An autopsy confirmed case of progressive supranuclear palsy with predominant cerebellar ataxia
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Progressive supranuclear palsy (PSP) is characterized by early appearance of postural instability and supranuclear gaze palsy . 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 . 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) . 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,...
KeywordsProgressive Supranuclear Palsy Cerebellar Ataxia Progressive Supranuclear Palsy Neuronal Cytoplasmic Inclusion Primary Progressive Aphasia
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
Conflicts of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
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.