sleep study motor neuron disease

BACKGROUND: Parkinson's disease (PD) is a neurodegenerative disorder that features motor and non-motor deficits. The use of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced dopamine neuron degeneration has been widely practiced to produce reliable animal models of PD. However, most previous preclinical studies focused on motor dysfunction, and few non-motor symptoms were evaluated. Thus far, there is a lack of comprehensive investigations of the non-motor symptoms in animal models...

BACKGROUND: Spinal muscular atrophy (SMA) is a severe neuromuscular disorder, the phenotype of the disease is caused by the mutation of the SMN1 (survival motor neuron 1) gene which encodes for the SMN protein. Innovative treatments for SMA have become available and the first molecule approved is Nusinersen, an antisense oligonucleotide that increases the production of SMN protein. Nusinersen has been shown to be associated with a significant motor improvement and an increase of the event-free survival...

Sleep disturbances are commonly non-motor symptoms in Parkinson's diseases (PD). However, standard dopamine replacement therapies for the treatment of motor symptoms often prove inadequate in combating sleep disturbances. Previous studies conducted by our research group have reported the neuroprotective effects of tenuigenin, a natural extract from Polygala tenuifolia root, which has been traditionally employed in treating insomnia. The objective of this study was to investigate the potential of tenuigenin in modulating sleep-wake behaviors and elucidate the underlying mechanisms...

Sleep-wake cycle disorders most often accompany the elderly and are frequently associated with the development of neurodegenerative processes, primarily Alzheimer's disease. Sleep disturbances can be diagnosed in patients with AD even before the onset of memory and cognitive impairment, and become more pronounced as the disease progresses. Therefore, the expansion of our knowledge of how sleep relates to AD pathogenesis needs to be addressed as soon as possible. Here, we investigated the influence of chronic sleep deprivation on the motor and orienting-exploratory activity of 5xFAD mice, as well as their spatial learning ability and long-term memory retention...

Type-B monoamine oxidase inhibitors, encompassing selegiline, rasagiline, and safinamide, are available to treat Parkinson's disease. These drugs ameliorate motor symptoms and improve motor fluctuation in the advanced stages of the disease. There is also evidence supporting the benefit of type-B monoamine oxidase inhibitors on non-motor symptoms of Parkinson's disease, such as mood deflection, cognitive impairment, sleep disturbances, and fatigue. Preclinical studies indicate that type-B monoamine oxidase inhibitors hold a strong neuroprotective potential in Parkinson's disease and other neurodegenerative diseases for reducing oxidative stress and stimulating the production and release of neurotrophic factors, particularly glial cell line-derived neurotrophic factor, which support dopaminergic neurons...

BACKGROUND: Brain-derived neurotrophic factor (BDNF) promotes neuroprotection and neuroregeneration. BDNF enhances the survival of dopaminergic neurons and improves dopaminergic neurotransmission and motor performance in patients with Parkinson's disease (PD). However, the association between BDNF levels and rapid eye movement (REM) sleep behavior disorder (RBD) in PD patients has received limited attention. METHODS: We employed the Rapid Eye Movement Sleep Behavior Disorder Questionnaire-Hong Kong version (RBDQ-HK) and the Rapid Eye Movement Sleep Behavior Disorder Screening Questionnaire (RBDSQ) for RBD diagnosis...

Rationale: Clinical care guidelines advise that lung volume recruitment (LVR) be performed routinely by people with neuromuscular disease (NMD) to maintain lung and chest wall flexibility and slow lung function decline. However, the evidence base is limited, and no randomized controlled trials of regular LVR in adults have been published. Objectives: To evaluate the effect of regular LVR on respiratory function and quality of life in adults with NMD. Methods: A randomized controlled trial with assessor blinding was conducted between September 2015 and May 2019...

BACKGROUND: It has been suggested that the loss of nigrostriatal dopaminergic axon terminals occurs before the loss of dopaminergic neurons in the substantia nigra (SN) in Parkinson's disease (PD). This study aimed to use free-water imaging to evaluate microstructural changes in the dorsoposterior putamen (DPP) of idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) patients, which is considered a prodromal stage of synucleinopathies. METHODS: Free water values in the DPP, dorsoanterior putamen (DAP), and posterior SN were compared between the healthy controls (n = 48), iRBD (n = 43) and PD (n = 47) patients...

BACKGROUND: The glucocerebrosidase (GBA) and leucine-rich repeat kinase 2 (LRRK2) genes are associated with the risk of sporadic Parkinson's disease (PD). As an environmental factor, hypoxic insults may impair dopamine neurons in the substantia nigra and exacerbate PD symptoms. However, covariants of GBA and LRRK2 combined with hypoxic insults in clinical cases of Parkinsonism have not yet been reported. CASE PRESENTATION: A 69-year-old male patient with PD and his relatives were clinically characterized and sequenced using the whole-exome technique...

Sleep benefits the restoration of energy metabolism and thereby supports neuronal plasticity and cognitive behaviors. Sirt6 is a NAD+ -dependent protein deacetylase that has been recognized as an essential regulator of energy metabolism because it modulates various transcriptional regulators and metabolic enzymes. The aim of this study was to investigate the influence of Sirt6 on cerebral function after chronic sleep deprivation (CSD). We assigned C57BL/6J mice to control or two CSD groups and subjected them to AAV2/9-CMV-EGFP or AAV2/9-CMV-Sirt6-EGFP infection in the prelimbic cortex (PrL)...

Cross-frequency coupling (CFC) reflects (nonlinear) interactions between signals of different frequencies. Evidence from both patient and healthy participant studies suggests that CFC plays an essential role in neuronal computation, interregional interaction, and disease pathophysiology. The present review discusses methodological advances and challenges in the computation of CFC with particular emphasis on potential solutions to spurious coupling, inferring intrinsic rhythms in a targeted frequency band, and causal interferences...

Dopamine is an important neurotransmitter that plays a biological role by binding to dopamine receptors. The dopaminergic system regulates neural activities, such as reward and punishment, memory, motor control, emotion, and sleep-wake. Numerous studies have confirmed that the dopaminergic system has the function of maintaining wakefulness in the body. In recent years, there has been increasing evidence that the sleep-wake cycle in the brain has similar neurobrain network mechanisms to those associated with the loss and recovery of consciousness induced by general anesthesia...

BACKGROUND: Sleep disorders are frequently associated with Parkinson's disease. Obstructive sleep apnea syndrome is one of these sleep disorders and is associated with the severity of motor symptoms in Parkinson's disease. Obstructive sleep apnea can lead to dopaminergic neuronal cell degeneration and may impair the clearance of α-synuclein in Parkinson's disease. Striatal dopamine uptake is a surrogate marker of nigral dopaminergic cell damage. OBJECTIVE: We aimed to investigate the differences in striatal dopamine availability between Parkinson's disease patients with or without obstructive sleep apnea...

Parkinson's disease (PD) is a complex illness with a constellation of environmental insults and genetic vulnerabilities being implicated. Strikingly, many studies only focus on the cardinal motor symptoms of the disease and fail to appreciate the major non-motor features which typically occur early in the disease process and are debilitating. Common comorbid psychiatric features, notably clinical depression, as well as anxiety and sleep disorders are thought to emerge before the onset of prominent motor deficits...

INTRODUCTION: Idiopathic Parkinson's Disease is a chronic, progressive, neurodegenerative disease that affects the substantia nigra pars compacta and dopaminergic neurons in the brain stem. Since zonulin and claudin-5 are involved in intestinal and brain endothelial permeability and it is hypothesized that the brain-gut axis is relevant in IPD, the aim of our study is to evaluate whether the relationship between Zonulin and Claudin-5 levels and Parkinson's Disease patients. METHODS: A total of 139 patients, including 34 mild, 33 moderate, 39 severe stage patients and 33 healthy controls were included...

Motor symptoms in Parkinson's disease (PD) are caused by degeneration of dopamine (DA) neurons of the substantia nigra (SN), while early non-motor symptoms such as anxiety and sleep disturbances are likely mediated by dysfunction of locus coeruleus (LC) norepinephrine (NE) neurons. The LC develops α-synuclein pathology prior to SN DA neurons in PD, and later undergoes degeneration, but the mechanisms responsible for its vulnerability are unknown. The SN and LC are the only structures in the brain that produces appreciable amounts of neuromelanin (NM), a dark cytoplasmic pigment...

STUDY OBJECTIVES: Exposure to postnatal chronic intermittent hypoxia (pCIH), as experienced in sleep-disordered breathing, is a risk factor for developing cardiorespiratory diseases in adulthood. pCIH causes respiratory instability and motor dysfunction that persist until adult life. In this study, we investigated the impact of pCIH on the sympathetic control of arterial pressure in rats. METHODS AND RESULTS: Neonate male Holtzman rats (P0-1) were exposed to pCIH (6% O2 for 30 s, every 10 min, 8 h/day) during their first 10-15 days of life, while control animals were maintained under normoxia...

BACKGROUND: In palliative care, the relief of discomfort is sought by an overall approach, combining prescribed medication and additional therapies, such as foot reflexology (FR). The main objective of this study was to assess the feasibility of FR in a population of inpatients in a palliative care unit (PCU).The precariousness of the patients led us to perform a feasibility study and not a cohort study from the outset. Its secondary objective was to assess the impact of an FR session on some symptoms of discomfort (anxiety, pain, troubled sleep, and psychological distress)...

Parkinson's disease (PD) is the second most common neurodegenerative disorder affecting the body and mind of millions of people in the world. As PD progresses, bradykinesia, rigidity, and tremor worsen. These motor symptoms are associated with the neurodegeneration of dopaminergic neurons in the substantia nigra. PD is also associated with non-motor symptoms, including loss of smell (hyposmia), sleep disturbances, depression, anxiety, and cognitive impairment. This broad spectrum of non-motor symptoms is in part due to olfactory and hippocampal dysfunctions...

Parkinson's disease (PD) is the second most common neurodegenerative disease after Alzheimer's disease (AD). The pathologic hallmarks of the disease are the loss of dopaminergic neurons of substantia nigra pars compacta and the presence of intraneuronal alpha synuclein (a-syn) aggregates. Clinical features of PD include motor symptoms such as bradykinesia, rigidity, tremors, postural instability, and gait impairment, and non-motor symptoms such as constipation, orthostatic hypotension, REM sleep disorder, depression and dementia...