Vestibular symptoms as the presenting feature of progressive supranuclear palsy

2018 
Abstract First described in 1964, progressive supranuclear palsy (PSP) is a chronic, sporadic, progressive neurodegenerative tauopathy. Substantial phenotypic variability inherent in PSP confers difficulty to diagnosis. Although the classic presentation, termed PSP-Richardson’s syndrome, has been well described, additional variants of PSP are increasingly emerging. Phenotypes described to date include PSP-parkinsonism, PSP-pure akinesia with gait freezing, PSP-corticobasal syndrome or PSP-progressive non-fluent aphasia. However, there has been scant description of vestibular features in PSP. Herein, we report three similar cases with ‘probable PSP’ who presented with a poorly defined vestibular syndrome and early falls, highlighting an uncommon and as yet under-recognised, vestibular presentation of PSP. Further description of the natural history of this unusual vestibular presentation of PSP may assist in earlier diagnosis and prevent mismanagement of such patients.
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