title: Liver Transplant Can Resolve Severe Neuropsychiatric Manifestations of Wilson Disease: A Case Report.
abstract: Although liver transplant for decompensated cirrhosis secondary to Wilson disease is well accepted, the use of transplant for patients with severe neurologic manifestations of this condition remains controversial, and these can be perceived as a contraindication. Here, we describe a 45-year-old woman who presented with an incidental hepatocellular carcinoma at the time of transplant. The patient had severe neurologic manifestations of Wilson disease pretransplant, including dysarthria, hyperreflexia, asymmetrical ataxia, tremor, bradyphrenia, and shuffling gait. She underwent successful transplant from a hepatic and surgical standpoint, but her postoperative course was marked by protracted mutism, hypophonia, and fluctuating akinesia and immobility that did not respond promptly to withdrawal of calcineurin inhibitors or pramipexole but did respond robustly to amantadine. At 9 months posttransplant, there was marked neurologic improvement, and, at 18 months, she exhibited subtle memory and organizational difficulties but was fully ambulatory and otherwise completely functional. Our experience suggests that even patients with severe neurologic Wilson disease may recover after transplant, albeit slowly, demonstrating the need for a multidisciplinary approach, including pre- and posttransplant neurologic and neuropsychiatric consultations.
authors.affiliation: From the Division of Neurology, University of British Columbia, Canada.
authors.forename: Gregory
authors.initials: G
authors.lastname: Walker
authors.forename: Trana
authors.initials: T
authors.lastname: Hussaini
authors.forename: Robert
authors.initials: R
authors.lastname: Stowe
authors.forename: Silke
authors.initials: S
authors.lastname: Cresswell
authors.forename: Eric M
authors.initials: EM
authors.lastname: Yoshida
mesh_terms: D001259:Ataxia / Q000209:etiology / Q000503:physiopathology; D004401:Dysarthria / Q000209:etiology / Q000503:physiopathology; D005260:Female; D020233:Gait Disorders, Neurologic / Q000209:etiology / Q000503:physiopathology; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D051346:Mobility Limitation; D009155:Mutism / Q000209:etiology / Q000503:physiopathology; D020127:Recovery of Function; D012021:Reflex, Abnormal; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome