Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos

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.

Spectrum and burden of movement disorder conditions in a tertiary movement disorders centre--a 10-year trend

<p><b>INTRODUCTION</b>The precise burden of movement disorder conditions in our movement disorders centre is unclear. This study investigated the clinical burden of the Movement Disorders Clinic (MDC) in National Neuroscience Institute (NNI) over 10 years, aiming to identify the burden and spectrum of movement disorders conditions, to facilitate future resource allocation.</p><p><b>MATERIALS AND METHODS</b>We identified all patient visits from January 2002 to December 2011 at MDC from the Movement Disorders (MD) database using a standardised data collection form.</p><p><b>RESULTS</b>There was a linear increase in the clinical burden of MDC during this period. Parkinsonism comprised 71.6% of this clinical burden of which 84.8% were Parkinson's disease (PD) patients. Dividing the incident cases of MD conditions into two 5 years' blocks, the proportion of PD cases had not changed. There was significant increase in time to diagnosis for PD, hemifacial spasm and dystonia.</p><p><b>CONCLUSION</b>There was nearly 4-fold increase in the burden of movement disorders conditions in our tertiary condition within a decade. However, we did not find increasing proportion of PD cases which would be in line with an ageing population. This could be due to the fact that we are still in the early stages of an ageing population and we postulate that this proportion will go up in the future. The increased time to diagnosis may indicate increasing waiting time to see a movement disorders specialist and that current outreach effort to promote awareness may not be reaching its target audience. The upward trend of clinical burden indicates a need for increased resource allocation to cope with demand for movement disorders services.</p>

Is EQ-5D a valid quality of life instrument in patients with Parkinson's disease? A study in Singapore

<p><b>INTRODUCTION</b>The purpose of the present study was to evaluate the validity of the EQ-5D in patients with Parkinson's disease (PD) in Singapore.</p><p><b>MATERIALS AND METHODS</b>In a cross-sectional survey, patients with PD completed English or Chinese version of the EQ-5D, the 8-item Parkinson's disease questionnaire (PDQ-8), and questions assessing socio-demographic and health characteristics. Clinical data were retrieved from patients' medical records. The validity of the EQ-5D was assessed by testing a-priori hypotheses relating the EQ-5D to the PDQ-8 and clinical data.</p><p><b>RESULTS</b>Two hundred and eight PD patients (English speaking: 135) participated in the study. Spearman correlation coefficients between the EQ-5D and PDQ-8 ranged from 0.25 to 0.75 for English-speaking patients and from 0.16 to 0.67 for Chinese-speaking patients. By and large, the EQ-5D scores were weakly or moderately correlated with Hoehn and Yahr stage (correlation coefficients: 0.05 to 0.43), Schwab and England Activities of Daily Living score (correlation coefficients: 0.10 to 0.60), and duration of PD (correlation coefficients: 0.16 to 0.43). The EQ-5D index scores for patients with dyskinesia or "wearing off" periods were significantly lower than those without these problems. The EQ-5D Visual Analog Scale (EQ-VAS) scores also differed for English-speaking patients with deferring dyskinesia, "wearing off" periods, or health transition status; however, such differences were not observed in patients who completed the survey in Chinese.</p><p><b>CONCLUSIONS</b>The EQ-5D questionnaire appears valid for measuring quality of life in patients with PD in Singapore. However, the validity of EQ-VAS in Chinese-speaking patients with PD should be further assessed.</p>