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.
Ataxia ; Atrophy ; Cerebellar Ataxia* ; Cerebellar Diseases ; Diagnosis ; Disease Progression ; Humans ; Mesencephalon ; Neuroimaging ; Paralysis* ; Phenotype ; Retrospective Studies ; Supranuclear Palsy, Progressive

Objective To construct a comprehensive evaluation model of long-term care for the elderly based on the situation of our country,which is suitable for the family,community and the pension service organization. Methods The model index item pool (7 one class indexes and 133 two class indexes) formed by literature research, theoretical extraction and qualitative interview were taken as the research object.To screen the model parameters by using the Delphi method,then formed a index system of hierarchical comprehensive evaluation model of long term care for the elderly. Results 25 experts were from 10 provinces of a total of 23 Medical Colleges and affiliated hospital,positive coefficients for the first round of 83.33% and the second round of 100.00%,the average value of the two authority coefficients were both >0.90, the coordination coefficient of the evaluation index for the first round of 0.221 and the second round of 0.266(both P<0.05).The primary model index system consists of 7 first level indicators,a total of 13 secondary indicators.Among them,the"daily life ability","cognitive ability","falling risk"and"pressure sore risk" evaluation were using very mature scales, the "medical care project", "abnormal performance/symptom" and "self care knowledge needs" evaluation were independently established in this study. Conclusions Preliminary construction process of the index system for the comprehensive evaluation model of long term care for the elderly was rigorous and had better theoretical support,and the Delphi method experts had higher degree of attention and participate in the research content, experts had good representativeness,can effectively guarantee the reliability of the selected items.