MRI T2 Hypointensities in basal ganglia of premanifest Huntington's disease
Open Access
- 8 September 2010
- journal article
- Published by Public Library of Science (PLoS) in PLoS Currents
- Vol. 2, RRN1173
- https://doi.org/10.1371/currents.rrn1173
Abstract
Increased iron levels have been demonstrated in the basal ganglia of manifest Huntington’s disease (HD). An excess in iron accumulation correlates with MRI T2-weighted hypointensity. Determination of the amount of hypointensities in the basal ganglia in the premanifest phase of HD may give more insight in the role of iron in the pathogenesis of HD. Therefore, the present study assessed whether the degree of hypointensities on T2-w MRI in the basal ganglia of premanifest gene carriers differs from non-carriers. Seventeen HD gene carriers without clinical motor signs and 15 non-carriers underwent clinical evaluation and MRI scanning. The amount of T2-w hypointensities was determined using a computer-assisted quantitative method that classified each pixel in the basal ganglia as hypointense or not, resulting in a total of hypointense pixels for each individual. Carriers showed an increased amount of hypointensities in the basal ganglia compared to non-carriers. More hypointensities were furthermore associated with a higher UHDRS total motor score, a longer CAG repeat length and a greater probability of developing symptoms within 5 years. We concluded that the increased amount of hypointensities in the basal ganglia of premanifest carriers of the HD gene may reflect excessive iron deposition and a role for iron in the neuropathology of HD. Furthermore, this phenomenon is associated with clinical and biological disease characteristics. An increased amount of hypointensities on T2-w MRI in the basal ganglia may be considered a biomarker for HD.This publication has 27 references indexed in Scilit:
- Characterizing iron deposition in Parkinson's disease using susceptibility-weighted imaging: An in vivo MR studyBrain Research, 2010
- Myelin Breakdown and Iron Changes in Huntington’s Disease: Pathogenesis and Treatment ImplicationsNeurochemical Research, 2007
- High‐field magnetic resonance imaging of brain iron: birth of a biomarker?NMR in Biomedicine, 2004
- A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG lengthClinical Genetics, 2004
- Brain iron pathways and their relevance to Parkinson's diseaseJournal of Neurochemistry, 2001
- MRI evaluation of basal ganglia ferritin iron and neurotoxicity in Alzheimer's and Huntingon's disease.2000
- Increased Basal Ganglia Iron Levels in Huntington DiseaseArchives of Neurology, 1999
- MR of human postmortem brain tissue: correlative study between T2 and assays of iron and ferritin in Parkinson and Huntington disease.1993
- ALTERATIONS IN THE LEVELS OF IRON, FERRITIN AND OTHER TRACE METALS IN PARKINSON'S DISEASE AND OTHER NEURODEGENERATIVE DISEASES AFFECTING THE BASAL GANGLIABrain, 1991
- THE EFFECT OF AGE ON THE NON‐HAEMIN IRON IN THE HUMAN BRAINJournal of Neurochemistry, 1958