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Progressive hepatic mitochondrial dysfunction in premanifest Huntington's disease
Author(s) -
Hoffmann Rainer,
Stüwe Sven H.,
Goetze Oliver,
Banasch Matthias,
Klotz Peter,
Lukas Carsten,
Tegenthoff Martin,
Beste Christian,
Orth Michael,
Saft Carsten
Publication year - 2014
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25862
Subject(s) - subclinical infection , medicine , huntington's disease , gastroenterology , analysis of variance , disease , parkinson's disease , oncology
Background A subclinical, hepatic involvement in manifest and premanifest Huntington's disease (HD) was recently demonstrated by using the 1 3 C‐methionine breath test (MeBT). In this longitudinal pilot study, we investigated whether there is evidence for progressive hepatic mitochondrial dysfunction in premanifest HD. Methods The MeBT was performed within a group of 25 well‐characterized premanifest HD mutation carriers at baseline and in a 14.5‐month follow‐up. Results The total group of mutation carriers ( P = 0.033; Cohen's d = 0.6) and the subgroup of mutation carriers from our PreHD‐B subgroup (nearer to disease onset; P = 0.030; Cohen's d = 1.12) revealed a lower amount of exhaled 13 CO 2 in the follow‐up. Conclusions This study demonstrates in vivo progressive, subclinical, hepatic involvement in premanifest HD. Limitations of the study, such as high variance in breath test results, are discussed. © 2014 International Parkinson and Movement Disorder Society © 2014 International Parkinson and Movement Disorder Society