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.

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.

Skin nerve phosphorylated α-synuclein deposits in idiopathic REM sleep behavior disorder

Neurology ◽  
2017 ◽  
Vol 88 (22) ◽  
pp. 2128-2131 ◽  
Author(s):  
Elena Antelmi ◽  
Vincenzo Donadio ◽  
Alex Incensi ◽  
Giuseppe Plazzi ◽  
Rocco Liguori
Keyword(s):  
Skin Biopsy ◽  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Healthy Controls ◽  
Class Iii ◽  
Immunofluorescence Method ◽  
Nonmotor Symptoms ◽  
Sleep Behavior

Objective:To test if phosphorylated α-synuclein (p-α-syn) deposits can be detected by means of skin biopsy in patients with idiopathic REM sleep behavior disorder (iRBD) as a potential early histopathologic marker of impending synucleinopathy.Methods:Proximal (cervical) and distal (legs) samples of skin biopsy were obtained from 12 patients with polysomnographically confirmed iRBD and 55 sex- and age-matched healthy controls (HC). P-α-syn deposits were assessed with a monoclonal antibody against p-α-syn at serine 129, disclosed by an immunofluorescence method. In addition, patients underwent an extensive workup in order to search for nonmotor symptoms and neuroimaging findings usually associated with impending neurodegeneration and to exclude subtle motor or cognitive signs.Results:P-α-syn deposits were detected in 9 (75%) out of 12 patients with iRBD and none of the HC. In iRBD, the sensitivity of the test was higher at the cervical site (67%) when compared to the leg site (58%).Conclusions:Our preliminary findings suggest that skin biopsy in patients with iRBD might be a safe and sensitive procedure to be further tested in order to detect p-α-syn deposits in the premotor stage of synucleinopathies.Classification of evidence:This study provides Class III evidence that p-α-syn skin deposits identify patients with iRBD.

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.

Sleep-Related Parasomnias

2021 ◽  
pp. 571-586
Author(s):  
Jaclyn L. Lewis-Croswell ◽  
José Colón
Keyword(s):  
Sleep Disorders ◽  
Eye Movement ◽  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Historical Narratives ◽  
Sleep Paralysis ◽  
Rapid Eye Movement ◽  
Sleep Behavior

Ever since there have been written historical narratives, sleep anomalies have been noted, from medieval paintings of demons terrorizing an individual in their sleep, to biblical revelations in dreams and nightmares, to tales of somnambulism from Shakespeare to Disney. Mysterious parasomnias have been recognized well before they could be classified into the International Classification of Sleep Disorders. With the invention in the 1950s of the electroencephalogram, generations of sleep researchers have been able to classify once mysterious sleep anomalies as either rapid eye movement (REM) or NREM parasomnias. Some parasomnias may be benign and self-limited, such as sleepwalking in a child. Others may lead to injury or can be a sign of other neurological disorders, such as REM sleep behavior disorder. Some can terrify patients and are commonly underreported, such as sleep paralysis, which has been confused by some as modern-day alien abductions. The treatment of parasomnias depends on proper identification based on well-established criteria. Subsequently, integrative approaches to the treatment of these disorders may be applied.

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.

Sign in / Sign up

Export Citation Format

Share Document