Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2011 Feb;15(1):58-64.
doi: 10.1111/j.1399-3046.2010.01411.x. Epub 2010 Oct 8.

Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series

D A Shellmer  1 , A DeVito Dabbs, M A Dew, R B Noll, H Feldman, K A Strauss, D H Morton, J Vockley, G V Mazariegos
Affiliations

Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series

D A Shellmer et al. Pediatr Transplant. 2011 Feb.

Abstract

MSUD is a complex metabolic disorder that has been associated with central nervous system damage, developmental delays, and neurocognitive deficits. Although liver transplantation provides a metabolic cure for MSUD, changes in cognitive and adaptive functioning following transplantation have not been investigated. In this report, we present data from 14 patients who completed cognitive and adaptive functioning testing pre- and one yr and/or three yr post-liver transplantation. Findings show either no significant change (n=8) or improvement (n=5) in IQ scores pre- to post-liver transplantation. Greater variability was observed in adaptive functioning scores, but the majority of patients evidenced no significant change (n=8) in adaptive scores. In general, findings indicate that liver transplantation minimizes the likelihood of additional central nervous system damage, providing an opportunity for possible stabilization or improvement in neurocognitive functioning.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Cognitive functioning scores pre- and one year post-liver transplantation
Fig. 2
Fig. 2
Adaptive functioning scores pre- and one year post-liver transplantation
Fig. 3
Fig. 3
Cognitive functioning scores pre, one-year, and three years post-liver transplantation.
Fig. 4
Fig. 4
Adaptive functioning scores pre, one-year, and three years post-liver transplantation.
See this image and copyright information in PMC

References

    1. Hoffmann B, Helbling C, Schadewaldt P, Wendel U. Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD. Pediatric Research. 2006;59(1):17–20. - PubMed
    1. Strauss K, Puffenberger EG, Morton DH. Maple Syrup Urine Disease, GeneReviews, initial posting January 2006. [Last accessed Dec. 2009]. 2006. Available from: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=msud.
    1. le Roux C, Murphy E, Hallam P, Lilburn M, Orlowska D, Lee P. Neuropsychometric outcome predictors for adults with maple syrup urine disease. Journal Inherited Metabolic Disease. 2006;29(1):201–202. - PubMed
    1. Strauss K, Mazariegos GV, Sindhi R, Squires R, Finegold DN, Vockley G, Robinson DL, Hendrickson C, Virji M, Cropcho L, Puffenberger EG, McGhee W, Seward LM, Morton DH. Elective liver transplantation for the treatment of classical maple syrup urine disease. American Journal of Transplantation. 2006;6(3):557–564. - PubMed
    1. Morton DH, Strauss KA, Robinson DL, Puffenberger EG, Kelley RI. Diagnosis and Treatment of Maple Syrup Disease: A Study of 36 Patients. Pediatrics. 2002;109(6):999–1008. - PubMed