590 research outputs found
Fatal familial insomnia and Agrypnia Excitata: Autonomic dysfunctions and pathophysiological implications
No full textFatal Familial Insomnia (FFI) is a hereditary prion disease caused by a mutation at codon 178 of the prion-protein gene leading to a D178N substitution in the protein determining severe and selective atrophy of mediodorsal and anteroventral thalamic nuclei. FFI is characterized by physiological sleep loss, which polygraphically appears to be a slow wave sleep loss, autonomic and motor hyperactivation with peculiar episodes of oneiric stupor. Alteration of autonomic functions is a great burden for FFI patients consisting in sympathetic overactivation, dysregulation of its physiological responses and disruption of circadian rhythms. The cardiovascular system is the most frequently and severely affected confirming the increased sympathetic drive with preserved parasympathetic responses. Sleep loss, autonomic and motor hyperactivation define Agrypnia Excitata (AE), which is not exclusive to FFI, but it has been canonically described also in Morvan Syndrome and Delirium Tremens. These three conditions present different pathophysiological mechanisms but share the same thalamo-limbic impairment of which AE is one of the possible clinical presentations. FFI, and consequently also AE, is a model for the investigation of the essential role of the thalamus in the organization of body homeostasis, integrating both sleep and autonomic function control
Differentiating Oneiric Stupor in Agrypnia Excitata From Dreaming Disorders
No full textOneiric 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
Excessive daytime sleepiness in Parkinson’s disease: the key is beyond sleep macrostructure
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Commentary on “Parasomnias manifest different phenotypes of sleep-related behaviors in age and sex groups. A YouTube-based video research highlighting the age slope of sleepwalking”
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Fragmentary Hypnic Myoclonus and Other Isolated Motor Phenomena of Sleep
No full textExcessive fragmentary hypnic myoclonus, hypnic jerks, hypnagogic foot tremor, alternating leg muscle activation, and sleep-related cramps are less known sleep-related motor disorders (SRMDs). These manifestations are frequently missed or misinterpreted polygraphic findings that can be frequently confused with the more frequent SRMDs. These symptoms can present as isolated motor symptoms but can be also the cause of otherwise cryptogenic insomnias and somnolence. Expanding the knowledge on these isolated symptoms and defining their polygraphic and clinical features are essential for their identification. However, clear cut-offs to discern between the isolated phenomenon and the disorder are still to be found
Sleep syncope: a new clinical entity or just a vasovagal syncope during wakefulness after sleep onset?
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Agrypnia excitata.
No full textAgrypnia (from the Greek: to chase sleep) excitata (AE) is a syndrome characterized by loss of sleep and permanent motor and autonomic hyperactivation (excitata). Disruption of the sleep-wake rhythm consists in the disappearance of spindle-delta activities, and the persistence of stage 1 non-rapid eye movement (NREM) sleep. Rapid eye movement (REM) sleep persists but fails to stabilize, appearing in short recurrent episodes, isolated, or mixed with stage 1 NREM sleep. Diurnal and nocturnal motor, autonomic and hormonal overactivity is the second hallmark of AE. Of particular interest is the finding that norepinephrine secretion is extremely elevated at all hours of the day and night whereas the nocturnal melatonin peak is lacking. Oneiric stupor is probably an exclusive sign of AE and consists in the recurrence of stereotyped gestures mimicking simple daily life activities. Agrypnia excitata aptly defines 3 different clinical conditions, fatal familial insomnia (FFI), an autosomal dominant prion disease, Morvan syndrome (MS), an autoimmune encephalitis, and delirium tremens (DT), the alcohol withdrawal syndrome. Agrypnia excitata is due to an intralimbic disconnection releasing the hypothalamus and brainstem reticular formation from cortico-limbic inhibitory control. This pathogenetic mechanism is visceral thalamus degeneration in FI, whereas it may depend on autoantibodies blocking voltage-gated potassium (VGK) channels within the limbic system in MS, and in the sudden changes in gabaergic synapses down-regulated by chronic alcohol abuse within the limbic system in DT
Parasomnias During the COVID-19 Pandemic
No full textParasomnias are undesirable physical events or experiences that occur during entry into sleep, within sleep, or during arousal from sleep. Parasomnias may occur during non-rapid eye movement sleep (NREM), rapid eye movement sleep (REM), or during transitions to and from sleep.1 Many parasomnias are the result of mixed states with the concomitant presence of multiple statedetermining biomarkers. According to the recent view of sleep as a "complex local phenomenon", different states of consciousness, such as wakefulness, NREM sleep and REM sleep, can concurrently result in these "altered", but not necessarily pathologic,2 states
Mental Activity During Episodes of Sleepwalking, Night Terrors or Confusional Arousals: Differences Between Children and Adults
No full textAnna Castelnovo,1– 3 Giuseppe Loddo,4 Federica Provini,5,6 Silvia Miano,1 Mauro Manconi1,2,7 1Sleep Medicine Unit, Neurocenter of Southern Switzerland, Ospedale Civico, Lugano, Switzerland; 2Faculty of Biomedical Sciences, Università della Svizzera Italiana, Lugano, Switzerland; 3University Hospital of Psychiatry and Psychotherapy, University of Bern, Bern, Switzerland; 4Department of Primary Care, Azienda USL di Bologna, Bologna, Italia; 5IRCSS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italia; 6Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italia; 7Department of Neurology, University Hospital, Inselspital, Bern, SwitzerlandCorrespondence: Anna Castelnovo Ospedale Civico Via Tesserete 46, Lugano, 6900, SwitzerlandEmail [email protected]/Background: Night terrors, sleepwalking and confusional arousals are behavioral manifestations of incomplete awakenings from sleep. According to international diagnostic criteria, these behaviors occur in the absence of any mental experience, or in the presence of very limited cognition or dream imagery (eg, a single visual scene). The aim of this study was to systematically and retrospectively investigate the mental content associated with sleep terrors and/or sleepwalking in both children and adults.Patients and Methods: Forty-five consecutive patients referred for a diagnosis of disorders of arousal (DOA) of all subtypes (sleepwalking/sleep terrors/confusional arousals) (25 adults: 30 ± 6 y, 15 females; 20 children: 10 ± 3 y, 6 females) underwent a detailed semi-structured interview about the mental content associated with their nocturnal episodes. The interview was comprehensive of specific questions about their subjective recall rate, several content details (characters, emotions, actions and setting/context), and hallucinatory or dissociative experiences during clinical episodes. Patients’ reports were classified for complexity (Orlinsky scale) and content (Hall and Van de Castle categories).Results: More than two-third of the children (n = 14) could not recall any mental activity associated with their episodes, whereas more than two-third (n = 16) of the adults recalled at least one mental experience. Half of the adult patients (n = 8) estimated that a specific mental content was subjectively present around 50% or more of the times. Seven adults and one child described clear and vivid hallucinatory experiences of “dreamed” objects or characters projected onto their real home environment, in the absence of any reality testing. Five adults and two children described one or more dissociative experiences. The content of the collected reports was dominated by dynamic actions acted out from a self-perspective, often with apprehension and in response to misfortune and danger, in a home-setting environment.Conclusion: These results suggest that current diagnostic criteria are tailored around the typical presentation of DOA in children, and do not always fit to adult patients with DOA. Furthermore, they support the concept that consciousness may reemerge in DOA patients during clinical episodes, in a peculiar dissociated, psychotic-like form.Keywords: somnambulism, confusional arousal, parasomnia, dream, consciousness, mental content, amnesi
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