz
Y
Mungan
Zeynel
Z
Okten
Atilla
A
D000328:Adult; D000368:Aged; D005260:Female; D006501:Hepatic Encephalopathy / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery; D006801:Humans; D008099:Liver / Q000473:pathology / Q000601:surgery*; D008103:Liver Cirrhosis / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D020336:Paraparesis, Spastic / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery; D011170:Portasystemic Shunt, Surgical; D015995:Prevalence; D011782:Quadriplegia / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery; D016896:Treatment Outcome