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Beyond the midbrain atrophy: wide spectrum of structural MRI finding in cases of pathologically proven progressive supranuclear palsy

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Abstract

Purpose

Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes.

Methods

With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated.

Results

In addition to typical symptoms, PSP patients can exhibit atypical symptoms including levodopa-responsive parkinsonism, pure akinesia, non-fluent aphasia, corticobasal syndrome, and predominant cerebellar ataxia. As well as clinical symptoms, the degree of midbrain atrophy, a well-known imaging hallmark, is not consistent in atypical PSP cases. This fact has important implications for the limitation of midbrain atrophy as a diagnostic imaging biomarker of PSP pathology. Additional evaluation of other imaging findings including various regional atrophies of the globus pallidus, frontal lobe, cerebral peduncle, and superior cerebellar peduncle is essential for the diagnosis of atypical PSP cases.

Conclusion

It is necessary for radiologists to recognize the wide clinical and radiological spectra of typical and atypical PSP cases.

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Authors and Affiliations

  1. Department of Diagnostic Radiology, Tokyo Metropolitan Medical Center of Gerontology, 35-2 Sakaecho, Itabashi-ku, Tokyo, 173-0015, Japan

    Keita Sakurai, Aya M Tokumaru & Keigo Shimoji

  2. Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan

    Shigeo Murayama, Kazutomi Kanemaru & Satoru Morimoto

  3. Department of Neurology, National Hospital Organization Higashi Nagoya National Hospital, Nagoya, Japan

    Ikuko Aiba

  4. Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan

    Motoo Nakagawa, Yoshiyuki Ozawa, Masashi Shimohira & Yuta Shibamoto

  5. Department of Neurology and Neuroscience, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan

    Noriyuki Matsukawa

  6. Choju Medical Institute, Fukushimura Hospital, Toyohashi, Japan

    Yoshio Hashizume

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  1. Keita Sakurai
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  2. Aya M Tokumaru
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  3. Keigo Shimoji
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  4. Shigeo Murayama
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  13. Yuta Shibamoto
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Corresponding author

Correspondence to Keita Sakurai.

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Funding

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; and Kakenhi Kiban C, 16K10305: AMT).

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Sakurai, K., Tokumaru, A.M., Shimoji, K. et al. Beyond the midbrain atrophy: wide spectrum of structural MRI finding in cases of pathologically proven progressive supranuclear palsy. Neuroradiology 59, 431–443 (2017). https://doi.org/10.1007/s00234-017-1812-4

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  • DOI: https://doi.org/10.1007/s00234-017-1812-4

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