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.

scholarly journals Reduced striatal vesicular monoamine transporter 2 in REM sleep behavior disorder: imaging prodromal parkinsonism

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Leah C. Beauchamp ◽  
Victor L. Villemagne ◽  
David I. Finkelstein ◽  
Vincent Doré ◽  
Ashley I. Bush ◽  
...  
Keyword(s):  
Rem Sleep ◽  
Time Window ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Vesicular Monoamine Transporter ◽  
Motor Symptoms ◽  
Monoamine Transporter ◽  
Sleep Behavior ◽  
Nigrostriatal Degeneration ◽  
Non Motor Symptoms

Abstract Motor deficits in parkinsonism are caused by degeneration of dopaminergic nigral neurons. The success of disease-modifying therapies relies on early detection of the underlying pathological process, leading to early interventions in the disease phenotype. Healthy (n = 16), REM sleep behavior disorder (RBD) (n = 14), dementia with Lewy bodies (n = 10), and Parkinson’s disease (PD) (n = 20) participants underwent 18F-AV133 vesicular monoamine transporter type-2 (VMAT2) PET to determine the integrity of the nigrostriatal pathway. Clinical, neurophysiological and neuropsychological testing was conducted to assess parkinsonian symptoms. There was reduced VMAT2 levels in RBD participants in the caudate and putamen, indicating nigrostriatal degeneration. RBD patients also presented with hyposmia and anxiety, non-motor symptoms associated with parkinsonism. 18F-AV133 VMAT2 PET allows identification of underlying nigrostriatal degeneration in RBD patients. These findings align with observations of concurrent non-motor symptoms in PD and RBD participants of the Parkinson’s Progression Markers Initiative. Together, these findings suggest that RBD subjects have prodromal parkinsonism supporting the concept of conducting neuroprotective therapeutic trials in RBD-enriched cohorts. Ongoing longitudinal follow-up of these subjects will allow us to determine the time-window of clinical progression.

scholarly journals Relations of non-motor symptoms and dopamine transporter binding in REM sleep behavior disorder

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Petr Dušek ◽  
Veronika Lorenzo y Losada Ibarburu ◽  
Ondrej Bezdicek ◽  
Irene Dall’antonia ◽  
Simona Dostálová ◽  
...  
Keyword(s):  
Dopamine Transporter ◽  
Rem Sleep ◽  
Muscle Activity ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Motor Symptoms ◽  
Sleep Behavior ◽  
Binding Ratio ◽  
Dat Spect ◽  
Non Motor Symptoms

Abstract The aim of this study was to evaluate associations of motor and non-motor symptoms with dopamine transporter binding in prodromal stage of synucleinopathies. We examined 74 patients with idiopathic REM sleep behavior disorder (RBD), which is a prodromal synucleinopathy, and 39 controls using Movement Disorders Society-Unified Parkinson’s Disease Rating Scale (MDS-UPDRS), Montreal Cognitive Assessment, University of Pennsylvania Smell Identification Test (UPSIT), Farnsworth-Munsell 100 hue test, orthostatic test, Scales for Outcomes in PD-Autonomic, Beck depression inventory-II, State-Trait Anxiety Inventory, and video-polysomnography. Electromyographic muscle activity during REM sleep was quantified according to Sleep Innsbruck-Barcelona criteria. In 65 patients, dopamine transporter single-photon emission computed tomography (DAT-SPECT) imaging was performed, putaminal binding ratio was calculated and scans were classified as normal, borderline, or abnormal. Compared to controls, RBD patients had significantly more severe scores in all examined tests. Patients with abnormal DAT-SPECT had higher MDS-UPDRS motor score (p = 0.006) and higher prevalence of orthostatic hypotension (p = 0.008). Putaminal binding ratio was positively associated with UPSIT score (p = 0.03) and negatively associated with tonic (p = 0.003) and phasic (p = 0.01) muscle activity during REM sleep. These associations likely reflect simultaneous advancement of underlying pathology in substantia nigra and susceptible brainstem and olfactory nuclei in prodromal synucleinopathy.

scholarly journals A Stage-Based Approach to Therapy in Parkinson’s Disease

Biomolecules ◽  
2019 ◽  
Vol 9 (8) ◽  
pp. 388 ◽  
Author(s):  
Claudia Carrarini ◽  
Mirella Russo ◽  
Fedele Dono ◽  
Martina Di Pietro ◽  
Marianna G. Rispoli ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Neurodegenerative Disorder ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Motor Symptoms ◽  
Pharmacological Management ◽  
Sleep Behavior ◽  
Heavy Burden ◽  
Non Motor Symptoms

Parkinson’s disease (PD) is a neurodegenerative disorder that features progressive, disabling motor symptoms, such as bradykinesia, rigidity, and resting tremor. Nevertheless, some non-motor symptoms, including depression, REM sleep behavior disorder, and olfactive impairment, are even earlier features of PD. At later stages, apathy, impulse control disorder, neuropsychiatric disturbances, and cognitive impairment can present, and they often become a heavy burden for both patients and caregivers. Indeed, PD increasingly compromises activities of daily life, even though a high variability in clinical presentation can be observed among people affected. Nowadays, symptomatic drugs and non-pharmaceutical treatments represent the best therapeutic options to improve quality of life in PD patients. The aim of the present review is to provide a practical, stage-based guide to pharmacological management of both motor and non-motor symptoms of PD. Furthermore, warning about drug side effects, contraindications, as well as dosage and methods of administration, are highlighted here, to help the physician in yielding the best therapeutic strategies for each symptom and condition in patients with PD.

A Questionnaire-Based Study on Clinical REM Sleep Behavior Disorder and Subtypes in Multiple System Atrophy

2021 ◽  
pp. 1-7
Author(s):  
Dong-dong Wu ◽  
Wen Su ◽  
Shu-hua Li ◽  
Jing He ◽  
Kai Li ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Sleep Problems ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Motor Symptoms ◽  
Sleep Behavior ◽  
Healthy Control ◽  
Patient Groups ◽  
The Difference

<b><i>Objectives:</i></b> Studies documenting the association between rapid eye movement sleep behavior disorder (RBD) and subtypes of multiple system atrophy (MSA) are rare. In this study, we investigated the presence of clinical RBD in MSA patients and compared the prevalence and severity of RBD in patients with MSA-P and MSA-C subtypes. <b><i>Methods:</i></b> We evaluated 54 consecutive patients presenting with MSA and hospitalized in the neurology ward of Beijing Hospital from February 2012 to June 2020. The healthy control (HC) group consisted of 100 healthy individuals who came to our hospital for physical examination. The clinical diagnosis of RBD was based on the minimal diagnostic criteria of International Classification of Sleep Disorders, revised. The severity of clinical RBD was rated on a digital scale from 0 to 3. The patients were divided into 2 subgroups: MSA-P and MSA-C. The MSA and HC groups were compared in terms of frequency of clinical RBD. The MSA-P and MSA-C subgroups were compared with each other for age, sex, onset age, disease duration, and features of clinical RBD. The correlation between severity of clinical RBD and clinical characteristics of MSA was analyzed in the patient groups. <b><i>Results:</i></b> The frequency of clinical RBD in MSA and HC groups was 70.4% (38/54) and 5% (5/100), respectively. The difference between 2 groups was significant (χ<sup>2</sup> = 74.453, <i>p</i> = 0.000). Among the patients, 57.4% (31/54) had the MSA-P subtype. There were no significant differences between MSA-P and MSA-C subtypes in the prevalence (χ<sup>2</sup> = 1.734, <i>p</i> = 0.188) and severity (χ<sup>2</sup> = 1.776, <i>p</i> = 0.412) of clinical RBD. The onset of clinical RBD during the premotor period was not different between the subtypes of MSA, either in patients’ number of preceding the onset of motor symptoms (χ<sup>2</sup> = 0.581, <i>p</i> = 0.446) or the preceding time (<i>Z</i> = −0.550, <i>p</i> = 0.582). For the MSA-C patients, there was a negative correlation between the score of severity of the RBD scale and RBD preceding motor symptoms (<i>r</i> = −0.482, <i>p</i> = 0.020). <b><i>Conclusion:</i></b> In our study, the prevalence of clinical RBD is unrelated to the subtypes of MSA. The onset of clinical RBD during the premotor period was not different between subtypes of MSA. However, we found that the severity of RBD occurring before the motor symptoms was more than that occurring after the motor symptoms in MSA-C patients. Our results showed that MSA-P and MSA-C patients may have a probable indicator for the similar pathologic mechanism of the disease and its sleep problems.

scholarly journals Parkinson’s disease and REM sleep behavior disorder result in increased non-motor symptoms

2014 ◽  
Vol 15 (8) ◽  
pp. 959-966 ◽  
Author(s):  
Ariel B. Neikrug ◽  
Julie A. Avanzino ◽  
Lianqi Liu ◽  
Jeanne E. Maglione ◽  
Loki Natarajan ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Motor Symptoms ◽  
Sleep Behavior ◽  
Non Motor Symptoms

scholarly journals SLEEP DISORDERS IN PARKINSON’S DISEASE

2019 ◽  
Vol 72 (3) ◽  
pp. 425-431
Author(s):  
Magdalena Doręgowska ◽  
Monika Rudzińska-Bar
Keyword(s):  
Sleep Apnea Syndrome ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Sleep Stages ◽  
Motor Symptoms ◽  
Sleep Behavior ◽  
Sleep Physiology ◽  
Obstructive Sleep ◽  
Sleeping Problems ◽  
Non Motor Symptoms

Sleep disorder are common non-motor symptoms in Parkinson`s disease (PD). They can be found in different sleep stages or appear during the daytime. They correlate with faster progression of motor problems and lower quality of a patient’s life. Sleep physiology, different sleep dysfunction such as: RBD-REM sleep behavior disorder, EDS – excessive daytime sleepiness, insomnia, OSAS-obstructive sleep apnea syndrome, and their clinical manifestation have been presented in this review. Diagnostic and therapy possibilities have been summarized as well. Particular attention has also been paid to the coexistence of various non-motor symptoms such as pain, depression or nocturia, and their correlations with sleeping problems.

Progression and prognosis in multiple system atrophy presenting with REM behavior disorder

Neurology ◽  
2020 ◽  
Vol 94 (17) ◽  
pp. e1828-e1834 ◽  
Author(s):  
Giulia Giannini ◽  
Vincenzo Mastrangelo ◽  
Federica Provini ◽  
Andrea Droghini ◽  
Annagrazia Cecere ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Disease Progression ◽  
Survival Data ◽  
Disease Onset ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Rapid Progression ◽  
Sleep Behavior ◽  
Risk Of Death ◽  
Progression Of Disease

ObjectivesTo investigate (1) the prevalence of REM sleep behavior disorder (RBD) as mode of disease onset in a cohort of patients with multiple system atrophy (MSA) and (2) disease progression and prognosis in patients with MSA with RBD predating (pre-RBD) and following (post-RBD) disease onset.MethodsWe retrospectively identified all patients with a clinical diagnosis of MSA evaluated at least once a year during the disease course. Type of onset was defined by the first reported motor or autonomic symptom/sign related to MSA. The occurrence of symptoms/signs and milestone of disease progression, and their latency from disease onset, were collected. Survival data were calculated. RBD was confirmed by video-polysomnography.ResultsOf a total of 158 patients, pre-RBD represented the mode of disease onset in 27% of patients, preceding disease onset according to the international criteria with a median of 3 (2–5) years. Comparing pre-RBD and post-RBD patients, the first group showed an increased prevalence of autonomic onset of disease, a reduced prevalence of parkinsonism, an earlier onset of stridor, pyramidal signs, symptomatic orthostatic hypotension, urinary dysfunction, severe dysphagia, and wheelchair dependency. The risk of death was higher in patients with pre-RBD.ConclusionsIn our MSA cohort, RBD represented the most frequent mode of disease presentation. A more rapid progression of disease was observed in the pre-RBD group. These findings suggested a careful assessment of sleep disorders to early recognize RBD and a closer follow-up of autonomic dysfunction and stridor in patients with pre-RBD.

scholarly journals Heterogeneity of prodromal Parkinson symptoms in siblings of Parkinson disease patients

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Luca Baldelli ◽  
Sebastian Schade ◽  
Silvia Jesús ◽  
Sebastian R. Schreglmann ◽  
Luisa Sambati ◽  
...  
Keyword(s):  
De Novo ◽  
Motor Impairment ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Prodromal Phase ◽  
Sleep Behavior ◽  
Heterogeneous Distribution ◽  
Increased Risk ◽  
Non Motor Symptoms ◽  
German Part

AbstractA prodromal phase of Parkinson’s disease (PD) may precede motor manifestations by decades. PD patients’ siblings are at higher risk for PD, but the prevalence and distribution of prodromal symptoms are unknown. The study objectives were (1) to assess motor and non-motor features estimating prodromal PD probability in PD siblings recruited within the European PROPAG-AGEING project; (2) to compare motor and non-motor symptoms to the well-established DeNoPa cohort. 340 PD siblings from three sites (Bologna, Seville, Kassel/Goettingen) underwent clinical and neurological evaluations of PD markers. The German part of the cohort was compared with German de novo PD patients (dnPDs) and healthy controls (CTRs) from DeNoPa. Fifteen (4.4%) siblings presented with subtle signs of motor impairment, with MDS-UPDRS-III scores not clinically different from CTRs. Symptoms of orthostatic hypotension were present in 47 siblings (13.8%), no different to CTRs (p = 0.072). No differences were found for olfaction and overall cognition; German-siblings performed worse than CTRs in visuospatial-executive and language tasks. 3/147 siblings had video-polysomnography-confirmed REM sleep behavior disorder (RBD), none was positive on the RBD Screening Questionnaire. 173/300 siblings had <1% probability of having prodromal PD; 100 between 1 and 10%, 26 siblings between 10 and 80%, one fulfilled the criteria for prodromal PD. According to the current analysis, we cannot confirm the increased risk of PD siblings for prodromal PD. Siblings showed a heterogeneous distribution of prodromal PD markers and probability. Additional parameters, including strong disease markers, should be investigated to verify if these results depend on validity and sensitivity of prodromal PD criteria, or if siblings’ risk is not elevated.

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