Liver transplantation in neurological Wilson’s disease in children: Where do we stand?

Table 1 Recent studies (published in last 10 years) reporting neurological outcomes of liver transplantation in Wilson’s disease

Ref.	Number of patients enrolled in the studies with their ages at LT	Number of transplanted patients with neurological manifestations	Indication of LT (n, %)	Outcome among pediatric patients with neurologic involvement: Neurologic improvement or death	NW/new-onset neurological symptoms	Mean/median (interquartile range) follow-up
Beyzaei et al[60], 2025	Total n = 106; pediatric, n = 98, (median pediatric age: 10.8 years)	n = 5/98 (P), all neuro-hepatic; n = 2/8 (A), neurologic	Hepatic, n = 93/98 (94.9%); neuro-hepatic, n = 5/98 (5.1%)	NI: 5/5; deaths: 11/98, 11.2% (neurological issue in one)	New-onset neurological symptoms in those without any pre-existing neurologic symptoms: n = 7	21 ± 1.8 months
Isa HM et al[68], 2024	Total n = 8; pediatric, n = 7	n = 1/7	Neurologic and hepatic	None	No neurological improvement	NA
Aksoy et al[29], 2022	Total n = 24 (including adults); mean age: 34, range: 14-57 years	n = 7, all neuro-hepatic; age NA	Hepatic: n = 24 (100%)	NI, n = 6/7; no improvement in one patient who had a brain lesion unrelated to WD	None	Up to 10 years
Couchonnal et al[57], 2021	Total n = 39, all pediatric	n = 6, neurologic	Hepatic, n = 33; neurologic, n = 6	NI, n = 6, one had persistent behavioral abnormalities after LT	None	4.3 ± 2.5 years
Poujois et al[18], 2020	Total n = 18 (including adults) (median age: 18.5 years)	n = 18; n = 13, neurologic; n = 5: Neuro-hepatic; age at LT for individual patient NA	Neurologic: n = 18, 100%	Overall NI: n = 14/18; death: n = 4 (pulmonary infection)	None	Median 40.9 (18.1-93.2) months
Öcal et al[65], 2020	Total: n = 15; pediatric: Nil; mean age: 35.2 years	n = 4, all neuro-hepatic	NA	NI, n = 4	New-onset neurological symptoms in those without any pre-existing neurologic symptoms: n = 1	NA
Moguilevitch and Straker[69], 2020	Total n = 1; age: 15 years	n = 1, neuro-hepatic	Neurologic and hepatic	NI, n = 1	None	12 months
Laurencin et al[52], 2017	Total n = 4 (including adults); age range: 15-23 years	n = 2, neuro-hepatic; n = 2, neurologic	Hepatic: n = 2 (50%); neurologic: n = 2 (50%)	NI, n = 4; death: None	Transient NW, improved on D-penicillamine: n = 1	1-4 years
Lankarani et al[48], 2016	Total n = 107 (including adults; age range: 5-59 years)	n = 60/107, all neuro-hepatic (adults included)	Hepatic: 107 (100%)	NI, n = 40/60 (66.67%); stable disease, n = 2/60 (3.3%); death: 16/107	NW: n = 18/60 (30%); new onset neurological symptoms: n = 4	4015 days
Yagci et al[49], 2015	Total n = 9 (including adults); pediatric, n = 4; median age: 19 (range: 10-25) years	n = 9, all neuro-hepatic	Hepatic: 9 (100%)	NI, n = 7/8 (87.5%); one patient had post-operative acute ischemic stroke, hence, excluded from analysis	NW, n = 1/8	36.6 months
Sutariya et al[70], 2015	n = 1, age: 14 years	n = 1, neurologic	Neurologic: 1	NI, n = 1; death after 1 year of LT due to discontinuation of immunosuppression leading to rejection	None	12 months
Guillaud et al[21], 2014	Total n = 121; pediatric, n = 46, median (range) age: 14 years (7-17)	n (adult + pediatric) = 26; mixed neuro-hepatic, n = 19; pure neurologic: n = 7	Hepatic: n = 104/121, (94.2%); pure neurologic: 7/121 (5.8%)	NI, n = 11/26; stable disease, n = 1/26; death, n = 4/26 (3 were due to sepsis); data NA, n = 9/26	NW: n = 1 (in mixed neuro-hepatic)	Median post-transplant follow-up: 72 months (0-23.5)
Mocchegiani et al[56], 2014	n = 1, age: 19 years	n = 1, neurologic	Neurologic: 1	NI, n = 1	None	4 years

LT: Liver transplantation; NW: Neurological worsening; A: Adults; P: Pediatric; NI: Neurological improvement; NA: Not available; WD: Wilson’s disease.