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The Neurobiology of Narcolepsy-Cataplexy
Progress in Neurobiology Vol. 41, pp. 533 to 541, 1993 0301-0082/93/$24.00 Printed in Great Britain. All rights reserved © 1993 Pergamon Press Ltd THE NEUROBIOLOGY OF NARCOLEPSY-CATAPLEXY MICHAEL S. ALDRICH Department of Neurology, Sleep Disorders Center, University of Michigan Medical Center, Ann Arbor, MI, U.S.A. (Received 17 July 1992) CONTENTS 1. Introduction 533 2. Clinical aspects 533 2.1. Sleepiness and sleep attacks 533 2.2. Cataplexy and related symptoms 534 2.3. Clinical variants 534 2.3.1. Narcolepsy without cataplexy 534 2.3.2. Idiopathic hypersomnia 534 2.3.3. Symptomatic narcolepsy 534 2.4. Treatment 534 3. Pathophysiology 535 4. Neurobiological studies 535 4.1. The canine model of narcolepsy 535 4.2. Pharmacology of human cataplexy 537 4.3. Postmortem studies 537 5. Genetic and family studies 537 6. Summary and conclusions 539 References 539 1. INTRODUCTION 2. CLINICAL ASPECTS Narcolepsy is a specific neurological disorder Narcolepsy has a prevalence that varies worldwide characterized by excessive sleepiness that cannot be from as little as 0.0002% in Israel to 0.16% in Japan; fully relieved with any amount of sleep and by in North America and Europe the prevalence is about abnormalities of rapid eye movement (REM) 0.03-0.06% (Dement et al., 1972; Honda, 1979; Lavie sleep. About two-thirds of patients also have brief and Peled, 1987). The onset of narcoleptic symptoms, episodes of muscle weakness usually brought on by usually in the second or third decade of life, may emotion, referred to as cataplexy. The disorder gener- occur over a few days or weeks or it may be so ally begins in adolescence and continues throughout gradual that the loss of full alertness is unrecognized life. -
Physiological and Pharmacological Factors of Insomnia in HIV Disease
University of Tennessee, Knoxville TRACE: Tennessee Research and Creative Exchange Faculty Publications and Other Works -- Nursing Nursing January 1999 Physiological and pharmacological factors of insomnia in HIV disease Kenneth D. Phillips University of Tennessee - Knoxville, [email protected] Follow this and additional works at: https://trace.tennessee.edu/utk_nurspubs Part of the Critical Care Nursing Commons Recommended Citation Phillips, K. D. (1999). Physiological and pharmacological factors of insomnia in HIV disease. Journal of the Association of Nurses in AIDS Care, 10(5), 93-97. This Article is brought to you for free and open access by the Nursing at TRACE: Tennessee Research and Creative Exchange. It has been accepted for inclusion in Faculty Publications and Other Works -- Nursing by an authorized administrator of TRACE: Tennessee Research and Creative Exchange. For more information, please contact [email protected]. Clinical Column JANACPhillips /Vol. Insomnia 10, No. and 5, September/OctoberHIV Disease 1999 Physiological and Pharmacological Factors of Insomnia in HIV Disease Kenneth D. Phillips, PhD, RN For almost two decades, HIV infection has been a Ungvarski, 1995), and the side effects of many antiret- progressive disease leading to early morbidity and roviral therapies and other drugs (Chohan, 1999) used mortality for more than a million Americans (Centers to treat HIV disease may produce insomnia. Although for Disease Control and Prevention [CDC], 1998). helping the client manage sleep disturbance is of great Although HIV infection strikes people of any age, it importance, available information in this area remains continues to be a disease of young persons in relatively modest. good health. Persons with HIV (PWHIV) who are in Insomnia frequently begins prior to the diagnosis of the advanced stages of the disease typically experience HIV,and it continues throughout the disease (Norman, very troubling symptoms. -
Daytime Sleepiness
Med Lav 2017; 108, 4: 260-266 DOI: 10.23749/mdl.v108i4.6497 Daytime sleepiness: more than just Obstructive Sleep Apnea (OSA) Luigi Ferini-Strambi, Marco Sforza, Mattia Poletti, Federica Giarrusso, Andrea Galbiati IRCCS San Raffaele Scientific Institute, Department of Clinical Neurosciences and Università Vita-Salute San Raffaele, Milan, Italy KEY WORDS: Daytime sleepiness; Obstructive Sleep Apnea (OSA); hypersomnia PAROLE CHIAVE: Sonnolenza diurna; Apnee Ostruttive del Sonno (OSA); ipersonnia SUMMARY Excessive Daytime Sleepiness (EDS) is a common condition with a significant impact on quality of life and general health. A mild form of sleepiness can be associated with reduced reactivity and modest distractibility symptoms, but more severe symptomatic forms are characterized by an overwhelming and uncontrollable need to sleep, causing sud- den sleep attacks, amnesia and automatic behaviors. The prevalence in the general population is between 10 and 25%. Furthermore, EDS has been considered a core symptom of obstructive sleep apnea (OSA), as well as being the main symptom of primary hypersomnias such as narcolepsy types 1 and 2, and idiopathic hypersomnia. Moreover, it can be considered secondary to other sleep disorders (Restless Legs Syndrome, Chronic insomnia, Periodic Limb Movements), psychiatric conditions (Depression, Bipolar Disorder) or a consequence of the intake/abuse of drugs and/or substances. An accurate medical history cannot be sufficient for the differential diagnosis, therefore instrumental recordings by means of polysomnography and the Multiple Sleep Latency Test (MSLT) are mandatory for a correct diagnosis and treatment of the underlying cause of EDS. RIASSUNTO «Sonnolenza diurna: più di una semplice Apnea Ostruttiva nel Sonno (OSA)». L’eccessiva sonnolenza diurna (Excessive Daytime Sleepiness, EDS) è una condizione molto comune con un impatto significativo sulla qualità di vita e sulla salute in generale. -
Case Report and Review of Literature on Hypersomnia As a Result of Stroke Pradeep C
ISSN: 2638-1583 Madridge Journal of Neuroscience Case Report Open Access Sleepiness after Stroke: Case Report and Review of Literature on Hypersomnia as a Result of Stroke Pradeep C. Bollu1, Ashutosh Pandey2, Siva Prasad Pesala3 and Krishna Nalleballe1* 1Assistant Professor, Department of Neurology, University of Missouri, Columbia, USA 2Albert Einstein School of Medicine, Bronx NY, USA 3Neurologist, Department of Neurology, University of Missouri, Columbia, USA Article Info Keywords: Hypersomnia; Stroke; Daytime sleepiness; Conventional angiogram. *Corresponding author: Abbreviations: MRA: Magnetic resonance angiography; MRI: Magnetic resonance Krishna Nalleballe imaging; PLMS: Periodic limb movements in sleep; NREM: Non rapid eye movement; Assistant Professor SDB: Sleep disordered breathing Department of Neurology University of Missouri, Columbia 65212, USA Introduction Tel. 6032897308/5738821515 E-mail: [email protected] Daytime sleepiness is defined as the inability to stay awake and alert during the major waking episode of the day resulting in irrepressible need for sleep or unintended Received: March 16, 2017 lapses into drowsiness. Excessive sleepiness affects up to 5% of the general population Accepted: March 23, 2017 and can result in significant disability. Hypersomnia can be either the result of unmet Published: March 28, 2017 sleep needs or can also be due to reduced activity of alerting systems in the brain. The Citation: Bollu CP, Pandey A, Pesala SP, former can be due to sleep restriction or from sleep disruption. The International Nalleballe K. Sleepiness after Stroke: Case Classification of Sleep disorders categorize various disorders of hyper somnolence into Report and Review of Literature on Hypersomnia different sub-groups. Exaggerated sleep propensity with daytime sleepiness and/ or as a Result of Stroke. -
Sleep Disorders Preeti Devnani
SPECIAL ISSUE 1: INVITED ARTICLE Sleep Disorders Preeti Devnani ABSTRACT Sleep disorders are an increasingly important and relevant burden faced by society, impacting at the individual, community and global level. Varied presentations and lack of awareness can make accurate and timely diagnosis a challenge. Early recognition and appropriate intervention are a priority. The key characteristics, clinical presentations and management strategies of common sleep disorders such as circadian rhythm disorders, restless legs syndrome, REM behavior disorder, hypersomnia and insomnia are outlined in this review. Keywords: Hypersomnia, Insomnia, REM behavior International Journal of Head and Neck Surgery (2019): 10.5005/jp-journals-10001-1362 INTRODUCTION Department of Neurology and Sleep Disorder, Cleveland Clinic, Abu Sleep disorders are becoming increasingly common in this modern Dhabi, United Arab Emirates era, resulting from several lifestyle changes. These complaints may Corresponding Author: Preeti Devnani, Department of Neurology present excessive daytime sleepiness, lack of sleep or impaired and Sleep Disorder, Cleveland Clinic, Abu Dhabi, United Arab Emirates, quality, sleep related breathing disorders, circadian rhythm disorder e-mail: [email protected] misalignment and abnormal sleep-related movement disorders.1 How to cite this article: Devnani P. Sleep Disorders. Int J Head Neck They are associated with impaired daytime functioning, Surg 2019;10(1):4–8. increased risk of cardiovascular and cerebrovascular disease, poor Source of support: Nil glycemic control, risk of cognitive decline and impaired immunity Conflict of interest: None impacting overall morbidity and mortality. Diagnosis of sleep disorders is clinical in many scenarios, The following circadian rhythm sleep–wake disorders adapted polysomnography is a gold standard for further evaluation of from the ICSD-3: intrinsic sleep disorder such as obstructive sleep apnea (OSA) • Delayed sleep–wake phase disorder and periodic limb movement disorder (PLMD). -
Cognitive Behavioral Therapy for Insomnia Enhances Depression Outcome in Patients with Comorbid Major Depressive Disorder and Insomnia
INSOMNIA AND DEPRESSION Cognitive Behavioral Therapy for Insomnia Enhances Depression Outcome in Patients with Comorbid Major Depressive Disorder and Insomnia Rachel Manber, PhD1; Jack D. Edinger, PhD2; Jenna L. Gress, BA1; Melanie G. San Pedro-Salcedo, MA1; Tracy F. Kuo, PhD1; Tasha Kalista, MA1 1Stanford University, Stanford, CA; 2VA Medical Center and Duke University Medical Center, Durham, NC Study Objective: Insomnia impacts the course of major depressive severity index (ISI), daily sleep diaries, and actigraphy. disorder (MDD), hinders response to treatment, and increases risk for EsCIT + CBTI resulted in a higher rate of remission of depression depressive relapse. This study is an initial evaluation of adding cogni- (61.5%) than EsCIT + CTRL (33.3%). EsCIT + CBTI was also associat- tive behavioral therapy for insomnia (CBTI) to the antidepressant medi- ed with a greater remission from insomnia (50.0%) than EsCIT + CTRL cation escitalopram (EsCIT) in individuals with both disorders. (7.7%) and larger improvement in all diary and actigraphy measures of Design and setting: A randomized, controlled, pilot study in a single sleep, except for total sleep time. academic medical center. Conclusion: This pilot study provides evidence that augmenting an Participants: 30 individuals (61% female, mean age 35±18) with MDD antidepressant medication with a brief, symptom focused, cognitive- and insomnia. behavioral therapy for insomnia is promising for individuals with MDD Interventions: EsCIT and 7 individual therapy sessions of CBTI or and comorbid insomnia in terms of alleviating both depression and in- CTRL (quasi-desensitization). somnia. Measurements and results: Depression was assessed with the Keywords: Major depressive disorder, Insomnia, Cognitive behavioral HRSD17 and the depression portion of the SCID, administered by raters therapy, Remission masked to treatment assignment, at baseline and after 2, 4, 6, 8, and Citation: Manber R; Edinger JD; Gress JL; San Pedro-Salcedo MG; 12 weeks of treatment. -
Periodic Hypersomnia, Congenital Ectodermal Disorders and Multiple Exostosis
PERIODIC HYPERSOMNIA, CONGENITAL ECTODERMAL DISORDERS AND MULTIPLE EXOSTOSIS RUBENS REIMÃO * — ARON DIAMENT ** SUMMARY — A case of periodic hypersomnia in an 11-year-old female with the unique features of mental deficiency, incontinentia pigmenti, acanthosis nigricans and hereditary multiple exostosis (diaphysial aclasis) is reported. The clinical, Polysomnographic and Multiple Sleep Latency test features of this case with a follow up of seven years are consistent with a diagnosis of periodic (intermittent) excessive somnolence. The unique presentation, however, does differ from Kleine-Levin syndrome and suggests a relationship between the predominantly ectodermal, congenital disorders and the sleep-wake, pattern dysfunction. Hipersônia periódica, malformações ectodérmicas e exostose múltipla. RESUMO — Relata-se um caso de sonolência excessiva periódica acompanhada de hiperfagia, com início na segunda década, documentado clinicamente, em tragados polissonográficos de noite inteira e no teste das Latências Múltiplas do Sono. Apresenta características que o distinguem da síndrome de Kleine-Levin, a saber, a ocorrência no sexo feminino, deficiência mental, incontinência pigmentar, acantose nigricans e exostose múltipla. There are two well defined syndromes of periodic hypersomnia!: menstrual cycle-related somnolence and the Kleine-Levin syndrome (KLS). The former is usually temporally linked with the menses; the latter, a rare condition, is generally limited to males in which the first episode of excessive sleepiness occurs in the second decade of life associated with hyperphagia. It supposedly results from hypothalamic or lymbic dysfunctions. This report describes a case of periodic hypersomnia and hyperphagia, beginning in the second decade but clearly distinct from KLS, occurring in a female with mental deficiency, incontinentia pigmenti and multiple exostosis. -
Excessive Daytime Sleepiness and Unintended Sleep Episodes
review article Oman Medical Journal [2015], Vol. 30, No. 1: 3–10 Excessive Daytime Sleepiness and Unintended Sleep Episodes Associated with Parkinson’s Disease Fatai Salawu1* and Abdulfatai Olokoba2 1Department of Medicine, Federal Medical Centre, Yola, Nigeria 2Department of General Internal Medicine, University of Ilorin Teaching Hospital, Ilorin. Nigeria ARTICLE INFO ABSTRACT Article history: This article looks at the issues of excessive daytime sleepiness and unintended sleep Received: 31 December 2012 episodes in patients with Parkinson’s disease (PD) and explores the reasons why patients Accepted: 7 December 2014 might suffer from these symptoms, and what steps could be taken to manage them. During the last decade, understanding of sleep/wake regulation has increased. Several brainstem Online: nuclei and their communication pathways in the ascending arousing system through the DOI 10.5001/omj.2015.02 hypothalamus and thalamus to the cortex play key roles in sleep disorders. Insomnia is Keywords: the most common sleep disorder in PD patients, and excessive daytime sleepiness is also Parkinson Disease; Sleep; common. Excessive daytime sleepiness affects up to 50% of PD patients and a growing Dopamine Agonists; body of research has established this sleep disturbance as a marker of preclinical and Pedunculopontine Nucleus. premotor PD. It is a frequent and highly persistent feature in PD, with multifactorial underlying pathophysiology. Both age and disease-related disturbances of sleep-wake regulation contribute to hypersomnia in PD. Treatment with dopamine agonists also contribute to excessive daytime sleepiness. Effective management of sleep disturbances and excessive daytime sleepiness can greatly improve the quality of life for patients with PD. -
Narcolepsy Caused by Acute Disseminated Encephalomyelitis
OBSERVATION Narcolepsy Caused by Acute Disseminated Encephalomyelitis Richard F. Gledhill, MD, MRCP; Peter R. Bartel, PhD; Yasushi Yoshida, MD, PhD; Seiji Nishino, MD, PhD; Thomas E. Scammell, MD Background: Narcolepsy with cataplexy is caused by ueduct, which are consistent with acute disseminated a selective loss of hypocretin-producing neurons, but nar- encephalomyelitis. colepsy can also result from hypothalamic and rostral brainstem lesions. Results: After treatment with steroids, this patient’s sub- jective sleepiness, hypersomnia, and hypocretin defi- Patient: We describe a 38-year-old woman with severe ciency partially improved. daytime sleepiness, internuclear ophthalmoplegia, and bilateral delayed visual evoked potentials. Her multiple Conclusions: Autoimmune diseases such as acute dis- sleep latency test results demonstrated short sleep laten- seminated encephalomyelitis can produce narcolepsy. cies and 4 sleep-onset rapid eye movement sleep peri- Most likely, this narcolepsy is a consequence of demy- ods, and her cerebrospinal fluid contained a low con- elination and dysfunction of hypocretin pathways, but centration of hypocretin. Magnetic resonance imaging direct injury to the hypocretin neurons may also occur. showed T2 and fluid-attenuated inversion recovery hy- perintensity along the walls of the third ventricle and aq- Arch Neurol. 2004;61:758-760 ARCOLEPSY IS CHARAC- REPORT OF A CASE terized by excessive daytime sleepiness and rapid eye movement A previously healthy, 38-year-old, black, (REM) sleep-related South African woman was admitted to Ga- Nsymptoms such as cataplexy. More than Rankuwa Hospital, Pretoria, Republic of 90% of people with narcolepsy with cata- South Africa, with 6 weeks of severe day- plexy have no detectable hypocretin/ time sleepiness and hypersomnia (total orexin in their cerebrospinal fluid sleep time about 16 of every 24 h). -
The Co-Occurrence of Sexsomnia, Sleep Bruxism and Other Sleep Disorders
Journal of Clinical Medicine Review The Co-Occurrence of Sexsomnia, Sleep Bruxism and Other Sleep Disorders Helena Martynowicz 1, Joanna Smardz 2, Tomasz Wieczorek 3, Grzegorz Mazur 1, Rafal Poreba 1, Robert Skomro 4, Marek Zietek 5, Anna Wojakowska 1, Monika Michalek 1 ID and Mieszko Wieckiewicz 2,* 1 Department of Internal Medicine, Occupational Diseases and Hypertension, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] (H.M.); [email protected] (G.M.); [email protected] (R.P.); [email protected] (A.W.); [email protected] (M.M.) 2 Department of Experimental Dentistry, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] 3 Department and Clinic of Psychiatry, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] 4 Division of Respiratory Critical Care and Sleep Medicine, Department of Medicine, University of Saskatchewan, Saskatoon, SK S7N 5A2, Canada; [email protected] 5 Department of Periodontology, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] * Correspondence: [email protected]; Tel.: +48-660-47-87-59 Received: 3 August 2018; Accepted: 19 August 2018; Published: 23 August 2018 Abstract: Background: Sleep sex also known as sexsomnia or somnambulistic sexual behavior is proposed to be classified as NREM (non-rapid eye movement) parasomnia (as a clinical subtype of disorders of arousal from NREM sleep—primarily confusional arousals or less commonly sleepwalking), but it has also been described in relation to REM (rapid eye movement) parasomnias. Methods: The authors searched the PubMed database to identify relevant publications and present the co-occurrence of sexsomnia and other sleep disorders as a non-systematic review with case series. -
Circadian Rhythm Sleep Disorders and Narcolepsy
TALK FOR NARCOLEPSY NETWORK CONFERENCE 2013: Circadian Rhythm Sleep Disorders and Narcolepsy Note: The slides for this talk may be viewed at http://www.circadiansleepdisorders.org/docs/talks/NNconf2013talkSlides.pdf . Slides with audio of the talk are at http://youtu.be/i70SqjCr-jY . I. Introduction [title slide] A. Hello Hi. I’m Peter Mansbach, and I’m president of Circadian Sleep Disorders Network. I’m really glad for this opportunity to talk about circadian sleep disorders, and also about possible connections with narcolepsy. B. Disclaimer Let me start by saying I am not a medical doctor. I don’t diagnose, and I don’t treat. C. Why should the narcolepsy community care? [Overview slide] The various sleep disorders overlap. I have DSPS, but I have some of the same symptoms as narcolepsy. And many of you have symptoms of DSPS. Diagnoses are fuzzy too, and in some cases another sleep disorder may be secondary or even dominant. I’ll talk more about this later. D. Intro How many of you have trouble waking up in the morning? How many of you like to stay up late? II. Circadian Rhythm Sleep Disorders A. What are circadian rhythms? [slide] 1. General Circadian means "approximately a day". Circadian rhythms are processes in living organisms which cycle daily. They are produced internally in all living things. They are also referred to as the body clock. 2. In Humans Humans have internal cycles lasting on average about 24 hours and 10 minutes, though the length varies from person to person. (Early experiments seemed to show a cycle of about 25 hours, and this still gets quoted, but it is now known to be incorrect. -
A Comparison of Idiopathic Hypersomnia and Narcolepsy-Cataplexy Using Self Report Measures and Sleep Diary Data
57676ournal ofNeurology, Neurosurgery, and Psychiatry 1996;60:576-578 SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.60.5.576 on 1 May 1996. Downloaded from A comparison of idiopathic hypersomnia and narcolepsy-cataplexy using self report measures and sleep diary data Dorothy Bruck, J D Parkes Abstract Published data comparing patients with IH Eighteen patients with idiopathic hyper- and patients with NLS outside the sleep labo- somnia (IH) were compared with 50 ratory are scarce. Using self report question- patients with the narcoleptic syndrome of naires and sleep diary data our aim was to cataplexy and daytime sleepiness (NLS) determine the extent of group differences and using self report questionnaires and a which variables best discriminated between IH diary of sleep/wake patterns. The IH and NLS. group reported more consolidated noc- turnal sleep, a lower propensity to nap, greater refreshment after naps, and a Patients and methods greater improvement in excessive day- DIAGNOSTIC CRITERIA time sleepiness since onset than the NLS Idiopathic hypersomnia group. In IH, the onset of excessive day- Patients were selected with a complaint of time sleepiness was predominantly asso- excessive daytime sleepiness without cataplexy ciated with familial inheritance or a viral and with no evidence of any medical, psycho- illness. Two variables-number of logical, drug related, or respiratory disorder. reported awakenings during nocturnal All patients met diagnostic criteria ascertained sleep and the reported change in sleepi- from questionnaire responses: Epworth sleepi- ness since onset-provided maximum ness scale score8 > 13; duration of excessive discrimination between the IH and NLS daytime sleepiness > five years, profile of groups.