BackgroundProgressive supranuclear palsy(PSP)is a common type of Parkinsonism-Plus syndrome.The clinical features of PSP include vertical supranuclear palsy,parkinsonian symptoms,postural instability,falling,and cognitive impairment.Compared with primary Parkinson’s disease(PD),PSP has a lower incidence,faster progression,and early postural instability and falls,which seriously affect the quality of life of patients.The diagnosis of PSP depends on neuropathology,which is difficult to perform when the patient is alive.Currently,the diagnosis of PSP is mainly based on the 2017 diagnostic criteria of the International Movement Disorder Society(MDS).Although the clinical core symptoms are relatively clear,PSP patients are often missed or misdiagnosed as PD or other Parkinsonian syndromes.Freezing of Gait(FOG)means a sudden or transient inability to move forward effectively,usually while starting,turning,or navigating narrow aisles.It is one of the more common disabling motor disorders in PD,PSP,and other atypical Parkinsonism,causing falls and postural instability.In PD,FOG mostly appears in the late stage,while in PSP patients,FOG can appear in the early stage and even as the first symptom,with an incidence of about 53%.Therefore,FOG is one of the common symptoms of PSP,which has a significant impact on patients’ quality of life and caregivers burden by increasing the likelihood of falls.Therefore,it is important to study the clinical characteristics and related factors of FOG in PSP patients for the diagnosis and treatment of PSP.ObjectiveWe evaluated the clinical symptoms and quality of life of PSP patients.To find the factors that may be related to FOG in PSP,we compared the demographic data and clinical characteristics of PSP patients with FOG to patients without FOG.We also analyzed the effects of clinical parameters on the quality of life of PSP patients.MethodsA total of 89 outpatients and inpatients with PSP in Shandong University Qilu Hospital during the period of March 2019 to December 2021 were collected.Patients were divided into groups with and without FOG(PSP-FOG and PSP-nFOG)based on the definition of FOG and the question 13 of the MDS-UPDRS part Ⅱ:"freezing when walking".General clinical data of PSP patients including gender,age,onset age,course of disease,education level and other basic information were collected by doctors specializing in movement disorders.Corresponding scale evaluation was completed,including 2008 MDS Unified Parkinson’s Disease Rating Scale(MDS-UPDRS),Progressive Supranuclear Palsy Rating Scale(PSP-RS),Montreal Cognitive Assessment Scale(MoCA),Mini-Mental State Examination(MMSE),Hamilton Anxiety Scale(HAMA),Hamilton Depression Scale(HAMD),Freezing of Gait-Questionnaires(FOG-Q)and the Parkinson’s Disease Questionnaires 39(PDQ-39).The general conditions and clinical parameters of PSP-FOG and PSP-nFOG patients were compared,the correlation between FOG-Q and other clinical variables in the PSP-FOG group were analyzed,and multiple linear regression analysis was used to evaluate the factors that may affect the quality of life of patients with PSP.Statistical data were analyzed using SPSS 25.0.Results1.Demographic characteristics of PSP-FOG group and PSP-nFOG groupA total of 89 patients with PSP were enrolled in this study.The mean age of diagnosis was 65.63 ± 6.37 years,the mean age of onset was 61.79±6.28 years,and the mean course of disease was 3.85 ± 1.91 years.There are 52 patients(58.4%)in the PSP-FOG group and 37 patients(41.6%)in the PSP-nFOG group.There is no significant differences in gender,age,onset age and years of education between the two groups(P>0.05).But the disease duration of the PSP-FOG group(median 4.00)was longer than that of the PSP-nFOG group(median 3.00),with statistical difference(P<0.05).2.Single factor analysis of FOG in PSP2.1 The MDS-UPDRS scores of the two groups showed no significant difference in total score,part Ⅰ and part Ⅱ scores(P>0.05),while part Ⅲ scores of the PSP-FOG group were higher than that of the PSP-nFOG group(P<0.05).2.2 PSP-RS scores of the two groups were compared,and there were no significant differences in part Ⅰ,Ⅱ,and Ⅲ scores(P>0.05),while PSP-RS total score and part Ⅳ,Ⅴ,and Ⅵ scores of the PSP-FOG group were higher than those of the PSP-nFOG group(P<0.05).The results showed that the scores of limb rigidity,leg agility,gait and postural stability in PSP-FOG group were higher than those of the PSP-nFOG group,and the differences were statistically significant(P<0.05).2.3 The MOCA and MMSE scores of the two groups showed no significant difference(P>0.05),but the visual space and executive function score and abstraction score in MOCA of the PSP-FOG group were significantly lower than that of the PSP-nFOG group,the differences were statistically significant(P<0.05).In MMSE,the language function score of the PSP-FOG group was significantly lower than that of the PSP-nFOG group,with statistical significance(P<0.05).2.4 HAMA and HAMD scores of the two groups were compared,and there was no significant difference in HAMA scores between the two groups(P>0.05),but the HAMD scores of the PSP-FOG group were higher than that of the PSP-nFOG group,the difference was statistically significant(P<0.05).3.Multifactor analysis of FOG in PSPLogistic regression analysis was performed using eight factors as independent variables,including course of disease,PSP-RS(single score of limb stiffness,toe tapping,gait and postural stability),MOCA visual space and executive function and part score,MMSE language function score,and FOG as dependent variable.Results showed that gait score in PSP-RS and abstract score in MOCA entered the regression equation,and the OR values were 2.131(1.204-3.772)and 0.402(0.200-0.807).4.Analysis of related factors of FOG-Q in PSP-FOG groupCorrelation analysis showed that FOG-Q score was positively correlated with disease course(r=0.306,P=0.027),PSP-RS-Ⅳ score(R=0.295,P=0.034),PSP-RS-Ⅴscore(R=0.496,P<0.001)and PDQ-39 score(r=0.383,P=0.005).And FOG-Q score was not correlated with age,PSP-RS,MOCA,HAMA and HAMD scores.5.Comparison of PDQ-39 scores between PSP-FOG group and PSP-nFOG groupThe PDQ-39 score of the PSP-FOG group was significantly higher than that of the PSP-nFOG group(P<0.05).6.Multiple linear regression analysis of PDQ-39The results of multiple stepwise linear regression analysis showed that PSP-RS,HAMD and FOG-Q scores were the influencing factors of PDQ-39 score.The fitting linear regression model was significant(R2=0.521,P<0.001).Conclusion1.Compared with PSP patients without FOG,PSP patients with FOG had longer duration and more severe symptoms,and both motor and non-motor symptoms were more severe than PSP patients without FOG.2.The course of disease,eye movement disorder,stiffness,abnormal posture and gait,cognitive function(visual space and executive function,abstract thinking,language ability),and depression were all correlated with FOG in PSP patients,and the course of disease,eye movement disorder,and limb stiffness were positively correlated with FOG severity.Gait disorder and abstract thinking disorder were independent risk factors of FOG in PSP patients.3.Freezing of gait,PSP-RS score and depression were independent risk factors for quality of life in PSP patients.It is very important to detect FOG early and give rehabilitation guidance and adjust drugs. |