S K Sethi1,2, S Sharma3, S Gharabaghi2, D Reese4, Y Chen5, P Adams6, M S Jog3, E M Haacke7,2. 1. From the Department of Radiology (S.K.S., E.M.H.), Wayne State University, Detroit, Michigan sethisea@gmail.com. 2. SpinTech MRI Inc (S.K.S., S.G., E.M.H.), Bingham Farms, Michigan. 3. Department of Clinical Neurological Sciences (S.S., M.S.J.), London Health Sciences Centre. 4. Imaging Research Laboratories (D.R.), Robarts Research Institute, London, Ontario, Canada. 5. Department of Neurology (Y.C.), Wayne State University School of Medicine, Detroit, Michigan. 6. Division of Gastroenterology (P.A.), Department of Medicine, Western University, London, Ontario, Canada. 7. From the Department of Radiology (S.K.S., E.M.H.), Wayne State University, Detroit, Michigan.
Abstract
BACKGROUND AND PURPOSE: Brain iron dyshomeostasis is increasingly recognized as an important contributor to neurodegeneration. Hereditary hemochromatosis is the most commonly inherited disorder of systemic iron overload. Although there is an increasing interest in excessive brain iron deposition, there is a paucity of evidence showing changes in brain iron exceeding that in healthy controls. Quantitative susceptibility mapping and R2* mapping are established MR imaging techniques that we used to noninvasively quantify brain iron in subjects with hereditary hemochromatosis. MATERIALS AND METHODS: Fifty-two patients with hereditary hemochromatosis and 47 age- and sex-matched healthy controls were imaged using a multiecho gradient-echo sequence at 3T. Quantitative susceptibility mapping and R2* data were generated, and regions within the deep gray matter were manually segmented. Mean susceptibility and R2* relaxation rates were calculated for each region, and iron content was compared between the groups. RESULTS: We noted elevated iron levels in patients with hereditary hemochromatosis compared with healthy controls using both R2* and QSM methods in the caudate nucleus, putamen, pulvinar thalamus, red nucleus, and dentate nucleus. Additionally, the substantia nigra showed increased susceptibility while the thalamus showed an increased R2* relaxation rate compared with healthy controls, respectively. CONCLUSIONS: Both quantitative susceptibility mapping and R2* showed abnormal levels of brain iron in subjects with hereditary hemochromatosis compared with controls. Quantitative susceptibility mapping and R2* can be acquired in a single MR imaging sequence and are complementary in quantifying deep gray matter iron.
BACKGROUND AND PURPOSE: Brain iron dyshomeostasis is increasingly recognized as an important contributor to neurodegeneration. Hereditary hemochromatosis is the most commonly inherited disorder of systemic iron overload. Although there is an increasing interest in excessive brain iron deposition, there is a paucity of evidence showing changes in brain iron exceeding that in healthy controls. Quantitative susceptibility mapping and R2* mapping are established MR imaging techniques that we used to noninvasively quantify brain iron in subjects with hereditary hemochromatosis. MATERIALS AND METHODS: Fifty-two patients with hereditary hemochromatosis and 47 age- and sex-matched healthy controls were imaged using a multiecho gradient-echo sequence at 3T. Quantitative susceptibility mapping and R2* data were generated, and regions within the deep gray matter were manually segmented. Mean susceptibility and R2* relaxation rates were calculated for each region, and iron content was compared between the groups. RESULTS: We noted elevated iron levels in patients with hereditary hemochromatosis compared with healthy controls using both R2* and QSM methods in the caudate nucleus, putamen, pulvinar thalamus, red nucleus, and dentate nucleus. Additionally, the substantia nigra showed increased susceptibility while the thalamus showed an increased R2* relaxation rate compared with healthy controls, respectively. CONCLUSIONS: Both quantitative susceptibility mapping and R2* showed abnormal levels of brain iron in subjects with hereditary hemochromatosis compared with controls. Quantitative susceptibility mapping and R2* can be acquired in a single MR imaging sequence and are complementary in quantifying deep gray matter iron.
Authors: Pierre Brissot; Antonello Pietrangelo; Paul C Adams; Barbara de Graaff; Christine E McLaren; Olivier Loréal Journal: Nat Rev Dis Primers Date: 2018-04-05 Impact factor: 52.329
Authors: Sean K Sethi; Shawn J Kisch; Kiarash Ghassaban; Ali Rajput; Alex Rajput; Paul S Babyn; Saifeng Liu; Peter Szkup; E Mark Haacke Journal: Magn Reson Imaging Date: 2018-10-13 Impact factor: 2.546
Authors: George Bartzokis; Po H Lu; Kathleen Tingus; Douglas G Peters; Chetan P Amar; Todd A Tishler; J Paul Finn; Pablo Villablanca; Lori L Altshuler; Jim Mintz; Elizabeth Neely; James R Connor Journal: Neuropsychopharmacology Date: 2011-03-09 Impact factor: 7.853