Progression of Motor and Non-Motor Symptoms in Multiple System Atrophy: A Prospective Study from the Catalan-MSA Registry

2021 ◽  
pp. 1-10
Author(s):  
Alexandra Pérez-Soriano ◽  
Darly M. Giraldo ◽  
Jose Ríos ◽  
Esteban Muñoz ◽  
Yaroslau Compta ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Neurodegenerative Disorder ◽  
Daily Activities ◽  
Motor Symptoms ◽  
Clinical Scales ◽  
A Prospective Study ◽  
Non Motor Symptoms ◽  
Significant Motor ◽  
Sleep Difficulties

Background/Objective: Multiple system atrophy (MSA) is a highly debilitating, rare neurodegenerative disorder with two clinical motor variants (parkinsonian or MSA-P and cerebellar or MSA-C). There is a wide span of motor and non-motor symptoms (NMS) that progress over time. We studied the cohort from the Catalan Multiple System Atrophy Registry (CMSAR) to determine which symptoms are most likely to progress throughout a 2-year follow-up. Methods: We analyzed baseline, 12-month, and 24-month follow-up evaluations from the 80 cases recruited by the CMSAR. Evaluations included the UMSARS assessment, cognitive and neuropsychiatric evaluations, and a non-motor scale (NMSS-PD). Statistical analysis was done using a Generalized Estimated Equations (GEE) model. Results: Both UMSARS I and II sub-scores significantly increased at 12- and 24-month follow-ups (p <  0.001), with a median total score increase of 11 and 12.5 points, respectively. Items on UMSARS I that significantly worsened were mostly motor affecting daily activities. NMS, including urinary and sexual dysfunction, as well as sleep difficulties showed a significant progression on the NMSS-PD; however, other NMS such as postural hypotension, gastrointestinal, and mood dysfunction, although prevalent, did not show a clear progression on clinical scales. Conclusion: Within 24 months and as early as 12 months, MSA cases may experience significant motor worsening, affecting basic daily activities. NMS are prevalent; however, not all clinical scales register a clear progression of symptoms, perhaps suggesting that they are not sensitive enough for non-motor evaluation.

scholarly journals Evaluation of fecal microbiota transplantation in Parkinson's disease patients with constipation

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Xiao-yi Kuai ◽  
Xiao-han Yao ◽  
Li-juan Xu ◽  
Yu-qing Zhou ◽  
Li-ping Zhang ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Rating Scale ◽  
Neurodegenerative Disorder ◽  
Fecal Microbiota Transplantation ◽  
Fecal Microbiota ◽  
Motor Symptoms ◽  
Gastrointestinal Dysfunction ◽  
Before And After ◽  
Non Motor Symptoms

AbstractParkinson’s disease (PD) is a neurodegenerative disorder and 70–80% of PD patients suffer from gastrointestinal dysfunction such as constipation. We aimed to assess the efficacy and safety of fecal microbiota transplantation (FMT) for treating PD related to gastrointestinal dysfunction. We conducted a prospective, single- study. Eleven patients with PD received FMT. Fecal samples were collected before and after FMT and subjected to 16S ribosomal DNA (rDNA) gene sequencing. Hoehn-Yahr (H-Y) grade, Unified Parkinson's Disease Rating Scale (UPDRS) score, and the Non-Motion Symptom Questionnaire (NMSS) were used to assess improvements in motor and non-motor symptoms. PAC-QOL score and Wexner constipation score were used to assess the patient's constipation symptoms. All patients were tested by the small intestine breath hydrogen test, performed before and after FMT. Community richness (chao) and microbial structure in before-FMT PD patients were significantly different from the after-FMT. We observed an increased abundance of Blautia and Prevotella in PD patients after FMT, while the abundance of Bacteroidetes decreased dramatically. After FMT, the H-Y grade, UPDRS, and NMSS of PD patients decreased significantly. Through the lactulose H2 breath test, the intestinal bacterial overgrowth (SIBO) in PD patients returned to normal. The PAC-QOL score and Wexner constipation score in after-FMT patients decreased significantly. Our study profiles specific characteristics and microbial dysbiosis in the gut of PD patients. FMT might be a therapeutic potential for reconstructing the gut microbiota of PD patients and improving their motor and non-motor symptoms.

scholarly journals Pramipexole and tolcapone alleviate thermal and mechanical nociception in naive rats.

Folia Medica ◽  
2021 ◽  
Vol 63 (3) ◽  
pp. 377-384
Author(s):  
Anita Mihaylova ◽  
Ilia Kostadinov ◽  
Nina Doncheva ◽  
Delian Delev ◽  
Hristina Zlatanova
Keyword(s):  
Neurodegenerative Disorder ◽  
Antinociceptive Effect ◽  
Positive Control ◽  
Motor Symptoms ◽  
Dopaminergic Drugs ◽  
Thermal Nociception ◽  
Dopaminergic Neurotransmission ◽  
Latent Time ◽  
Male Wistar Rats ◽  
Non Motor Symptoms

Introduction: Parkinson&rsquo;s disease (PD) is &#1072; neurodegenerative disorder characterized mainly by its motor symptoms. The non-motor symptoms including pain are increasingly recognized in the last few decades. Existing evidence suggests that the dopaminergic neurotransmission has an essential role in pain control. Aim: The aim of the present study was to investigate the antinociceptive effect of dopaminergic drugs pramipexole and tolcapone against chemical and thermal stimuli in naive rats. Materials and methods: Male Wistar rats divided into 8 groups (n=8): saline; diclofenac 25 mg/kg body weight (bw) (positive control); pramipexole 0.5; 1 and 3 mg/kg bw; tolacapone 5; 15 and 30 mg/kg bw. Paw pressure and plantar tests were performed. Paw withdrawal pressure and latent time were measured. Statistical analysis was done by SPSS 19. Results: In the paw pressure test, pramipexole, in a dose of 1 and 3 mg/kg bw and tolcapone in a dose of 30 mg/kg bw, increased significantly the latency at 1, 2, and 3 hours compared to saline (p<0.05). In the plantar test, only the highest dose of pramipexole reached significance at 3 hours compared to the control rats (p<0.05). In contrast to pramipexole the three experimental groups with tolcapone markedly increased the latent time at 1 and 3 hours compared to saline (p<0.05). Conclusions: Pramipexole and tolcapone reduce mechanical and thermal nociception in na&iuml;ve rats by enhancing dopaminergic neurotransmission at both spinal and supraspinal levels. In addition, tolcapone stimulates noradrenergic mediation which may contribute to its antinociceptive effect.

Impact of sleep-related breathing disorder on motor and non-motor symptoms in multiple system atrophy

2018 ◽  
Vol 22 (4) ◽  
pp. 981-987
Author(s):  
Bei Cao ◽  
Qian-Qian Wei ◽  
Ruwei Ou ◽  
Bi Zhao ◽  
Tao Hu ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Motor Symptoms ◽  
Sleep Related Breathing Disorder ◽  
Non Motor Symptoms

scholarly journals Swallowing dysfunctions in Parkinson’s disease patients: a novel challenge for the internist

2019 ◽  
Vol 13 (2) ◽  
pp. 91-94 ◽  
Author(s):  
Elena Barbagelata ◽  
Antonello Nicolini ◽  
Paola Tognetti
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Clinical Practice ◽  
Aspiration Pneumonia ◽  
Neurodegenerative Disorder ◽  
Movement Pattern ◽  
Morbidity And Mortality ◽  
Motor Symptoms ◽  
Common Causes ◽  
Non Motor Symptoms

Parkinson’s disease (PD) is a chronic neurodegenerative disorder with a typical movement pattern, as well as different, less studied non-motor symptoms such as dysphagia. Disease-related disorders in efficacy or safety in the process of swallowing usually lead to malnutrition, dehydration or pneumonia. Dysphagia and subsequent aspiration pneumonia are common causes of morbidity and mortality in those with PD. The aim of this review is to identify and evaluate the existing literature on swallowing disorders in PD and providing recommendations for clinical practice routine.

scholarly journals A clinical-anatomical signature of Parkinson’s Disease identified with partial least squares and magnetic resonance imaging

2017 ◽  
Author(s):  
Yashar Zeighami ◽  
Seyed-Mohammad Fereshtehnejad ◽  
Mahsa Dadar ◽  
D. Louis Collins ◽  
Ronald B. Postuma ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Least Squares ◽  
Partial Least Squares ◽  
Large Scale ◽  
De Novo ◽  
Neurodegenerative Disorder ◽  
Clinical Manifestations ◽  
Motor Symptoms ◽  
Non Motor Symptoms

AbstractParkinson’s disease (PD) is a neurodegenerative disorder characterized by a wide array of motor and non-motor symptoms. It remains unclear whether neurodegeneration in discrete loci gives rise to discrete symptoms, or whether network-wide atrophy gives rise to the unique behavioural and clinical profile associated with PD. Here we apply a data-driven strategy to isolate large-scale, multivariate associations between distributed atrophy patterns and clinical phenotypes in PD. In a sample of N = 229 de novo PD patients, we estimate disease-related atrophy using deformation based morphometry (DBM) of T1 weighted MR images. Using partial least squares (PLS), we identify a network of subcortical and cortical regions whose collective atrophy is associated with a clinical phenotype encompassing motor and non-motor features. Despite the relatively early stage of the disease in the sample, the atrophy pattern encompassed lower brainstem, substantia nigra, basal ganglia and cortical areas, consistent with the Braak hypothesis. In addition, individual variation in this putative atrophy network predicted longitudinal clinical progression in both motor and non-motor symptoms. Altogether, these results demonstrate a pleiotropic mapping between neurodegeneration and the clinical manifestations of PD, and that this mapping can be detected even in de novo patients.

scholarly journals Excessive Daytime Sleepiness Is Associated With Non-motor Symptoms of Multiple System Atrophy: A Cross-Sectional Study in China

2022 ◽  
Vol 12 ◽  
Author(s):  
Hui Wang ◽  
Xiangdong Tang ◽  
Junying Zhou ◽  
Yanming Xu
Keyword(s):  
Risk Factors ◽  
Multiple System Atrophy ◽  
Excessive Daytime Sleepiness ◽  
Disease Duration ◽  
Cross Sectional Study ◽  
Motor Dysfunction ◽  
Motor Symptoms ◽  
Sectional Study ◽  
Cross Sectional ◽  
Non Motor Symptoms

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.

scholarly journals Genotype-Phenotype Correlations in Monogenic Parkinson Disease: A Review on Clinical and Molecular Findings

2021 ◽  
Vol 12 ◽  
Author(s):  
Daniele Guadagnolo ◽  
Maria Piane ◽  
Maria Rosaria Torrisi ◽  
Antonio Pizzuti ◽  
Simona Petrucci
Keyword(s):  
Parkinson Disease ◽  
Movement Disorders ◽  
Sleep Disturbances ◽  
Cell Function ◽  
Neurodegenerative Disorder ◽  
Current Knowledge ◽  
Mendelian Inheritance ◽  
Motor Symptoms ◽  
The Many ◽  
Non Motor Symptoms

Parkinson disease (PD) is a complex neurodegenerative disorder, usually with multifactorial etiology. It is characterized by prominent movement disorders and non-motor symptoms. Movement disorders commonly include bradykinesia, rigidity, and resting tremor. Non-motor symptoms can include behavior disorders, sleep disturbances, hyposmia, cognitive impairment, and depression. A fraction of PD cases instead is due to Parkinsonian conditions with Mendelian inheritance. The study of the genetic causes of these phenotypes has shed light onto common pathogenetic mechanisms underlying Parkinsonian conditions. Monogenic Parkinsonisms can present autosomal dominant, autosomal recessive, or even X-linked inheritance patterns. Clinical presentations vary from forms indistinguishable from idiopathic PD to severe childhood-onset conditions with other neurological signs. We provided a comprehensive description of each condition, discussing current knowledge on genotype-phenotype correlations. Despite the broad clinical spectrum and the many genes involved, the phenotype appears to be related to the disrupted cell function and inheritance pattern, and several assumptions about genotype-phenotype correlations can be made. The interest in these assumptions is not merely speculative, in the light of novel promising targeted therapies currently under development.

scholarly journals Improvement of non-motor symptoms and quality of life after DBS stimulation for dystonia: one-year follow-up

2021 ◽  
Author(s):  
Clarice Listik ◽  
Rubens Gisbert Cury ◽  
Sara Carvalho Barbosa Casagrande ◽  
Eduardo Listik ◽  
Debora Arnaut ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Chronic Pain ◽  
Parkinson's Disease ◽  
Prospective Study ◽  
Mcgill Pain Questionnaire ◽  
Motor Symptoms ◽  
One Year ◽  
A Prospective Study ◽  
Prospective Study Design ◽  
Non Motor Symptoms

Background: DBS is an established treatment option in refractory dystonia, and motor outcomes have been extensively evaluated instead of the usually neglected NMS (e.g., pain). Objective: To describe the non-motor symptoms (NMS) after Deep Brain Stimulation (DBS) surgery for refractory generalized inherited/idiopathic dystonia in a prospective study. Design and setting: A prospective study that evaluated patients in the Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo. Methods: This study evaluated patients before and one year after DBS surgery. We applied the following scales: Burke-Fahn-Marsden Rating Scale (BFMRS), Hospital Anxiety and Depression Scale (HADS), Non-Motor Symptoms Scale for Parkinson’s Disease (NMSS-PD), Parkinson’s Disease Questionnaire-8 (PDQ8) Brief Pain Inventory (BPI), Neuropathic Pain Symptom Inventory (NPSI) and McGill pain questionnaire. Results: 11 patients (38.35 ± 11.30 years) underwent surgery (36.3% women). Motor BFMRS subscore was 64.36 ± 22.94 at baseline and 33.55 ± 17.44 after surgery (p=0.003, 47.9% improvement on motor symptoms). HADS scores remained unchanged. NMSS-PD had a significant change after DBS, from 70.91 ± 59.07 to 37.18 ± 55.05 (p=0.013, 47,5% improvement). Seven patients reported pain before DBS surgery, and after one year, four patients reported chronic pain (i.e., pain improved by 42.28%). BPI’s severity and interference scores were 4.61 ± 2.84 and 4.12 ± 2.67, respectively before surgery, and 2.79 ± 2.31 (0.00–6.25) and 1.12 ± 1.32 (0.00–3.00) after DBS (p=0.043 and p=0.028). NPSI total score was 15.29 ± 13.94 before DBS, and reduced to 2.29 ± 2.98 afterward (p=0.028). McGill’s total score was 9.00 ± 3.32 before DBS, achieving 2.71 ± 2.93 after surgery (p=0.028), mostly driven by the sensory sub-score. Conclusions: We found that DBS improves NMS in dystonia, including chronic pain, anxiety, gastrointestinal symptoms, besides the already established improvement in QoL and motor symptoms.

Various Motor and Non-Motor Symptoms in Early Multiple System Atrophy

2019 ◽  
Vol 19 (5-6) ◽  
pp. 238-243 ◽  
Author(s):  
Yu Jin Jung ◽  
Han-Joon Kim ◽  
Dallah Yoo ◽  
Ji-Hyun Choi ◽  
Jin Hee Im ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Disease Duration ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Accurate Diagnosis ◽  
Motor Symptoms ◽  
Red Flags ◽  
Sleep Behavior ◽  
Autonomic Dysfunctions ◽  
Non Motor Symptoms

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.

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