Sighs during sleep in multiple system atrophy

Sighs are physiological phenomena and may occasionally occur during sleep in healthy young adults. Although inspiratory sighs are considered a diagnostic red flag for the parkinsonian form of multiple system atrophy (MSA), its frequency and characteristics are unclear. We aimed to define sigh frequency during sleep recordings in patients with MSA compared to Parkinson's disease (PD) patients, as well as evaluate possible associated breathing disorders or autonomic changes. We analyzed 9 polysomnography's from patients with MSA and 9 from matched PD patients. The proportion of MSA patients (both MSA-P and MSA-C) with sleep-related sighs was significantly higher than that of PD patients, and these occurred predominantly in stages N1 and N2. The median sigh index in sleep and wakefulness were also significantly higher in MSA, although with a significant inter-subject variability. Higher sigh indexes were not associated to other breathing disturbances or with longer disease duration. In MSA, 12% of sighs were associated with oxygen desaturation, while none of the events in PD patients presented with significant changes in oxygen saturation. Respiratory events followed 45% of sighs in MSA, predominantly central sleep apneas, and 29% of sighs in PD, predominantly hypopneas. Our data suggests that high sigh frequencies during sleep should also be considered a red flag for MSA, and future studies should aim to determine whether increased sighing frequency during sleep is specific for this disorder.     

Sleep disturbances in Malaysian patients with Parkinson's disease using polysomnography and PDSS

BackgroundSleep disturbances such as sleep fragmentation, sleep disordered breathing (SDB), periodic limb movements (PLM), excessive daytime somnolence (EDS) and insomnia are prevalent in Parkinson's disease (PD). However, studies in the Asian population are limited.MethodsThis was a cross-sectional study involving 46 Malaysians with PD using polysomnography (PSG) and standardized translated Parkinson's disease sleep scale (PDSS). Overnight PSG recordings, UPDRS and PDSS scores, and baseline demographic data were obtained.ResultsData from 44 patients were analysed. Thirty-six patients (81.8%) had PSG-quantified sleep disorders. Twenty-three (52.3%) had sleep fragmentation, 24 (54.6%) had SDB and 14 (32%) had PLM. EDS was present in 9.1%. Insomnia was reported by 31.8%. Patients with sleep fragmentation had significantly higher UPDRS scores and lower PDSS insomnia sub-scores. The UPDRS scores correlated negatively with the TST and sleep efficiency. All patients with EDS had SDB (p = 0.056). The PDSS insomnia sub-items correlated with sleep fragmentation on PSG.Conclusion: The prevalence of sleep disorders based on PSG and PDSS in our PD patients was high, the commonest being sleep fragmentation and SDB, while EDS was the least prevalent. Problem specific sub-items of the PDSS were more accurate in predicting the relevant PSG-related changes compared to the PDSS as a whole.     

快速眼动期睡眠行为障碍与突触核蛋白病的关系

目的 研究快速眼动(REM)期睡眠行为障碍(RBD)在突触核蛋白病中的出现率、出现时间、电生理特点,探讨RBD与突触核蛋白病之间的关系以及电生理诊断指标.方法 通过对患者的睡眠状况调查以及夜间多导睡眠监测(NPSG),研究本组疾病的睡眠障碍特征:(1)主观睡眠调查:帕金森病(PD)患者66例,多系统萎缩(MSA)患者30例,性别、年龄匹配的健康对照组65名,询问睡眠史,了解RBD出现的比例及出现时间.(2)NPSG:PD组8例、MSA组13例,健康对照组15名,所有受试者行连续两夜NPSG监测.分析伴发RBD的突触核蛋白病患者的NPSG特点.结果 PD和MSA合并RBD比例分别是59.1%(39/66)和86.6%(26/30),明显高于对照组(4.6%,3/65),其中RBD早于两种变性病临床发病的患者比例分别是46.2%(18/39)和84.6%(22/26).PD和MSA合并RBD最主要的NPSG特点是:REM期肌肉弛缓现象消失(RWA)和运动增多.RWA比例和位相性肌电活动密度可能成为神经变性病合并RBD的NPSG诊断指标.结论 RBD在PD和MSA患者中出现率明显增高,部分RBD发生先于变性病,提示RBD与突触核蛋白病关系密切,RBD有可能是突触核蛋白病的早期表现.NPSG特征应作为RBD的主要诊断标准,RWA比例和位相性肌电活动密度可能成为神经变性病合并RBD的NPSG诊断指标.     

Sleep disruption, daytime somnolence and ''sleep attacks'' in Parkinson''s disease: a clinical survey in PD patients and age-matched healthy volunteers

Recent case reports of 'sleep attacks' (SA) in patients with Parkinson's disease (PD) generated concerns about drug-induced daytime somnolence in this population. However, there are nearly no comparative data on sleep and vigilance problems between PD patients and normal controls. We performed a cross-sectional survey in PD patients and age-matched controls using a structured questionnaire on PD history, treatments, co-morbidity, activities of daily living, habits, exercise, sleep pattern, driving, pre-existing nocturnal problems, daytime somnolence, episodes of SA and the circumstances in which such episodes occurred. Daytime somnolence was also measured with the Epworth Sleepiness Scale (ESS) and sleep quality with the Pittsburgh Sleep Quality Index (PSQI). 176 PD patients and 174 controls were included. The same proportion of PD patients (27%) and controls (32%) reported episodes of SA, but these were more frequent in PD patients and occurred more frequently during situations requiring attention (10.8% vs. 1.7%, p<10(-3)). More PD patients had abnormal daytime somnolence (ESS) and poor sleeping quality (PSQI). The most consistent factor associated with SA was the duration of levodopa therapy and the predictive value of an abnormal ESS score was rather poor (40.7%). Abnormal daytime somnolence and poor sleep quality at night are more frequent in PD patients than in normals. However, SA are reported in both groups, although less frequently in the normals during activities that requires attention.     

Deep brain stimulation and its effect on sleep in Parkinson's disease

Deep Brain Stimulation (DBS) is an effective treatment for patients with advanced Parkinson's disease (PD) and motor complications whose condition can no longer be improved by adjustment of medical therapy. PD patients often report increased daily somnolence and night sleep abnormalities partially related to dopaminergic treatment. In a survey of 386 consecutive non-demented non-depressed PD patients seen in our clinic over a period of 3 months we found increased daily somnolence to be relatively uncommon in non-demented PD patients, although it may be associated with stable treatment with high dose dopamine agonists. Disease related factors seemed responsible for night sleep abnormalities. Because DBS of the subthalamic nucleus (STN) reduces motor disability, as well as total medication intake, one would expect a similar benefit on sleep abnormalities. Indeed, recent evidence suggests that chronic STN-DBS may improve sleep quality through increased nocturnal mobility and reduction of sleep fragmentation.     

Excessive daytime sleepiness and sleep benefit in Parkinson's disease: a community-based study.

The objective of this study was to investigate the frequency of excessive daytime sleepiness (EDS) and the beneficial effect of sleep on motor performance in an unselected community-based sample of patients with Parkinson's disease (PD). Furthermore, we wanted to identify possible risk factors to these phenomena. Detailed information on somnolence and sleep during daytime, as well as sleep benefit (SB) on awakening, was collected through a questionnaire among 245 patients with PD. Daytime somnolence was graded in groups of no somnolence, mild daytime sleepiness, and EDS. In addition, the occurrence of somnolence in the patients with PD was compared with the occurrence among control groups of patients with diabetes mellitus and of healthy elderly subjects. The correlations between EDS and SB and various motor- and non-motor symptoms of PD were evaluated. Among the patients with PD, 15.5% experienced EDS, significantly more than in the patients with diabetes mellitus (4%) and the healthy control subjects (1%). The frequency of mild daytime sleepiness was similar (10%) in patients with PD and control subjects. The patients with EDS had significantly higher staging of PD, were more disabled, and showed a higher frequency of cognitive decline compared with the patients without somnolence. They also had been using levodopa for a longer time and had more hallucinations. The occurrence of nocturnal sleeping problems and the use of sleeping pills was similar in the two groups, as was the mean age at examination, duration of PD, and presence of fluctuations and dyskinesias. SB was found in 42.2% of the patients with PD. These patients had been using levodopa for significantly longer and had significantly more fluctuations and dyskinesias compared with the patients without SB. Our results suggest that mild daytime sleepiness may be a result of normal aging, whereas more severe EDS can be explained by the neuropathologic changes of PD. The data from this community-based study confirms the previously reported high frequencies of SB.     

Sleep disturbances and excessive daytime sleepiness in Parkinson disease: an overview

Sleep disturbances are frequent in Parkinson disease. These disorders can be broadly categorized into those that involve nocturnal sleep and excessive daytime sleepiness. The disorders that are often observed during the night in PD include sleep fragmentation that may be due to recurrent PD symptoms, sleep apnea, Restless Leg Syndrome/ periodic limb movements and REM sleep behavior disorder. Excessive daytime sleepiness is also a common occurrence in PD. EDS can arise from several etiologies, and patients may have more than one etiology responsible. The causes of EDS include nocturnal sleep disorder with sleep deprivation and resulting daytime somnolence, the effect of drugs used to treat PD, and possibly neurodegeneration of central sleep/wake areas. Appropriate diagnosis of the sleep disturbance affecting a PD patient can lead to specific treatments that can consolidate nocturnal sleep and enhance daytime alertness.     

Sleep-Disordered Breathing in Neurodegenerative Diseases

Sleep disorders are common in neurodegenerative diseases such as Parkinson’s disease (PD), multiple system atrophy (MSA), amyotrophic lateral sclerosis (ALS), hereditary ataxias, and Alzheimer’s disease (AD). Type, frequency, and severity of sleep disturbances vary depending on each of these diseases. Cell loss of the brainstem nuclei that modulates respiration, and dysfunction of bulbar and diaphragmatic muscles increase the risk for sleep-disordered breathing (SDB) in MSA and ALS. The most relevant SDB in MSA is stridor, whereas in ALS nocturnal hypoventilation due to diaphragmatic weakness is the most common sleep breathing abnormality. Stridor and nocturnal hypoventilation are associated with reduced survival in MSA and ALS. In contrast, sleep apnea seems not to be more prevalent in PD than in the general population. In some PD patients, however, coincidental obstructive sleep apnea (OSA) can be the cause of excessive daytime sleepiness (EDS). SDB can also occur in some hereditary ataxias, such as stridor in spinocerebellar ataxia type 3 (Machado-Joseph disease). The presence of concomitant OSA in patients with AD can have deleterious effects on nocturnal sleep, may result in EDS, and might aggravate the cognitive deficits inherent to the disease. However, whether OSA is more frequent in patients with AD than in the general population is uncertain. Recognition of SDB in neurodegenerative disease is important because they are associated with significant morbidity and potential effective treatments are available.     

Comparison of the clinical features of rapid eye movement sleep behavior disorder in patients with Parkinson's disease and multiple system atrophy

Aims: The aim of this study was to evaluate differences in the clinical presentation and polysomnographic characteristics of rapid eye movement sleep behavior disorder (RBD) between patients with Parkinson's disease (PD) and those with multiple system atrophy (MSA). Methods: We conducted clinical interviews examining RBD symptoms, including violent and non‐violent behaviors, in 49 patients with PD and 16 patients with MSA (as well as their bed partners) and performed polysomnography on all subject patients. Results: Twenty‐seven patients with PD (55.1%) and 11 patients with MSA (68.8%) had rapid eye movement sleep without atonia (RWA) on polysomnogram. The relative amounts of RWA were quite similar between the two groups. For most of the RWA‐positive patients in both groups, RBD symptoms remained non‐violent or silent. RBD symptoms in PD patients seemed to increase with the course of PD, while most of the RBD symptoms in the MSA patients occurred just prior to or at the onset of MSA and then disappeared within a short period. Conclusion: Although PD and MSA frequently accompany RWA, RBD symptoms often remain non‐violent or silent. Differences in the course of RBD symptoms in patients with PD and MSA may reflect the difference in the degeneration process of the two disorders.     

Evaluation of somnolence in Parkinson's disease: comparison with age- and sex-matched controls

The authors found a significantly higher prevalence of daytime somnolence in 201 patients with PD compared with 214 age- and sex-matched healthy control subjects (Epworth Sleepiness Scale score 5.6 vs 4.6). The prevalence of "sleep attacks" (SA) was about seven times higher in patients with PD than in control subjects (13.9% vs 1.9%; p < 0.0005). Multivariate analysis demonstrated that a higher dose of levodopa and longer duration of disease significantly predicted for SA in patients with PD. Epworth Sleepiness Scale scores of > or =10 had 71.4% sensitivity and 88.4% specificity for SA.     

Sleep disturbances and brain MRI morphometry in Parkinson's disease,multiple system atrophy and progressive supranuclear palsy – a comparative study

Despite common reports in Parkinson's disease (PD), in other parkinsonian syndromes, sleep disturbances have been less frequently described. This study evaluated and compared sleep disturbances in patients with PD, multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) and analyzed associations with brain magnetic resonance imaging (MRI) morphometry. This was a cross-sectional study of 16 PD cases, 13 MSA, 14 PSP and 12 control. Sleep disturbances were evaluated by Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index (PSQI), Restless Legs Scale and Berlin questionnaire. Pons area, midbrain area, medial cerebellar peduncle (MCP) width, and superior cerebellar peduncle width were measured using MRI. Poor quality sleep, risk of obstructive sleep apnea (OSA) and restless legs syndrome (RLS) were detected in all groups. Patients with MSA showed higher risk of OSA and less frequent RLS. In MSA, a correlation between PSQI scores and Hoehn and Yahr stage was observed (p < 0.05). In PSP, RLS was frequent (57%) and related with reduced sleep duration and efficiency. In PD, excessive daytime sleepiness was related to atrophy of the MCP (p = 0.01). RLS was more frequent in PD and PSP, and in PSP, was associated with reduced sleep efficiency and sleep duration. Brain morphometry abnormalities were found in connection with excessive daytime sleepiness and risk of OSA in PD and PSP suggesting widespread degeneration of brainstem sleep structures on the basis of sleep abnormalities in these patients.     

Sleep disorders and sleep effect in Parkinson's disease

It has been suggested that sleep may have a positive effect on morning motor symptoms in Parkinson's disease (PD). We examined this possibility and also looked at common sleep disorders in PD. Seventy-eight PD patients and 43 normal elderly subjects answered a questionnaire. Of the PD patients, 43.6% reported improved motor symptoms in the morning, 37.2% worse, and 19.2% unchanged compared to the rest of the day. No difference was found between morning-better and -worse groups with respect to age, duration or stage of PD; antiparkinsonian medications utilized, and predominant motor symptoms. However, the morning-same group had a shorter duration of PD and less severe disease and required fewer dopaminergic medications. Sleep disorders were seen with equal frequency in the morning-better and -worse groups. Our results suggest that sleep does not have a direct effect on morning motor function. Alterations in morning motor symptomatology probably represent a manifestation of motor fluctuations. Sleep fragmentation and spontaneous daytime dozing occurred much more frequently in PD patients than controls. In addition, nocturnal vocalizations and daytime hallucinations occurred only in the PD group.     

Videourodynamic and sphincter motor unit potential analyses in Parkinson's disease and multiple system atrophy

OBJECTIVES: Urinary dysfunction is a prominent autonomic feature in Parkinson's disease (PD) and multiple system atrophy (MSA), which is not only troublesome but also a cause of morbidity in these disorders. Recent advances in investigative uroneurology offer a better insight into the underlying pathophysiology and appropriate management for urinary dysfunction. METHODS: twenty one patients with PD (15 men, six women, mean age 64 (49-76), mean disease duration 4 years (1-8 years), median Hoehn and Yahr grade 3 (1-4), all taking 300 mg/day of levodopa (100-500 mg)) and 15 with MSA (eight men, seven women, mean age 59 (48-72), mean disease duration 3 years (0.5-6 years)) were recruited. Videourodynamic and sphincter motor unit potential analyses in the patients with PD and MSA were carried out, looking for distinguishing hallmarks that might be useful in the differential diagnosis of these two diseases. RESULTS: Urinary symptoms were found in 72% of patients with PD and in 100% with MSA. Filling phase abnormalities in the videourodynamic study included detrusor hyperreflexia in 81% of patients with PD and 56% with MSA, and uninhibited external sphincter relaxation in 33% of patients with PD and 33% of those with MSA. However, open bladder neck at the start of filling was not seen in patients with PD but was present in 53% of those with MSA, suggestive of internal sphincter denervation. Sphincter motor unit potential analysis showed neurogenic motor unit potentials in 5% of patients with PD and in 93% of those with MSA, suggestive of external sphincter denervation. On voiding, detrusor-external sphincter dyssynergia was not seen in patients with PD but was present in 47% of those with MSA. Pressure-flow analysis showed that the Abrams-Griffiths number, a grading of urethral obstruction (outflow obstruction >40), in PD (40 in women and 43 in men) was larger than that in MSA (12 in women and 28 in men). Weak detrusor in PD (66% of women and 40% of men) was less common than that in MSA (71% of women and 63% of men). Postmicturition residuals >100 ml were absent in patients with PD but were present in 47% of patients with MSA. CONCLUSION: Patients with PD had less severe urinary dysfunction with little evidence of internal or external sphincter denervation, by contrast with the common findings in MSA. The findings of postmicturition residuals >100 ml, detrusor-external sphincter dyssynergia, open bladder neck at the start of bladder filling, and neurogenic sphincter motor unit potentials are highly suggestive of MSA.     

Thought disorders among non-demented outpatients with Parkinson’s disease: prevalence and associated factors

The objectives of the study are to evaluate the prevalence and the associated factors of thought disorders in a large cross-sectional population of non-demented out patients with Parkinson’s disease (PD). Four-hundred and nineteen consecutive non-demented PD patients were studied through the DoPaMiP cross-sectional study. Demographic and clinical variables were recorded, including motor and cognitive status, dependency, depressive and anxious symptoms, dysautonomia and sleep disorders. The presence of thought disorders over the past 15 days was assessed by the Unified Parkinson’s Disease Rating Scale part I. Patients with and without thought disorders were compared using parametric tests. Logistic regression was applied to significant data. Thought disorders were present in 105 patients (25%) including vivid dreams in 83 (19.8%), benign hallucinations in 17 (4.1%), and hallucinations without insight in 5 (1.2%). No patient had delusion. Patients with thought disorders were more dependent than the others. Thought disorders were associated with longer PD duration, greater UPDRS scores and the presence of motor complications. Conversely, UPDRS tremor sub-score was lower in patients without thought disorders. Thought disorders were also associated with dysautonomia, lower MMSE score, depression and sleep disorders. Logistic regression identified PD duration, lower MMSE score, depressive and dysautonomic signs as independent risk factors. In conclusion, mild thought disorders were present in 25% of non-demented outpatients with PD, but hallucinations were present in 5% only. Thought disorders were associated with PD duration, depressive and dysautonomic symptoms and lower MMSE score.     

Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study

OBJECTIVE: Although both orthostatic hypotension and urinary incontinence have been reported in a number of parkinsonian syndromes, such as Parkinson's disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP), differences in the evolution of these features have not been studied systematically in pathologically confirmed cases. METHODS: 77 cases with pathologically confirmed parkinsonian syndromes (PD, n=11; MSA, n=15; DLB, n=14; CBD, n=13; PSP, n=24), collected up to 1994, formed the basis for a multicentre clinicopathological study organised by the NINDS to improve the differential diagnosis of parkinsonian disorders. The present study determined the time course-that is, latency to onset and duration from onset to death, of symptomatic orthostatic hypotension, and urinary incontinence in the NINDS series. Furthermore, the diagnostic validity of a predefined latency to onset within 1 year of disease onset of symptomatic orthostatic hypotension or urinary incontinence was analysed. RESULTS: Significant group differences for latency, but not duration, of symptomatic orthostatic hypotension and urinary incontinence were found. Latencies to onset of either feature were short in patients with MSA, intermediate in patients with DLB, CBD, and PSP, and long in those with PD. Symptomatic orthostatic hypotension occurring within the first year after disease onset predicted MSA in 75% of cases; early urinary incontinence was less predictive for MSA (56%). CONCLUSION: Latency to onset, but not duration, of symptomatic orthostatic hypotension or urinary incontinence differentiates PD from other parkinsonian syndromes, particularly MSA.     

Daytime sleepiness and other sleep disorders in Parkinson's disease.

BACKGROUND: PD is associated with a variety of sleep problems. The dopamine agonists (DA) pramipexole and ropinirole were recently implicated in causing "sleep attacks" and motor vehicle accidents. METHODS: In order to determine the overall rate of subjective sleep problems in PD and to determine if any factors, including specific medications, correlate with sleep pathology, the authors surveyed consecutive patients with PD seen over a 3-month period in a Movement Disorders Clinic. The authors collected demographic and medication data, and the patients completed the Epworth Sleepiness Scale (ESS), questions assessing the presence of restless legs syndrome (RLS), a modified National Sleep Foundation sleep survey, and specific questions regarding falling asleep while driving. RESULTS: A total of 320 patients completed the questionnaire. The authors eliminated 17, six for incomplete data and 11 for having a primary diagnosis other than PD. The mean age of the remaining 303 patients was 67.1 +/- 10.7 years, and the mean duration of PD was 9.1 +/- 5.7 years. The ESS scores averaged 11.1 +/- 5.9, and in 50.2% of patients the score was abnormally high (>10). Stepwise regression analysis found that sleepiness correlated with longer duration of PD (p < 0.001), more advanced PD (p < 0.004), male sex (p < 0.001), and the use of any DA (p < 0.003). The soporific effects of the three most common DA (pramipexole, ropinirole, and pergolide) were similar. Falling asleep while driving was reported by 63/279 (22.6%) of current drivers and correlated with higher ESS scores (p < 0.05). Other sleep disorders, including RLS, were also frequently reported. CONCLUSION: Daytime sleepiness is common in PD and correlates with more advanced and longer duration of PD, and male sex. The DA were also independently associated with daytime sleepiness, but in this group, no single DA was more culpable than the others.     

SCOPA‐sleep and PDSS: Two scales for assessment of sleep disorder in Parkinson's disease

This study evaluated the comparative validity and usefulness of the Parkinson's Disease Sleep Scale (PDSS) and the Scales for Outcomes in PD‐Sleep Scale (SCOPA‐S), two disease‐specific rating scales for assessing sleep disorders in Parkinson's disease (PD). Hoehn and Yahr staging (HY), SCOPA‐Motor, Mini‐Mental State Examination, Clinical Impression of Severity Index for PD, Hospital Anxiety and Depression Scale, EuroQoL, and SCOPA‐Psychosocial, in addition to PDSS and SCOPA‐S (night‐time sleep (NS) and daytime sleepiness (DS) subscales), were applied to 187 consecutive PD patients. PDSS and SCOPA‐S proved similar in acceptability, scaling assumptions, precision, and internal consistency (Cronbach's α = 0.82–0.84). Factor analysis revealed five separate factors for PDSS (67% of the variance) and one factor for each SCOPA‐S subscale (60% of the variance for NS and 57% for DS). Correlation coefficient between PDSS and SCOPA‐S NS was ?0.60. Sleep scales correlated moderately with mood, low‐to‐moderate with HRQoL, and low with the rest of measures. PDSS and SCOPA‐S DS discriminated between patients grouped by HY severity levels and disease duration. Cutoff points of 82/83 for PDSS and 6/7 for SCOPA‐S NS were drawn to identify PD patients with sleep problems. Depression/anxiety scores explained 26% for PDSS and 22% for SCOPA‐S NS scores. Both scales provide valid, reliable, and useful means to evaluate sleep disorders in PD. PDSS may be used to obtain a profile about potential causes of “bad sleep,” but is barely useful to assess DS, whereas SCOPA‐S assesses nocturnal sleep disorders and daytime somnolence at a similar extent, without exploring the potential causes. © 2008 Movement Disorder Society     

Incidence of Parkinson's disease and parkinsonism in northern Sweden: A population‐based study

The differential diagnosis of parkinsonian disorders is difficult, especially early in the course of the diseases. The clinical subtypes of Parkinson's disease (PD) have not so far been described in newly diagnosed patients. We present a prospective incidence cohort study of patients with idiopathic parkinsonian syndromes in the Umeå region in northern Sweden identified over a 4‐year period. The clinical diagnoses were re‐evaluated at follow‐up visits at 12 months. We found 138 patients with parkinsonism: 112 PD, 12 multiple system atrophy with predominant parkinsonism (MSA‐P), six progressive supranuclear palsy (PSP) and eight unclassifiable patients. The crude incidences for all age ranges per 100,000 were: PD 19.7 (95% confidence interval 16.1–23.3); MSA‐P 2.1 (1.1–3.7); PSP 1.1 (0.4–2.4); idiopathic parkinsonism 24.3 (20.2–28.4). Age‐standardized to the average Swedish population 2004–2007: PD 22.5 (18.3–26.7); MSA‐P 2.4 (1.2–4.2); PSP 1.2 (0.4–2.6); idiopathic parkinsonism 27.5 (22.9–32.1). The crude annual incidence rate for PD, with exclusion of patients with normal dopamine receptor uptake (FP‐CIT‐SPECT), was 18.8 per 100,000 (95% confidence interval 15.2–22.4), age‐adjusted to the average Swedish population 2004 to 2007: 21.5 (17.4–25.6). The incidence rates did not differ significantly between men and women. The cumulative incidence of PD up to 89 years of age was for men 3.4%, for women 2.6%, and for both sexes combined 2.9%. The annual incidence rates found for PD, idiopathic parkinsonism, MSA‐P and PSP are among the highest reported. © 2010 Movement Disorder Society     

Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy.

Our objective was to examine whether rapid eye movement (REM) sleep behavior disorder occurs in disproportionally greater frequency in multiple system atrophy (MSA), Parkinson's disease (PD), and dementia with Lewy bodies (DLB), collectively known as the synucleinopathies, compared to other nonsynucleinopathy neurodegenerative disorders. In study 1, we reviewed the clinical records of 398 consecutive patients evaluated at Mayo Clinic Rochester for parkinsonism and/or cognitive impairment. The frequency of suspected and polysomnogram (PSG)-confirmed REM sleep behavior disorder (RBD) among subjects with the synucleinopathies MSA, PD, or DLB was compared to the frequency among subjects with the nonsynucleinopathies Alzheimer's disease (AD), frontotemporal dementia (FTD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), mild cognitive impairment (MCI), primary progressive aphasia (PPA), and posterior cortical atrophy (PCA). In study 2, we reviewed the clinical records of 360 consecutive patients evaluated at Mayo Clinic Jacksonville for parkinsonism and/or cognitive impairment. The frequency of probable RBD among patients with PD and DLB was compared to the frequency among patients with AD and MCI. In study 3, we reviewed the brain biopsy or postmortem autopsy diagnoses of 23 Mayo Clinic Rochester patients who had been clinically examined for possible RBD and a neurodegenerative disorder. In study 1, patients with MSA, PD, or DLB were more likely to have probable and PSG-confirmed RBD compared to subjects with the nonsynucleinopathies (probable RBD 77/120=64% vs. 7/278=3%, p < 0.01; PSG-confirmed RBD 47/120=39% vs. 1/278=0%, p < 0.01). In study 2, patients with PD and DLB were more likely to have probable RBD compared to those with AD and MCI (56% vs. 2%, p < 0.01). In study 3, of the 23 autopsied patients who had been questioned about possible RBD, 10 were clinically diagnosed with RBD. The neuropathologic diagnoses in these 10 included Lewy body disease in nine, and MSA in one. Of the other 13 cases, 12 did not have a history suggesting RBD, and the one case who did had normal electromyographic atonia during REM sleep on PSG and autopsy findings of PSP. Only one of these 13 had a synucleinopathy. The positive predictive values for RBD indicating a synucleinopathy for studies 1-3 were 91.7%, 94.3%, and 100.0%, respectively. Clinically suspected and PSG-proven RBD occurs with disproportionally greater frequency in MSA, PD, and DLB compared to other neurodegenerative disorders. In the setting of degenerative dementia and/or parkinsonism, we hypothesize that RBD is a manifestation of an evolving synucleinopathy.     

Continuous positive airway pressure for sleep-related breathing disorders in multiple system atrophy: long-term acceptance

BACKGROUND AND PURPOSE: To assess the long-term acceptance of non-invasive nasal continuous positive air pressure (CPAP) in multiple system atrophy (MSA) patients with polysomnographic (PSG)-confirmed sleep apneas and/or nocturnal stridor. PATIENTS AND METHODS: Sleep-related breathing disorders were investigated by PSG in 22 MSA patients in whom stridor and sleep-related respiratory disturbances were clinically suspected. Patients in whom the first PSG disclosed either a sleep apnea/hypopnea index (AHI)> or =10 or stridor with or without apneas underwent a second PSG for CPAP titration. RESULTS: Three patients presented with an obstructive sleep apnea syndrome without stridor, whereas 15 patients presented stridor occurring alone or accompanied by apneas. Twelve patients pursued CPAP. Two severely disabled patients died a few days after CPAP initiation, and five discontinued CPAP because of discomfort. One patient died after 17 months of follow-up. Since the onset of CPAP, the four remaining patients reported more efficacious sleep and improved daytime alertness. These patients had significantly less severe disease at the time of CPAP initiation. Age, disease duration, the presence of sleep complaints, excessive daytime somnolence (EDS) and AHI did not account for CPAP compliance. CONCLUSION: The severity of motor impairment at the initiation of treatment appears to be the most significant limiting factor for CPAP long-term acceptance.