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https://www.readbyqxmd.com/read/29718878/fatal-familial-insomnia-presenting-with-agrypnia-excitata-and-very-low-atonia-index-level-a-case-report-and-literature-review
#1
Tae-Won Yang, Byeongsu Park, Keun Tae Kim, Jin-Sun Jun, Young-Soo Kim, Soon-Tae Lee, Keun-Hwa Jung, Kon Chu, Sang Kun Lee, Ki-Young Jung
RATIONALE: Fatal familial insomnia (FFI) is a human prion disease that is characterized by sleep-wake cycle deterioration, loss of slow-wave sleep, and motor overactivation over the daily 24-hour period. PATIENT CONCERNS: Here, we report the case of a 57-year-old man who had an irregular sleep-wake cycle and exhibited frequent movements and vocalizations during sleep. DIAGNOSES: Video-polysomnography showed disrupted sleep structure, rapid alternation between sleep stages, and an absence of sleep spindles and slow-wave sleep...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29245265/agrypnia-excitata-and-obstructive-apnea-in-a-patient-with-fatal-familial-insomnia-from-china-a-case-report
#2
Congcong Sun, Wen Xia, Ying Liu, Guoyong Jia, Cuilan Wang, Chuanzhu Yan, Yi Li
RATIONALE: Fatal familial insomnia (FFI) linked to a D178N/129M haplotype mutation in the PRNP gene is the most common genetic prion disease in the Han Chinese population. Here, we describe a Han Chinese patient with FFI who exhibited agrypnia excitata and obstructive apnea. PATIENT CONCERNS: A 46-year-old man displayed involuntary movements during sleep time, snoring, autonomic nervous system dysfunction, cognitive deficit, brainstem symptoms, myoclonus and ataxia in order within 8 months...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26873956/fulminant-corticobasal-degeneration-agrypnia-excitata-in-corticobasal-syndrome
#3
Federico Rodriguez-Porcel, Lindsey Lowder, Rosa Rademakers, Thomas Ravenscroft, Bernardino Ghetti, Mathew C Hagen, Alberto J Espay
No abstract text is available yet for this article.
March 22, 2016: Neurology
https://www.readbyqxmd.com/read/26431902/from-state-dissociation-to-status-dissociatus
#4
REVIEW
Elena Antelmi, Raffaele Ferri, Alex Iranzo, Isabelle Arnulf, Yves Dauvilliers, Kailash P Bhatia, Rocco Liguori, Carlos H Schenck, Giuseppe Plazzi
The states of being are conventionally defined by the simultaneous occurrence of behavioral, neurophysiological and autonomic descriptors. State dissociation disorders are due to the intrusion of features typical of a different state into an ongoing state. Disorders related to these conditions are classified according to the ongoing main state and comprise: 1) Dissociation from prevailing wakefulness as seen in hypnagogic or hypnopompic hallucinations, automatic behaviors, sleep drunkenness, cataplexy and sleep paralysis 2) Dissociation from rapid eye movement (REM) sleep as seen in REM sleep behavior disorder and lucid dreaming and 3) Dissociation from NREM sleep as seen in the disorders of arousal...
August 2016: Sleep Medicine Reviews
https://www.readbyqxmd.com/read/24796072/-meta-analysis-on-acupuncture-moxibustion-for-amblyopia-correction-of-agrypnia
#5
REVIEW
Ying-Hua Hu, Tie Li, Dong-Yue Han, Xi-Chen Wang, Fu-Chun Wang, Zhi-Hong Wang
The effectiveness and safety of acupuncture-moxibustion for the treatment of agrypnia was systematically reviewed. The clinical randomized controlled trial (RCT) of acupuncture-moxibustion for agrypnia were collected. The literature and document on acupuncture-moxibustion RCT for the treatment of agrypnia that published from January of 2001 to March of 2012 was searched with computer in PubMed, CNKI, Wanfang and VIP database. According to Cochrane Handbook 5. 1. 0, the bias risk and quality assessment were performed on every included trial and RevMan 5...
February 2014: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
https://www.readbyqxmd.com/read/24210608/oculomasticatory-myorhythmia-and-agrypnia-excitata-guide-the-diagnosis-of-whipple-disease
#6
Giovanna Calandra-Buonaura, Federica Provini, Pietro Guaraldi, Fabio Pizza, Annagrazia Cecere, Giorgio Barletta, Elio Lugaresi, Giulia Pierangeli, Pietro Cortelli
No abstract text is available yet for this article.
December 2013: Sleep Medicine
https://www.readbyqxmd.com/read/23617938/characteristics-and-related-factors-of-nonfatal-injuries-among-adolescents-and-college-students-in-shenzhen-city-of-china
#7
Li Zhou, Dingyan Chen, Guoying Dong
BACKGROUND: Injuries impact adolescents and young adults in unique ways. The purpose of this study was to determine the incidence rate of nonfatal injuries, and identify characteristics and risk factors for the injuries among adolescents and college students in Shenzhen, China. METHODS: A total of 4,138 students from 79 classes were selected using a purposive sampling method in 2010. The questionnaire included personal demographics, behavioral factors, and self-perceived agrypnia...
2013: BMC Public Health
https://www.readbyqxmd.com/read/23423537/agrypnia-excitata
#8
REVIEW
Federica Provini
Agrypnia (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...
April 2013: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/22763207/augustin-morvan-1819-1897-a-little-known-rural-physician-and-neurologist
#9
O Walusinski, J Honnorat
Augustin Morvan (1819-1897) was a contemporary of Jean-Martin Charcot who practised medicine in rural Brittany. A perspicacious and astute clinician, he described three clinical pictures not previously isolated: in 1875 the semiology of myxoedema, in 1883 the neurological semiology of syringomyelia which he called "paretic analgesia of the upper extremities", and finally in 1890 the semiology of "fibrillary chorea", currently considered a model of synaptic pathology involving immunological damage to potassium channels and causing (as perfectly described by Morvan) myokymia, autonomic nervous system disturbances and agrypnia...
January 2013: Revue Neurologique
https://www.readbyqxmd.com/read/22136903/oneiric-stupor-the-peculiar-behaviour-of-agrypnia-excitata
#10
Pietro Guaraldi, Giovanna Calandra-Buonaura, Rossana Terlizzi, Pasquale Montagna, Elio Lugaresi, Paolo Tinuper, Pietro Cortelli, Federica Provini
Agrypnia excitata (AE) is a syndrome characterized by the inability to sleep associated with a generalized motor and autonomic over-activation. AE is caused by a thalamo-limbic system dysfunction and comprises three different conditions: Fatal Familial Insomnia (FFI), Delirium Tremens (DT), and Morvan Syndrome (MS). Oneiric Stupor episodes (OS) are the peculiar motor behaviour of AE. During OS patients perform simple automatic gestures mimicking daily-life activities. This paper is the first description of the different characteristics of OS in two patients with MS and another with FFI, emphasizing the specific clinical features that reliably differentiate OS from REM sleep behaviour disorders...
December 2011: Sleep Medicine
https://www.readbyqxmd.com/read/22136896/agrypnia-excitata
#11
REVIEW
Elio Lugaresi, Federica Provini, Pietro Cortelli
The concept of Agrypnia excitata (AE) was originally proposed as a concept deriving from the clinical and anatomo-pathological observations obtained in three different diseases, Fatal familial insomnia (FFI), Delirium tremens (DT), and Morvan syndrome (MS). Agrypnia refers to a condition of severely reduced or absent sleep due to organic disorders. Excitata refers to the association of agrypnia with generalized motor and autonomic hyperactivation. AE is a syndrome that has been claimed to relate to a dysfunction in the thalamo-limbic circuits that govern sleep-wake cycles and autonomic activities...
December 2011: Sleep Medicine
https://www.readbyqxmd.com/read/22033118/morvan-chorea-and-agrypnia-excitata-when-video-polysomnographic-recording-guides-the-diagnosis
#12
Federica Provini, Sara Marconi, Marcello Amadori, Pietro Guaraldi, Giulia Pierangeli, Pietro Cortelli, Elio Lugaresi, Pasquale Montagna, Paolo Tinuper
INTRODUCTION: Morvan chorea is an antibody-mediated limbic encephalopathy characterized by severe insomnia, mental confusion, hallucinations, enacted dreams, hyperhidrosis, and neuromyotonia. CASE DESCRIPTION: In a 78 years old man presenting with progressive insomnia apathy and depression, a video-polysomnogram documented enacted dreams mimicking daily life activity (oneiric stupor). This finding led us to perform a search for serum antibodies to voltage-gated K+ channels, which was positive...
December 2011: Sleep Medicine
https://www.readbyqxmd.com/read/19622683/agrypnia-excitata-current-concepts-and-future-prospects-in-management
#13
REVIEW
Ribhi Hazin, Jamil Y Abuzetun, Pierre Giglio, Faisal Khan
Agrypnia excitata is an extremely rare, life-threatening syndrome characterized by autonomic activation, persistent insomnia, and generalized overactivity. Agrypnia excitata describes a triad of three separate conditions: delirium tremens, Morvan's chorea, and familial fatal insomnia (FFI). Each of the aforementioned three conditions have sleep disturbances as a unifying theme and results in distinct neurophysiological findings. The following is an overview of agrypnia excitata with a particular emphasis placed upon each of the three individual conditions that constitute the syndrome with recommendations on appropriate management...
2009: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/19442577/agrypnia-excitata-a-microneurographic-study-of-muscle-sympathetic-nerve-activity
#14
V Donadio, P Montagna, M Pennisi, R Rinaldi, V Di Stasi, P Avoni, E Bugiardini, M P Giannoccaro, P Cortelli, G Plazzi, A Baruzzi, R Liguori
OBJECTIVE: Agrypnia Excitata (AE) is characterized by autonomic over-activity and cardiovascular fluctuations but direct evidence of sympathoexcitation is lacking. AE is a common feature of acquired (i.e. Morvan's syndrome--MS) and genetic (i.e. fatal familial insomnia--FFI) conditions where a dysfunction of the thalamo-limbic system has been suggested. The aim of this study is to report the first microneurographic recordings of sympathetic activity in acquired and genetic AE to investigate the pattern of sympathetic activation...
June 2009: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/19213035/case-report-adult-phenotype-of-mulvihill-smith-syndrome
#15
Tatsuhiko Yagihashi, Motoichiro Kato, Kosuke Izumi, Rika Kosaki, Kaori Yago, Kazuo Tsubota, Yuji Sato, Minoru Okubo, Goro Watanabe, Takao Takahashi, Kenjiro Kosaki
Mulvihill-Smith syndrome (MSS) is characterized by premature aging, multiple pigmented nevi, decreased facial subcutaneous fat, microcephaly, short stature, mental retardation and recurrent infections, however the adult phenotype of MSS has yet to be delineated. We report a 28-year-old woman with Mulvihill-Smith syndrome, who had a solid pseudopapillary cystic tumor of her pancreas at age 17 years. Her distinctive sleep pattern includes severe insomnia with disappearance of sleep spindles and K-complexes, persisting muscle tone, and loss of slow wave sleep...
March 2009: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/18805303/fatal-insomnia-and-agrypnia-excitata-sleep-and-the-limbic-system
#16
REVIEW
F Provini, P Cortelli, P Montagna, P Gambetti, E Lugaresi
Fatal familial insomnia, a human prion disease, Morvan's chorea, an autoimmune limbic encephalopathy, and delirium tremens, the well-known alcohol (or benzodiazepine [BDZ]) withdrawal syndrome, share a clinical phenotype largely consisting in an inability to sleep associated with motor and autonomic activation. Agrypnia excitata is the term which aptly defines this clinical condition, whose pathogenetic mechanism consists in an intralimbic disconnection releasing the hypothalamus and brainstem reticular formation from corticolimbic inhibitory control...
August 2008: Revue Neurologique
https://www.readbyqxmd.com/read/17943067/fatal-familial-insomnia-and-agrypnia-excitata
#17
REVIEW
Elio Lugaresi, Federica Provini
This review summarizes the pioneering steps culminating in the identification of a novel disease, fatal familial insomnia (FFI), a hereditary prion disease. Together with Morvan's chorea and delirium tremens, FFI is characterized by an inability to sleep associated with motor and autonomic overactivation. We named this pattern agrypnia excitata, a syndrome caused by a dysfunction in thalamolimbic circuits. This review highlights the strategic role of the limbic thalamus in the central autonomic network running from the limbic cortex to the lower brainstem and regulating sleep and wakefulness...
2007: Reviews in Neurological Diseases
https://www.readbyqxmd.com/read/17679681/agrypnia-excitata-in-fatal-familial-insomnia-a-video-polygraphic-study
#18
J Iriarte, T Ayuso, C Echavarri, M Alegre, E Urrestarazu, F Lacruz, J Gállego, J Artieda
No abstract text is available yet for this article.
August 7, 2007: Neurology
https://www.readbyqxmd.com/read/17493345/-peripheral-blood-stem-cell-mobilization-with-low-dose-rhg-csf-in-56-unrelated-healthy-donors
#19
Pei Li, Guo-Yun Zhang, Pin Zhu, Bei-Qian Wu, Qiang Niu, Yi Xie
The study was aimed to observe the effect of recombinant human granulocyte-colony stimulating factors (rhG-CSF) in low dose on peripheral blood stem cell (PBSC) mobilization in unrelated healthy normal donors. G-CSF was administered at 5 microg/(kg x d) subcutaneously for successive 5 or 6 days to 56 unrelated donors. Stem cells were harvested on the fourth and fifth days or on the fifth and sixth days. The numbers of mononuclear cells (MNC), CD34(+) cells and Hb, Plt, and CD3(+), CD4(+), CD8(+) and CD20(+) cells were determined during the mobilization...
April 2007: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/17336294/a-human-anti-neuronal-autoantibody-against-gaba-b-receptor-induces-experimental-autoimmune-agrypnia
#20
Giovanni Frisullo, Giacomo Della Marca, Massimiliano Mirabella, Marcella Caggiula, Aldobrando Broccolini, Marco Rubino, Gioacchino Mennuni, Pietro Attilio Tonali, Anna Paola Batocchi
In the serum and cerebrospinal fluid of a patient with recurrent acute episodes of respiratory crises, autonomic symptoms and total insomnia (agrypnia), we identified a novel anti-neural complement fixing antibody directed against GABA(B) receptor (GABA(B)R). Patient purified IgG recognized a band of approximately 110 kDa on protein extracts of mouse cerebellum, cortex and brainstem and immunolabelled cultured Chinese hamster ovary (CHO) cells, transfected with human GABA(B)R1 and rat GABA(B)R2 receptors. Western blot analysis of transfected CHO homogenates showed the same band using both patient purified IgG and anti-GABA(B)R1 antibody...
April 2007: Experimental Neurology
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