scholarly journals The Cold Hand Sign in Multiple System Atrophy: Frequency-Associated Factors and Its Impact on Survival

2021 ◽  
Vol 13 ◽  
Author(s):  
Bei Cao ◽  
Yan Liang ◽  
Ling-Yu Zhang ◽  
Yan-Bing Hou ◽  
Ru-Wei Ou ◽  
...  

Background: Few studies have focused on the cold hand sign (CHS), a red flag symptom, in multiple system atrophy (MSA).Objective: This study aimed to investigate the frequency and correlative factors of CHS in patients with MSA and the impact of its early occurrence on the survival of these patients.Methods: A total of 483 patients with MSA were enrolled in this study, and the motor and non-motor symptoms between patients with MSA with and without CHS were compared. Moreover, patients with disease duration ≤ 3 years at baseline were followed, and the association between CHS and survival of patients with MSA was examined.Results: The frequencies of CHS in patients with MSA were 20, 15.4, and 25.3% in MSA, MSA-parkinsonian subtype (MSA-P), and MSA-cerebellar subtype (MSA-C), respectively. Higher Unified Multiple System Atrophy Rating Scale (UMSARS) scores and higher Non-Motor Symptom Scale (NMSS) scores at baseline were associated with CHS in MSA. CHS was associated with shorter survival after adjusting for baseline diagnosis subtype, age at onset, sex, orthostatic hypotension, disease duration, autonomic onset, UMSARS total score, and NMSS score (p = 0.001; HR = 3.701; 95% CI = 1.765–7.760).Conclusion: CHS is not rare in patients with MSA. Greater disease severity and more severe non-motor symptoms were associated with CHS in patients with MSA. Patients with early occurrence of CHS had a poor prognosis.

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ling Yu Zhang ◽  
Bei Cao ◽  
Qian-Qian Wei ◽  
Ru Wei Ou ◽  
Bi Zhao ◽  
...  

Abstract Background Camptocormia is common in patients with multiple system atrophy (MSA). The current study was aimed at assessing the frequency of camptocormia and its related factors in MSA patients with different disease durations. Also, the impact of camptocormia on disability was evaluated. Methods A total of 716 patients were enrolled in the study. They were classified into three groups based on disease duration (≤ 3, 3–5, ≥ 5 years). Specific scales were used to evaluate the motor and non-motor symptoms. Disease severity was assessed using the Unified Multiple System Atrophy Rating Scale (UMSARS). The binary logistic regression model was used to explore the factors related to camptocormia. To analyze the impact of camptocormia on disability in patients with disease duration less than 5 years, propensity score matching (PSM) and stratified Cox regression analysis were used. Results In the current study, we found that the frequency of camptocormia was 8.9, 19.7 and 19.2% when the disease duration was ≤3, 3–5, ≥ 5 years, respectively. In the disease duration ≤3 years group, we found that MSA-parkinsonian subtype (MSA-P) (OR = 2.043, P = 0.043), higher total UMSARS score (OR = 1.063, P < 0.001), older age of onset (OR = 1.047, P = 0.042), and lower score on the frontal assessment battery (FAB) (OR = 0.899, P = 0.046) were associated with camptocormia. Only greater disease severity was associated with camptocormia in the group of patients with disease duration 3–5 years (OR = 1.494, P = 0.025) and in the group of patients with disease duration ≥5 years (OR = 1.076, P = 0.005). There was no significant impact of camptocormia on disability in patients with a disease duration of < 5 years (HR = 0.687, P = 0.463). Conclusion The frequency of camptocormia increased with prolonged disease duration. Disease severity was related to camptocormia at different stages of the disease. The MSA-P subtype, older age of onset, and lower FAB score were associated with camptocormia in the early stage of the disease.


2022 ◽  
Vol 12 ◽  
Author(s):  
Hui Wang ◽  
Xiangdong Tang ◽  
Junying Zhou ◽  
Yanming Xu

Objectives: Excessive daytime sleepiness (EDS) in multiple system atrophy (MSA) has received scant attention in the literature, thus the present cross-sectional study aimed to investigate the prevalence of EDS and its potential risk factors among Chinese patients with MSA.Methods: A total of 66 patients with MSA (60.6% males) were consecutively recruited. Eighteen patients (27.3%, 13 men) with Epworth Sleepiness Scale score &gt;10 were defined as having EDS. Demographic, motor [Unified Multiple-System Atrophy (UMSARS)] and non-motor symptoms [Non-Motor Symptoms Scale (NMSS)], and sleep parameters [polysomnography (PSG)] were compared between patients with MSA with and without EDS. A logistic regression analysis was used to calculate the risk factors of EDS in patients with MSA.Results: There were no significant differences in age, sex, MSA onset age, disease duration, MSA sub-type, and motor symptom severity between MSA patients with and without EDS. However, compared with the MSA patients without EDS, their counterparts with EDS had higher scores of NMSS (65.3 ± 23.1 vs. 43.4 ± 25.3, P = .0002), Hamilton Anxiety (HAMA) [15.3 (10.3–20.0) vs. 9.5 (3.0–15.0), P = 0.006], Hamilton Depression (HAMD) [13.7 (12.5–17.8) vs. 9.0 (4.0–13.0), P = 0.015], and Fatigue Severity Scale (FSS) [29.8 (17.3–47.8) vs. 18.7 (10.3–21.8), P = 0.040]. Conversely, the patients with EDS had lower score of Mini-Mental State Examination (MMSE) [23.3 (20.3–27.0) vs. 25.7 (22.0–29.0), P = 0.023]. Similarly, there was a significantly lower percentage of N3 sleep (%) [0.3 (0–0) vs. 2.0 (0–0), P = 0.007] and a higher apnea-hypopnea index (AHI/h) [30.5 (14.5–47.8) vs. 19.3 (5.0–28.7), P = 0.034] in patients with EDS. After adjusting for age, sex, disease duration, MSA sub-type, and UMSARS score, the odds ratio (OR) (95% CI) of EDS was higher while increasing scores in FSS [1.06 (1.02–1.11)], HAMA [1.16 (1.04–1.28)], HAMD [1.13 (1.02–1.25)], NMSS [1.04 (1.01–1.07)], and AHI [1.03 (1.00–1.10)]. The OR of EDS was lower while the MMSE score was increasing [0.85 (0.72–1.00)].Conclusions: The presence and severity of EDS may be significantly associated with the non-motor dysfunction, including fatigue, anxiety, depression, cognitive dysfunction, and sleep-related breathing disorder, but not with the motor dysfunction in MSA.


2022 ◽  
pp. 1-11
Author(s):  
Zuzana Forejtová ◽  
Tereza Serranová ◽  
Tomáš Sieger ◽  
Matěj Slovák ◽  
Lucia Nováková ◽  
...  

Abstract Background Patients with functional neurological disorders (FND) often present with multiple motor, sensory, psychological and cognitive symptoms. In order to explore the relationship between these common symptoms, we performed a detailed clinical assessment of motor, non-motor symptoms, health-related quality of life (HRQoL) and disability in a large cohort of patients with motor FND. To understand the clinical heterogeneity, cluster analysis was used to search for subgroups within the cohort. Methods One hundred fifty-two patients with a clinically established diagnosis of motor FND were assessed for motor symptom severity using the Simplified Functional Movement Disorder Rating Scale (S-FMDRS), the number of different motor phenotypes (i.e. tremor, dystonia, gait disorder, myoclonus, and weakness), gait severity and postural instability. All patients then evaluated each motor symptom type severity on a Likert scale and completed questionnaires for depression, anxiety, pain, fatigue, cognitive complaints and HRQoL. Results Significant correlations were found among the self-reported and all objective motor symptoms severity measures. All self-reported measures including HRQoL correlated strongly with each other. S-FMDRS weakly correlated with HRQoL. Hierarchical cluster analysis supplemented with gap statistics revealed a homogenous patient sample which could not be separated into subgroups. Conclusions We interpret the lack of evidence of clusters along with a high degree of correlation between all self-reported and objective measures of motor or non-motor symptoms and HRQoL within current neurobiological models as evidence to support a unified pathophysiology of ‘functional’ symptoms. Our results support the unification of functional and somatic syndromes in classification schemes and for future mechanistic and therapeutic research.


2019 ◽  
Vol 19 (5-6) ◽  
pp. 238-243 ◽  
Author(s):  
Yu Jin Jung ◽  
Han-Joon Kim ◽  
Dallah Yoo ◽  
Ji-Hyun Choi ◽  
Jin Hee Im ◽  
...  

Background: Multiple system atrophy (MSA) patients pre­sent a variety of symptoms other than autonomic dysfunctions, parkinsonism, and cerebellar ataxia. The aim of this study was to evaluate the frequency of various motor and non-motor symptoms including so-called “red flags” in patients with early MSA and to determine whether the frequency of these symptoms was different between the parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes. Methods: Sixty-one probable or possible MSA patients with disease duration of 3 years or less were included. Patients were classified into MSA-P, MSA-C, and MSA-PC. The frequency of 13 features including various motor and non-motor symptoms that commonly occur in MSA was assessed. Results: Dysarthria was the most prevalent feature (98.4%) followed by sexual dysfunction (95.1%). Probable REM sleep behavior disorder was present in 90.2%. The frequency of constipation (82.0%), dysphagia (68.9%), and snoring (70.5%) was also high. Stridor was present in 42.6% and more common in MSA-C than in MSA-P. Conclusions: Increasing awareness of various motor and non-motor symptoms may assist clinicians to make an early, accurate diagnosis and to improve management of patients with MSA. We suggest that the diagnostic accuracy can be improved if these features are appropriately reflected in the new diagnostic criteria for MSA.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yi-Chieh Chen ◽  
Rou-Shayn Chen ◽  
Yi-Hsin Weng ◽  
Ying-Zu Huang ◽  
Chiung Chu Chen ◽  
...  

AbstractNonmotor symptoms (NMSs) cause major burden in patients with Parkinson’s disease (PD). Previous NMSs progression studies mostly focused on the prevalence. We conducted a longitudinal study to identify the progression pattern by the severity. PD patients recruited from the outpatient clinics of a tertiary medical center were evaluated by the Unified Parkinson's Disease Rating Scale and Non-Motor Symptoms Scale (NMSS). A retrospective study with three-step analysis was performed. Step 1, the NMSs severity was compared among patients stratified by disease duration every 2 years up to 10 years. Step 2, patients with repeated tests in 2 years were categorized into 4 groups by the diseased duration of every 5 years. Step 3, the NMSS score changes in 6 years follow-up were determined, and the dosage of anti-PD drugs was compared to the NMSs severity changes. 676 patients completed the step 1 analysis, which showed a trend of NMSs worsening but not significant until the disease duration longer than 4–6 years. Furthermore, the severity did not change between repeated evaluations in 2 years in all patients. The progression became apparent after 6 years. Individual symptoms had different progression patterns and the increment of medications was independent to NMSs evolution. We demonstrated the NMSs severity progression in Taiwanese PD patients and the independence of the medications and NMSs progression.


2021 ◽  
Vol 12 ◽  
Author(s):  
Sha Zhu ◽  
Min Zhong ◽  
Yu Bai ◽  
Zhuang Wu ◽  
Ruxin Gu ◽  
...  

Background and Purpose: The unilateral onset and persistent asymmetry of motor symptoms are important characteristics of Parkinson's disease (PD). By using scales and wearable sensors, this study explored whether motor symptom laterality could affect non-motor symptom and gait performance.Methods: A total of 130 right-handed patients with PD were enrolled in our study and were divided into two groups according to the side of predominant motor symptom presentation by using the Unified Parkinson's Disease Rating Scale part III. We measured the non-motor symptoms with the Non-motor symptoms Scale, sleep quality with the Parkinson's Disease Sleep Scale and Pittsburgh sleep quality index, cognitive function with the Mini-mental State Examination and Montreal Cognitive Assessment, quality of life with the Parkinson's Disease Questionnaire-39, and the severity of anxiety and depression with the Hamilton Anxiety Scale and Hamilton Depression Scale, respectively. All participants underwent the instrumented stand and walk test, and gait data were collected using a set of JiBuEn gait analysis system.Results: We observed that left-dominant symptom PD patients (LPD) were associated with a greater impairment of sleep quality than right-dominant symptom PD patients (RPD). We found no difference between LPD and RPD in terms of gait performance. However, compared with the severe asymmetry RPD patients (RPD-S), severe asymmetry LPD patients (LPD-S) showed a shorter stride length and decreased range of motion of hip joints.Conclusions: In this study, LPD was associated with a more severe sleep-related dysfunction than RPD. In addition, LPD-S exhibited more gait impairments than RPD-S. Considering that motor symptom laterality may affect the non-motor symptom and gait performance, it should be taken into account when evaluating and treating PD patients.


2020 ◽  
Vol 26 (1) ◽  
pp. 25-29
Author(s):  
Ana Cristina Tillmann ◽  
Alessandra Swarowsky ◽  
Alexandro Andrade ◽  
Jéssica Moratelli ◽  
Leonessa Boing ◽  
...  

ABSTRACT Introduction: People with Parkinson's disease constantly have low levels of physical activity. Dancing has become increasingly important for treating the disease and can help improve non-motor symptoms. Objective: To analyze the influence of Brazilian samba on the non-motor symptoms of PD according to TD and PGID subtypes. Methods: A 12-week, non-randomized clinical trial, through comparison with a control group. The 23 individuals who agreed to participate in the activities formed the experimental group (EG) and the 24 individuals who opted not to participate in the Brazilian samba classes comprised the control group (CG). A questionnaire was applied, composed of validated instruments. Mini Mental State Examination – MMSE; HY – Disability Scale; Unified Parkinson's Disease Rating Scale – UPDRS 1 and total values; Parkinson's Disease Questionnaire – PDQ-39, Parkinson's Disease Sleep Scale – PDSS; Beck Depression Inventory – BDI; Fatigue Severity Scale – FSS and Magnitude of Perceived Changes. Results: After the twelve weeks of intervention, it was observed that the EG showed improvement in the scores of all the tests. The comparison between groups, however, indicated a significant difference in the post-UPDRS1 period in which the EG presented improvement in cognitive impairment, while the CG presented a deficit in these values. The results of the division between disease subtypes show a greater change in the values between individuals of the TD group, when comparing the EG with the CG. For the EG, the greatest difference between pre- and post- intervention was fatigue. Conclusion: There was a positive trend in all the variables studied after the application of the protocol. This demonstrates that interventions such as dance may have greater effects on non-motor symptoms, depending on the expected progression of the disease. The scarcity of studies that use this approach in their analyses may explain the lack of evidence in this symptomatology related to dance. Level of evidence II; Therapeutic studies – Investigating the results of treatment.


2019 ◽  
Vol 90 (3) ◽  
pp. e24.2-e24
Author(s):  
DV Wamelen ◽  
A Podlewska ◽  
V Leta ◽  
A Rizos ◽  
K Chaudhuri

ObjectivesTo determine the effect of the change of season on non-motor symptoms in Parkinson’s disease (PD).DesignCross-sectional retrospective study.Subjects364 consecutive Parkinson’s patients in the London area taking part in the Non-motor Longitudinal International Study (NILS) study at King’s College Hospital London between November 2011 and April 2018.MethodsParticipants were divided into three groups based on their assessment date, using a simplified ecological seasonal model:November until February;March until 15 June;16 June until October.The primary outcome was a seasonal difference in non-motor symptom scale (NMSS) total scores and the secondary outcomes were NMSS subscores. Outcomes were adjusted for multiple testing using formal Bonferroni correction.Results102 patients were allocated to group 1, 105 patients to group 2 and 157 patients to group 3. Seasonal differences were present in NMSS total scores (p=0.042), especially between groups 1 and 3 (p=0.037). Differences were also present for NMSS domain 1 (cardiovascular and falls; p=0.004), 2 (sleep and fatigue; p=0.049), 4 (hallucinations; p=0.003) and 9 (miscellaneous; p=0.031). Within domain 9 there was only a significant difference for question 28 (smell; p=0.008).ConclusionsThese results indicate that non-motor symptoms in Parkinson’s fluctuate throughout the year with lowest scores in the summer months and highest in the winter months. These variations need to be taken into account to avoid inappropriate changes in medication regimes.


2020 ◽  
Vol 10 (4) ◽  
pp. 1817-1825
Author(s):  
Pritha Ghosh ◽  
Paola Imbriani ◽  
Nicoletta Caputi ◽  
Silvia Natoli ◽  
Tommaso Schirinzi ◽  
...  

Background: Pain is a disabling and often underestimated non-motor symptom (NMS) detrimentally affecting the quality of life of patients with Parkinson’s disease (PD). Objective: Here, we conducted a cross-sectional, observational international study on 167 patients with idiopathic PD in order to analyze the potential relationship between pain and other NMS. Methods: Subjects were assessed with the Unified Parkinson’s Disease Rating Scale (UPDRS) part III, Hoehn and Yahr (H&Y) stage, King’s Parkinson’s Disease Pain Scale (KPPS), Brief Pain Inventory (BPI), Non-Motor Symptoms Scale (NMSS), and Beck Depression Inventory (BDI). Spearman’s rank correlation coefficient, multiple regression and multiple index-based clustering algorithms were used for data analysis. Results: The prevalence of pain was 88.6%, was not correlated with age, motor severity (UPDRS part III) or disease duration, whereas a weak correlation with female gender and H&Y stage >2.5 was found. Multiple NMS correlated significantly with pain. Specifically, sleep disturbance had the strongest correlation with pain, followed by depression, gastrointestinal and cardiovascular disturbances. Further analyses showed that sleep and cardiovascular disturbance were independently associated with pain, and that these symptoms clustered together in a subset of PD patients. The relationship between pain, sleep and dysautonomia persisted independently from dopamine replacement therapy. Conclusion: Our study suggests that sleep disruption and cardiovascular disturbance are associated with pain in PD, and possibly identifies a specific subtype within PD patients with pain. Our data also indicate that sleep disruption, pain and dysautonomia may have a common pathophysiology, possibly involving non-dopaminergic pathways.


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