Advertisement

Multifaceted structural magnetic resonance imaging findings in demented patients with pathologically confirmed TDP-43 proteinopathy

  • Keita SakuraiEmail author
  • Satoru Morimoto
  • Takuya Oguri
  • Hiroyuki Yuasa
  • Yuto Uchida
  • Kentaro Yamada
  • Masahiro Muto
  • Yufuko Saito
  • Ikuko Aiba
  • Masaki Takao
  • Shohei Inui
  • Keiko Toyoda
  • Asako Yamamoto
  • Hidetsuna Utsunomiya
  • Hiroshi Oba
  • Aya.M Tokumaru
  • Motoo Nakagawa
  • Yoshio Hashizume
  • Mari Yoshida
Short Report

Abstract

This short report clarifies the heterogeneity of structural magnetic resonance imaging (MRI) findings in seven demented patients due to pathologically accumulated TAR DNA-binding protein-43 (TDP-43) protein using visual analyses including visual rating scales (i.e., global cortical atrophy and medial temporal atrophy scales). In addition to the well-known frontotemporal lobar atrophy, structural MRI has revealed multifaceted imaging findings including asymmetric atrophy of the frontoparietal lobe and cerebral peduncle, midbrain atrophy, and localized or diffuse white matter T2 hyperintensity. Understanding of these multifaceted neuroimaging findings is important for the precise antemortem diagnosis of TDP-43 proteinopathy.

Keywords

Atrophy Frontotemporal lobar degeneration Magnetic resonance imaging Signal change TDP-43 proteinopathy Visual rating scale 

Notes

Funding information

This study was supported by Grants-in-Aid for Scientific Research from the Japanese Ministry of Education, Culture, Sports, Science and Technology (Kakenhi Wakate B, 16K19839: KS).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

We declare that all human and animal studies have been approved by the Ethics Committee for Clinical Research of the Nagoya City University Graduate School of Medical Sciences, Tosei General Hospital, Nagoya City East Medical Center, Fukushimura Hospital, Higashi Nagoya National Hospital, and Mihara Memorial Hospital, and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Due to the study nature evaluating pathologically confirmed patients, it was difficult to acquire the informed consent from all patients. Therefore, with the permission of IRB, we provided the opportunity to opt out for bereaved families of these patients.

References

  1. 1.
    Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, Dearmond SJ, Miller BL, Seeley WW (2010) TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology 75(24):2204–2211CrossRefGoogle Scholar
  2. 2.
    Whitwell JL, Jack CR Jr, Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, Senjem ML, Knopman DS, Petersen RC, Dickson DW, Josephs KA (2010) Imaging correlates of pathology in corticobasal syndrome. Neurology 75(21):1879–1887CrossRefGoogle Scholar
  3. 3.
    Whitwell JL, Jack CR Jr, Parisi JE, Senjem ML, Knopman DS, Boeve BF, Rademakers R, Baker M, Petersen RC, Dickson DW, Josephs KA (2010) Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration? Neurology 75(24):2212–2220CrossRefGoogle Scholar
  4. 4.
    Whitwell JL, Jack CR Jr, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW, Josephs KA (2011) Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia. J Mol Neurosci 45(3):372–378CrossRefGoogle Scholar
  5. 5.
    Mackenzie IR, Neumann M, Baborie A, Sampathu DM, Du Plessis D, Jaros E, Perry RH, Trojanowski JQ, Mann DM, Lee VM (2011) A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 122(1):111–113CrossRefGoogle Scholar
  6. 6.
    Kipps CM, Davies RR, Mitchell J, Kril JJ, Halliday GM, Hodges JR (2007) Clinical significance of lobar atrophy in frontotemporal dementia: application of an MRI visual rating scale. Dement Geriatr Cogn Disord 23(5):334–342CrossRefGoogle Scholar
  7. 7.
    Scheltens P, Leys D, Barkhof F, Huglo D, Weinstein HC, Vermersch P, Kuiper M, Steinling M, Wolters EC, Valk J (1992) Atrophy of medial temporal lobes on MRI in “probable” Alzheimer’s disease and normal ageing: diagnostic value and neuropsychological correlates. J Neurol Neurosurg Psychiatry 55(10):967–972CrossRefGoogle Scholar
  8. 8.
    Mori H, Yagishita A, Takeda T, Mizutani T (2007) Symmetric temporal abnormalities on MR imaging in amyotrophic lateral sclerosis with dementia. AJNR Am J Neuroradiol 28(8):1511–1516CrossRefGoogle Scholar
  9. 9.
    Harper L, Fumagalli GG, Barkhof F, Scheltens P, O'Brien JT, Bouwman F, Burton EJ, Rohrer JD, Fox NC, Ridgway GR, Schott JM (2016) MRI visual rating scales in the diagnosis of dementia: evaluation in 184 post-mortem confirmed cases. Brain 139(Pt4):1211–1225CrossRefGoogle Scholar
  10. 10.
    Tokumaru AM, Saito Y, Murayama S, Kazutomi K, Sakiyama Y, Toyoda M, Yamakawa M, Terada H (2009) Imaging-pathologic correlation in corticobasal degeneration. AJNR Am J Neuroradiol 30(10):1884–1892CrossRefGoogle Scholar
  11. 11.
    Sakurai K, Tokumaru AM, Shimoji K, Murayama S, Kanemaru K, Morimoto S, Aiba I, Nakagawa M, Ozawa Y, Shimohira M, Matsukawa N, Hashizume Y, Shibamoto Y (2017) Beyond the midbrain atrophy: wide spectrum of structural MRI finding in cases of pathologically proven progressive supranuclear palsy. Neuroradiology 59(5):431–443CrossRefGoogle Scholar
  12. 12.
    Whitwell JL, Weigand SD, Boeve BF, Senjem ML, Gunter JL, DeJesus-Hernandez M, Rutherford NJ, Baker M, Knopman DS, Wszolek ZK, Parisi JE, Dickson DW, Petersen RC, Rademakers R, Jack CR Jr, Josephs KA (2012) Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain 135(Pt3):794–806CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Keita Sakurai
    • 1
    Email author
  • Satoru Morimoto
    • 2
  • Takuya Oguri
    • 3
  • Hiroyuki Yuasa
    • 3
  • Yuto Uchida
    • 4
  • Kentaro Yamada
    • 5
  • Masahiro Muto
    • 6
  • Yufuko Saito
    • 7
  • Ikuko Aiba
    • 7
  • Masaki Takao
    • 8
  • Shohei Inui
    • 1
    • 9
  • Keiko Toyoda
    • 10
  • Asako Yamamoto
    • 1
  • Hidetsuna Utsunomiya
    • 1
  • Hiroshi Oba
    • 1
  • Aya.M Tokumaru
    • 11
  • Motoo Nakagawa
    • 12
  • Yoshio Hashizume
    • 13
  • Mari Yoshida
    • 14
  1. 1.Department of RadiologyTeikyo University School of MedicineTokyoJapan
  2. 2.Department of Physiology, School of MedicineKeio UniversityTokyoJapan
  3. 3.Department of NeurologyTosei General HospitalSetoJapan
  4. 4.Department of NeurologyToyokawa City HospitalToyokawaJapan
  5. 5.Department of NeurologyNagoya City East Medical CenterNagoyaJapan
  6. 6.Department of RadiologyNagoya City East Medical CenterNagoyaJapan
  7. 7.Department of NeurologyNational Hospital Organization Higashi Nagoya National HospitalNagoyaJapan
  8. 8.Department of Neurology and Cerebrovascular MedicineSaitama Medical University International Medical CenterHidakaJapan
  9. 9.Department of Radiology, Graduate School of MedicineThe University of TokyoTokyoJapan
  10. 10.Department of RadiologyThe Jikei University Daisan HospitalKomaeJapan
  11. 11.Department of Diagnostic RadiologyTokyo Metropolitan Medical Center of GerontologyTokyoJapan
  12. 12.Department of RadiologyNagoya City University Graduate School of Medical SciencesNagoyaJapan
  13. 13.Choju Medical InstituteFukushimura HospitalToyohashiJapan
  14. 14.Department of Neuropathology, Institute for Medical Science of AgingAichi Medical UniversityNagakuteJapan

Personalised recommendations