ATTRV30M amyloidosis post-liver transplant: cognition and long-term survival.

Background: Patients with hereditary amyloidosis related to transthyretin (ATTRv amyloidosis) treated with liver transplant (LTx) often have central nervous system (CNS) manifestations, including cognitive dysfunction. The aim of this study was to explore the long-term outcome associated with neuropsychological test performance.

Methods: A retrospective longitudinal review was conducted in a cohort of 289 ATTRv amyloidosis patients with the Val30Met mutation (ATTRV30M amyloidosis) who underwent a neuropsychological assessment (T1) 1-23 years (median = 11) post-LTx and 20-189 months (median = 81) prior to the study review. Clinical records were reviewed. The Kaplan-Meier and Cox regression methods were used to estimate survival and adjusted hazard ratios for all-cause mortality.

Results: Impaired performance on Dementia Rating Scale-2, Semantic Fluency, Phonemic Fluency and Trail Making Test Part B were predictive of shorter survival after neuropsychological assessment, even when demographic and clinical variables (i.e. education, age at disease onset $\ge$50, disease duration at LTx, interval between LTx and T1, age at T1, Modified Polyneuropathy Disability score at T1, and history of focal neurological episodes at T1) were taken into account. Measures of verbal learning and memory were not predictive of mortality.

Conclusions: Study results demonstrate that cognitive impairment in ATTRV30M amyloidosis patients treated with LTx predicts long-term survival.