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Rapidly progressive dementia with severe insomnia: an unusual case of progressive supranuclear palsy mimicking dementia with Lewy bodies

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Funding

This research was supported by a fund (2021-ER1001-01) by Research of Korea Centers for Disease Control and Prevention (to Sung-Hye Park) and the Soonchunhyang University Research Fund (No. 20220802) (to Kyum-Yil Kwon).

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Authors and Affiliations

  1. Department of Neurology, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, 59 Daesagwan-ro, Yongsan-gu, Seoul, 04401, Republic of Korea

    Rae On Kim, Eun Ji Lee & Kyum-Yil Kwon

  2. Department of Pathology, Seoul National University College of Medicine, Seoul, Republic of Korea

    Seong-Ik Kim & Sung-Hye Park

Authors
  1. Rae On Kim
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  2. Eun Ji Lee
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  3. Seong-Ik Kim
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  4. Sung-Hye Park
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  5. Kyum-Yil Kwon
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Corresponding author

Correspondence to Kyum-Yil Kwon.

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Ethics 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 1975 Helsinki declaration and its later amendments or comparable ethical standards. All samples derived from the Seoul National University Hospital Brain Bank were obtained with informed consent under institutional review board-approved protocols (IRB: 1808-087-966).

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Written informed consent was obtained from the patient.

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The authors declare no competing interests.

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Supplementary information

A 65-year-old woman initially presents with mild dysarthria, mild left-handed bradykinesia, inapparent degree of saccadic slowness in vertical gaze, and mild unsteadiness on tandem gait

After one year, the patient exhibits mild saccadic slowing without limitation of vertical gaze, moderate bradykinesia in all limbs, body-part-as-object in apraxia test, and severe progressive gait disturbance with severe postural instability (first half). After three years, the patient presents with severe dysarthria, poor cooperation, and in a bedridden state, although she has no known neck or limb rigidity (second half)

Supplementary Figure 1.

Encephalography (EEG) findings of the patient. a Initial EEG findings one year after symptom onset reveal unremarkable findings. There are neither pathological slowing nor epileptiform discharges. b Follow-up EEG findings show 7-8 Hz posterior background rhythm intermittently mixed with generalized theta to delta slowing activities indicating mild cerebral dysfunction. There are no epileptiform discharges

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Kim, R.O., Lee, E.J., Kim, SI. et al. Rapidly progressive dementia with severe insomnia: an unusual case of progressive supranuclear palsy mimicking dementia with Lewy bodies. Neurol Sci 44, 2953–2955 (2023). https://doi.org/10.1007/s10072-023-06766-0

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  • DOI: https://doi.org/10.1007/s10072-023-06766-0

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