Oneiric Stupor (OS) in Agrypnia Excitata represents a peculiar condition characterized by the recurrence of stereotyped gestures such as mimicking daily-life activities associated with the reporting ...of a dream mentation consisting in a single oneiric scene. It arises in the context of a completely disorganized sleep structure lacking any physiological cyclic organization, thus, going beyond the concept of abnormal dream. However, a proper differential diagnosis of OS, in the complex world of the “disorders of dreaming” can become quite challenging. The aim of this review is to provide useful clinical and videopolygraphic data on OS to differentiate it from other dreaming disorders. Each entity will be clinically evaluated among the areas of dream mentation and abnormal sleep behaviors and its polygraphic features will be analyzed and distinguished from OS.
In experimental models, the traditional approach to investigate sleep function is by depriving research animals of sleep.1 However, this approach might have confounding effects due to the stress ...induced by the procedure, potentially affecting the results.1 Generally, in old animals, the effects of ageing outweigh the effects of sleep deprivation. Sleep deprivation contributes to the accumulation of amyloid β and the release of tau, which are proteins associated with Alzheimer's disease.3 The glymphatic clearance of these proteins from the brain occurs during slow-wave sleep. Because slow-wave sleep changes with aging, investigating if changes in the percentage of sleep spent in slow-wave sleep are linked to risk of dementia later in life has become a topic of interest. 346 participants—with a mean age of 69 years—from the community-based Framingham Heart Study Offspring and Omni cohorts whose percentage of slow-wave sleep at baseline was comparable with that of healthy controls, were examined approximately every 4 years to analyse the decline in slow-wave sleep percentage.4 The investigators reported 52 cases of incident dementia, of which 44 were consistent with Alzheimer's disease dementia, over a mean follow-up of 12 years. Obstructive sleep apnoea is also relevant when considering the link between insomnia and increased risk of cardiovascular disease, heart failure, and mortality.5 Investigators assessed the association of three different insomnia presentations with subclinical myocardial injury, as measured by cardiac troponin T, in 2188 participants from the Multi-Ethnic Study of Atherosclerosis: insomnia with comorbid obstructive sleep apnoea, insomnia with objectively short sleep duration, and insomnia with fragmented sleep.6 The insomnia with comorbid obstructive sleep apnoea and the insomnia with short sleep duration were associated with increased circulating troponin T in older adults (the study did not include adults older than 79 years).6 Rapid eye movement (REM) sleep behaviour disorder is an early predictor of eventual phenoconversion to synucleinopathies.7 A study reported in 2023, analysed follow-up data from 1160 people from 28 centres of the International REM Sleep Behavior Disorder Study Group.
A higher frequency of motor and breathing sleep-related disorders in multiple system atrophy (MSA) populations is reported. REM sleep behaviour disorder (RBD) is one of the most robust markers of an ...underlying alpha-synucleinopathy. Although a large corpus of literature documented the higher prevalence of RBD in MSA, few studies have systematically investigated the prevalence of RBD as mode of disease onset and its role in disease progression. Moreover, there has been increasing interest in phenoconversion into synucleinopathies of cohorts of patients with isolated RBD (iRBD). Finally, some studies investigated RBD as predictive factor of conversion in isolated autonomic failure, a synucleinopathy presenting with autonomic failure as the sole clinical manifestation that could convert to a manifest central nervous system synucleinopathy. As the field of neurodegenerative disorders moves increasingly towards developing disease-modifying therapies, detecting individuals in the prodromal stage of these synucleinopathies becomes crucial. The aims of this review are to summarise (1) the prevalence of RBD during the course of MSA and as presenting feature of MSA (iRBD), (2) the RBD features in MSA, (3) MSA progression and prognosis in the subgroup of patients with RBD predating disease onset, and (4) the prevalence of MSA conversion in iRBD cohorts. Moreover, we summarise previous results on the role of RBD in the context of isolated autonomic failure as marker of phenoconversion to other synucleinopathies and, in particular, to MSA.
Sleep disorders (SDs) are one of the most frequent non-motor symptoms of Parkinson's disease (PD), usually increasing in frequency over the course of the disease and disability progression. SDs ...include nocturnal and diurnal manifestations such as insomnia, REM sleep behavior disorder, and excessive daytime sleepiness. The causes of SDs in PD are numerous, including the neurodegeneration process itself, which can disrupt the networks regulating the sleep-wake cycle and deplete a large number of cerebral amines possibly playing a role in the initiation and maintenance of sleep. Despite the significant prevalence of SDs in PD patients, few clinical trials on SDs treatment have been conducted. Our aim is to critically review the principal therapeutic options for the most common SDs in PD. The appropriate diagnosis and treatment of SDs in PD can lead to the consolidation of nocturnal sleep, the enhancement of daytime alertness, and the amelioration of the quality of life of the patients.
Abstract
Objective
The differential diagnosis between sleep-related hypermotor epilepsy (SHE) and disorders of arousal (DOA) may be challenging. We analyzed the stage and the relative time of ...occurrence of parasomnic and epileptic events to test their potential diagnostic accuracy as criteria to discriminate SHE from DOA.
Methods
Video-polysomnography recordings of 89 patients with a definite diagnosis of DOA (59) or SHE (30) were reviewed to define major or minor events and to analyze their stage and relative time of occurrence. The “event distribution index” was defined on the basis of the occurrence of events during the first versus the second part of sleep period time. A group analysis was performed between DOA and SHE patients to identify candidate predictors and to quantify their discriminative performance.
Results
The total number of motor events (i.e. major and minor) was significantly lower in DOA (3.2 ± 2.4) than in SHE patients (6.9 ± 8.3; p = 0.03). Episodes occurred mostly during N3 and N2 in DOA and SHE patients, respectively. The occurrence of at least one major event outside N3 was highly suggestive for SHE (p = 2*e-13; accuracy = 0.898, sensitivity = 0.793, specificity = 0.949). The occurrence of at least one minor event during N3 was highly suggestive for DOA (p = 4*e-5; accuracy = 0.73, sensitivity = 0.733, specificity = 0.723). The “event distribution index” was statistically higher in DOA for total (p = 0.012) and major events (p = 0.0026).
Conclusion
The stage and the relative time of occurrence of minor and major motor manifestations represent useful criteria to discriminate DOA from SHE episodes.
•We evaluated iron deposition in N1 in HC and patients with early PD (ePD) and iRBD.•N1 aspect was pathological in T2*-w images in 45% of iRBD patients and in most ePD.•ePD N1 χ was higher than iRBD ...and HC χ but had no correlation with disease duration.•N1 χ in iRBD was similar to HC but increased with disease duration.•N1 χ may be a presymptomatic biomarker for neurodegeneration in prodromal PD.
Idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) is a prodromal stage of α-synucleinopathies, such as Parkinson's disease (PD), which are characterized by the loss of dopaminergic neurons in substantia nigra, associated with abnormal iron load. The assessment of presymptomatic biomarkers predicting the onset of neurodegenerative disorders is critical for monitoring early signs, screening patients for neuroprotective clinical trials and understanding the causal relationship between iron accumulation processes and disease development. Here, we used Quantitative Susceptibility Mapping (QSM) and 7T MRI to quantify iron deposition in Nigrosome 1 (N1) in early PD (ePD) patients, iRBD patients and healthy controls and investigated group differences and correlation with disease progression. We evaluated the radiological appearance of N1 and analyzed its iron content in 35 ePD, 30 iRBD patients and 14 healthy controls via T2*-weighted sequences and susceptibility (χ) maps. N1 regions of interest (ROIs) were manually drawn on control subjects and warped onto a study-specific template to obtain probabilistic N1 ROIs. For each subject the N1 with the highest mean χ was considered for statistical analysis. The appearance of N1 was rated pathological in 45% of iRBD patients. ePD patients showed increased N1 χ compared to iRBD patients and HC but no correlation with disease duration, indicating that iron load remains stable during the early stages of disease progression. Although no difference was reported in iron content between iRBD and HC, N1 χ in the iRBD group increases as the disease evolves. QSM can reveal temporal changes in N1 iron content and its quantification may represent a valuable presymptomatic biomarker to assess neurodegeneration in the prodromal stages of PD.
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To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD).
Cross-sectional cohort study.
Sleep laboratory.
Thirty HD patients, 16 ...women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y).
Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale UHDMRS), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG).
The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m(2). No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P < 0.02). UHMDRS correlated positively with the ESS score (P < 0.005), and negatively with the percentage of REM sleep.
Patients with Huntington disease showed a severe sleep disruption and a high prevalence of periodic limb movements, but no evidence of sleep disordered breathing or REM sleep behavior disorder.
To compile an objective accurate description of the motor patterns of adult arousal disorders (ADs).
We reviewed 59 nocturnal video-polysomnographic (VPSG) recordings of 30 adult patients (>15 years) ...with a history of sleepwalking (SW). We scrutinized the semeiology of all 184 episodes recorded, classifying them into three groups according to three semeiological motor patterns characterized by increasing intensity and complexity: simple arousal movements (pattern I), characterized by head flexion/extension, head flexion/extension and limb movement or head flexion/extension and partial trunk flexion/extension; rising arousal movements (pattern II), characterized by a complete trunk flexion with patient sitting up in bed; and complex arousal with ambulatory movements (pattern III) characterized by SW. The VPSG recordings were compared to those of 10 healthy controls.
AD patients presented with 169 pattern I, 37 pattern II, and nine pattern III episodes. Pattern I developed into pattern II in 17 cases and into pattern II followed by pattern III in five patients. Pattern II developed into pattern III in four patients. Onset was abrupt in 55% of the episodes. Episodes lasted a mean (±standard deviation) of 33 ± 35 s. Movements tended to halt temporarily during 72% of the episodes. We recorded 248 movements during sleep in the healthy controls, none of whom presented with AD patterns.
We identified three specific motor patterns in AD patients not previously reported and not observed in healthy controls. Identification of these patterns could be important for diagnosis and serve as the basis for a new definition of AD in adults.
•We identified three specific sleep motor patterns in patients with arousal disorders.•No healthy controls presented with the motor patterns of arousal disorders patients.•Motor patterns identified have an increasing intensity and complexity.•Motor pattern recognition is useful to establish arousal disorder diagnoses.
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