Detection of dentate nuclei abnormality in a patient with dentatorubral-pallidoluysian atrophy using the quantitative susceptibility mapping


      • QSM directly maps brain tissue magnetic susceptibility reflecting iron deposition.
      • QSM showed dentate nuclei abnormality for DRPLA compared to HD and NCs.
      • Assessing the dentate nuclei using QSM may be useful for diagnosing DRPLA.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Koide R.
        • et al.
        Atrophy of the cerebellum and brainstem in dentatorubral pallidoluysian atrophy influence of CAG repeat size on MRI findings.
        Neurology. 1997; 49: 1605-1612
        • Ross C.A.
        • et al.
        Huntington disease: natural history, biomarkers and prospects for therapeutics.
        Nat. Rev. Neurol. 2014; 10: 204-216
        • Deistung A.
        • et al.
        Toward in vivo histology: a comparison of quantitative susceptibility mapping (QSM) with magnitude-, phase-, and R 2-imaging at ultra-high magnetic field strength.
        Neuroimage. 2013; 65: 299-314
        • Van Bergen J.M.
        • et al.
        Quantitative susceptibility mapping suggests altered brain iron in premanifest Huntington disease.
        Am. J. Neuroradiol. 2016; 37: 789-796
        • Liu J.
        • et al.
        Morphology enabled dipole inversion for quantitative susceptibility mapping using structural consistency between the magnitude image and the susceptibility map.
        NeuroImage. 2012; 59: 2560-2568
        • Sunami Y.
        • et al.
        Radiologic and neuropathologic findings in patients in a family with dentatorubral-pallidoluysian atrophy.
        Am. J. Neuroradiol. 2011; 32: 109-114
        • Waldvogel H.J.
        • et al.
        The neuropathology of Huntington's disease.
        in: Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease. Springer, 2014: 33-80
        • Yamada M.
        Dentatorubral-pallidoluysian atrophy (DRPLA).
        Neuropathology. 2010; 30: 453-457
        • Langkammer C.
        • et al.
        Quantitative susceptibility mapping (QSM) as a means to measure brain iron? A post mortem validation study.
        Neuroimage. 2012; 62: 1593-1599
        • McCarthy R.C.
        • et al.
        Inflammation-induced iron transport and metabolism by brain microglia.
        J. Biol. Chem. 2018; 293 (p. jbc. RA118. 001949): 7853-7863