Please use this identifier to cite or link to this item: https://doi.org/10.14802/jmd.16059
Title: Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos.
Authors: Xu, Zheyu 
Lim, Tchoyoson CC 
Au, Wing Lok 
Tan, Louis CS 
Keywords: Progressive supranuclear palsy
ataxia
Issue Date: May-2017
Publisher: The Korean Movement Disorder Society
Citation: Xu, Zheyu, Lim, Tchoyoson CC, Au, Wing Lok, Tan, Louis CS (2017-05). Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos.. J Mov Disord 10 (2) : 87-91. ScholarBank@NUS Repository. https://doi.org/10.14802/jmd.16059
Abstract: Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.
Source Title: J Mov Disord
URI: https://scholarbank.nus.edu.sg/handle/10635/213576
ISSN: 2005940X
20934939
DOI: 10.14802/jmd.16059
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