Progressive supranuclear palsy presenting with hyperkinetic movement disorder and hemiplegic dystonia: a case report

Abstract

Background: Progressive supranuclear palsy (PSP) is a progressive neurodegenerative brain disease which has been rarely described in association with hyperkinetic symptoms. Here, we report a case of PSP that was presented with hyperkinetic movement disorder, hemiplegic dystonia, and other clinical features that overlap with behavioral variant frontotemporal dementia (bvFTD) and corticobasal syndrome (CBS).

Case presentation: A 63-year-old female presented to our hospital with a history of frontal lobe symptoms, impaired cognition, hyperkinetic movement disorders, dystonia, and frequent falls. Her magnetic resonance imaging (MRI) scan showed atrophy of midbrain and right temporal lobe. [¹⁸F]FDG PET result revealed reduced 18F-FDG uptake with obvious laterality (right > left). [¹⁸F]THK5317 PET scan showed evident increased uptake in the brain stem and basal ganglia. Treatment with Tiapride significantly improved hyperkinetic symptoms, but other motor symptoms were not alleviated. Three years later, the patient could hardly walk even with assistance.

Conclusion: PSP can present hyperkinetic movement disorders and asymmetry in image that widen the existing phenotypic spectrum.

Keywords:

• Progressive supranuclear palsy
• hyperkinetic movement disorder
• hemiplegic dystonia
• PET

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

Beijing Municipal Administration of Hospitals’Mission Plan, Code: SML20150803. Beijing Municipal Science & Technology Commission No. Z171100000117013. The National Key R&D Program of China, No. 2018YFC1312001, 2017YFC0840105.