1317 - Generalized Dystonia Secondary to Wilson’s Disease (WD) Occurring After Curative Orthotopic Liver Transplant (LT)

Case Diagnosis: Wilson's Disease (WD)
Parkinsonism
Status post orthotopic liver transplant
Infectious Encephalopathy


Case Description: 27 y/o female with WD cirrhosis s/p LT was admitted to inpatient rehabilitation. Prior to transplant, she exhibited a mild tremor without other movement disorder symptoms. Post-transplant, she developed severe tremors/jerking movements/encephalopathy/dystonia. She was diagnosed with HHV-6 viremic encephalopathy and treated with Foscarnet. Despite this treatment of infectious encephalopathy, her movement disorder symptoms worsened. She was initiated on Baclofen, Trihexyphenidyl, Amantadine, and Lorazepam which, in time, along with multidisciplinary inpatient rehabilitation, improved her neurological symptoms and led to functional gains. 

Discussions: Our patient developed multiple complications s/p LT, notably a severe movement disorder. It is likely that copper overload build up in the brain leading to necrosis of the basal ganglia cells did not happen instantly, explaining the onset of her movement disorder despite cure of her WD. Over the next few months, her dystonia improved remarkably, gaining dexterity with finger movements and improvements in ambulation. 

Conclusions: In 2022 a pubmed systematic literature review evaluated LT as a treatment for neurological symptoms in WD. Based on this review, evidence on whether LT can cure the neurological symptoms of WD is conflicting. Recent studies demonstrate that LT shows improvement in said symptoms, however older studies showed there could be little to no benefit at all in alleviating symptoms after transplant. Although physiotherapy for dystonia has been analyzed, there is a lack of research in therapy in WD patients with these symptoms, especially after liver transplant. Thus, we must highlight the integral role that physiotherapy played in our patient’s improvement in dystonia and functional status after LT. Our patient who received inpatient rehabilitation soon after her transplant demonstrates the vital and beneficial role that therapy may have in alleviating neurological symptoms in WD if provided early, and perhaps prior, to transplantation.