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Journal of Neurology

, Volume 263, Issue 12, pp 2540–2543 | Cite as

An autopsy confirmed case of progressive supranuclear palsy with predominant cerebellar ataxia

  • Myung Jun Lee
  • Jeong Hee Lee
  • Baik-Kyun Kim
  • Jae-Hyeok Lee
  • Young Min Lee
  • Seong-Jang Kim
  • Jin-Hong Shin
  • Myung-Jun Shin
  • Jae Woo Ahn
  • Suk Sung
  • Kyung-Un Choi
  • Dae Soo Jung
  • Na-Yeon Jung
  • William W. Seeley
  • Gi Yeong HuhEmail author
  • Eun-Joo KimEmail author
Letter to the Editors

Dear Sirs,

Progressive supranuclear palsy (PSP) is characterized by early appearance of postural instability and supranuclear gaze palsy [1]. However, considerable clinical variability has been reported, such as Richardson’s syndrome, PSP-parkinsonism, PSP-pure akinesia with gait freezing, behavioral variant frontotemporal dementia, and non-fluent/agrammatic variant primary progressive aphasia [1]. Kanazawa et al. recently defined a new clinical subtype of PSP with cerebellar ataxia as the initial and prominent symptom, called PSP with predominant cerebellar ataxia (PSP-C) [2]. Here, we present our observation of a pathologically confirmed PSP-C patient, who was initially diagnosed with multiple system atrophy-cerebellar type (MSA-C).

A 64-year-old man had been referred to our hospital for frequent falls and disequilibrium. His symptoms started with orthostatic dizziness and gait unsteadiness at the age of 59. Dysarthria, hitting and kicking his wife during sleep, severe snoring,...

Keywords

Progressive Supranuclear Palsy Cerebellar Ataxia Progressive Supranuclear Palsy Neuronal Cytoplasmic Inclusion Primary Progressive Aphasia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

This study was supported by the Original Technology Research Program for Brain Science through the National Research Foundation of Korea (NRF) funded by the Korean government (MSIP) (No. 2014M3C7A1064752), and clinical research grant from Pusan National University Hospital 2015. We thank our patient and his family for donating brain to the Pusan National University Hospital Brain Bank to contribute to dementia research.

Compliance with ethical standards

Financial disclosure

None reported.

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard

The study has been approved by the institutional review boards and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Supplementary material

415_2016_8303_MOESM1_ESM.docx (1.6 mb)
Supplementary material 1 (DOCX 1613 kb)

References

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Myung Jun Lee
    • 1
  • Jeong Hee Lee
    • 2
  • Baik-Kyun Kim
    • 3
  • Jae-Hyeok Lee
    • 3
  • Young Min Lee
    • 4
  • Seong-Jang Kim
    • 5
  • Jin-Hong Shin
    • 3
  • Myung-Jun Shin
    • 6
  • Jae Woo Ahn
    • 2
  • Suk Sung
    • 7
  • Kyung-Un Choi
    • 8
  • Dae Soo Jung
    • 1
  • Na-Yeon Jung
    • 3
  • William W. Seeley
    • 9
  • Gi Yeong Huh
    • 10
    Email author
  • Eun-Joo Kim
    • 1
    Email author
  1. 1.Department of Neurology, Pusan National University HospitalPusan National University School of Medicine and Medical Research InstituteBusanRepublic of Korea
  2. 2.Department of PathologyPusan National University Yangsan HospitalYangsanSouth Korea
  3. 3.Department of Neurology, Research Institute for Convergence of Biomedical Science and TechnologyPusan National University Yangsan HospitalYangsanSouth Korea
  4. 4.Department of Psychiatry, Pusan National University HospitalPusan National University School of Medicine and Medical Research InstituteBusanSouth Korea
  5. 5.Department of Nuclear Medicine, Pusan National University HospitalPusan National University School of Medicine and Medical Research InstituteBusanSouth Korea
  6. 6.Department of Rehabilitation Medicine, Pusan National University HospitalPusan National University School of Medicine and Medical Research InstituteBusanSouth Korea
  7. 7.Department of AnatomyPusan National University School of Medicine and Medical Research InstituteYangsanSouth Korea
  8. 8.Department of Pathology, Pusan National University HospitalPusan National University School of Medicine and Medical Research InstituteBusanSouth Korea
  9. 9.Department of Neurology, Memory and Aging CenterUniversity of CaliforniaSan FranciscoUSA
  10. 10.Department of Forensic MedicinePusan National University School of Medicine and Medical Research InstituteYangsanSouth Korea

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