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Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

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Nội dung chi tiết: Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2shi KanbayashiIntroductionIn this chapter, the clinical and pathophysiological aspects of idiopathic and symptomatic narcolepsy cataplexy syndromes an

d hypersomnia (or excessive daytime sleepiness, EDS) are discussed. Although no systematic epidemiological study has been conducted, available data su Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ggest that hypersomnia (both idiopathic and symptomatic) is common but under-diagnosed; both types of hypersomnia significantly reduce the quality of

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

life (QOL) of the subjects. Narcolepsy-cataplexy type 1. narcolepsy without cataplexy (a prototypical hypersomnia) type 2. and idiopathic hypersomnia

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2clinical overlap among these disorders lias been noted, as each disorder is currently diagnosed by mostly sleep phenotypes and not by biologically.pat

hophysiologically based tests, similarly, symptomatic hypersomnia IS a heterogeneous disease entity and the biological pathophysiological mechanisms u Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

nderlying symptomatic hypersomnia are mostly unknown.Recent progress for understanding the pathophysiology of EDS particularly owes to the discovery o

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

f narcolepsy genes (i.e.. hypocretin receptor and peptide genes) in animals in 1999 and the subsequent discovery in 2000. of hypocretin ligand deficie

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2cted by cerebrospinal fluid (CSF) hypocretin-1 measures: low csr hypocrclin-l levels are seen in over 90% of narcolepsy-s. Nishiflo IÙL I ■ M. Sato -

M. MatsumuraStanford university sleep and Circadian Neurobiology Laboratory.Department of Psychiatry and Behavioral Sciences, StanfordUniversity Schoo Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

l of Medicine. 3165 Porter Drive. RM1195. PaloAlto. CA 94304, USAe-mail: nishinogstanford.eduT. KanbayashiDepartment of Neuropsychiatry. Akita Univers

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ity, Akita, Japans. Chftkroveny. M. Billiard (ed3.). sieep Medicine. DOI 10.1007/978-1-4939-2089-1_26.© Springer Science-Business Media, LLC 2015catap

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2CSF hypocretin-1 levels have been included in the third revision of the international classifications of sleep disorder as a positive diagnosis for na

rcolepsy-cataplexy [1].Narcolepsy-cataplexy is tightly associated with human leukocyte antigen (IILA) DQB 1*0602. Hypocretin deficiency in narcolepsy- Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

cataplexy is also tightly associated with HL A positivity, suggesting an involvement of immune-mediated mechanisms for the loss of hypocretin neurons.

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

However, the specificity of HLA positivity for narcolepsycataplexy is much lower than that of low CSF hypocretin-1 levels, as up to 30% of the genera

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2.005%. respectively, but the prevalence of symptomatic (secondary) hypersomnia may be much higher. For example, about several million subjects in the

USA suffer from chronic brain injury, and 75% of those people have sleep problems, and about half of them claim sleepiness [2], Symptomatic narcolepsy Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

has also been reported, but the prevalence of symptomatic narcolepsy is much smaller, and only about 120 cases have been reported m the literature in

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

the past 30 years [3], rhe meta-analysis of these symptomatic cases indicates lhal hypocrelin deficiency may also partially explain the neurobiologic

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2elin systems integrate and coordinate the multiple wake-promoting systems, such as monoamine and acetylcholine systems to keep subjects fully alert |4

|. suggesting that understanding of the roles of hypocretin peptidergic systems in sleep regulation in normal and pathological conditions is important Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

, as alternations of these systems may also be responsible not only for narcolepsy but also for other less well-defined hypersomnias.205206s. Nishino

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

et al.Since a large majority of patients with EDS are currently treated with pharmacological agents, new knowledge about the neurobiology of EDS will

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2s.This chapter focuses on pathophysiological mechanisms and nosological aspects of idiopathic and symptomatic hypersomnia. For the treatments of these

conditions, refer to more specific publications available [5-8].Symptoms of NarcolepsyExcessive Daytime SleepinessEDS and cataplexy are considered to Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

be the two primary symptoms of narcolepsy, with EDS often being the most disabling symptom. The EDS most typically mimics the feeling tliat people ex

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

perience when they are severely sleep-deprived but may also manifest Itself as a chronic tiredness or fatigue. Narcoleptic subjects generally experien

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2ften relieved by short naps (15-30 min), but in most cases the refreshed sensation only lasts a short time after awaking The refreshing value of short

naps is of considerable diagnostic value. Sleepiness also occurs in irresistible waves in these patients, a phenomenon best described as “sleep attac Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ks." Sleep attacks may occur in very unusual circumstances. such as in the middle of a meal, a conversation, or riding a bicycle. These attacks are of

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ten accompanied by microsleep episodes [9]. where the patient “blanks out.” The patient may then continue his or her activity in a semiconscious manne

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2s and impaired concentration are frequently associated [9-13]. but psychophysiological testing is generally normal.Sleepiness is usually the first sym

ptom to appear, followed by cataplexy, sleep paralysis, and hypnagogic hallucinations [14-IS], Cataplexy onset occurs within 5 years after the occurre Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

nce of daytime somnolence in approximately two-thirds of the cases (15, 17], Less frequently, cataplexy appears many years after the onset of sleepine

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ss. The mean age of onset of sleep paralysis and hypnagogic hallucinations is also 2-7 years later than that of sleepiness [14. 19],In most cases. EDS

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2tivities), daytime napping, and adjustment of nighttime sleep.CataplexyCataplexy is distinct from EDS and pathognomonic of the disease [20]. The impor

tance of cataplexy for the diagnosis of narcolepsy has been recognized since its description [21. 22] and in subsequent reviews on narcolepsy (23. 24] Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

, Most authors now recognize patients with recurring sleepiness and cataplectic attacks as a homogeneous clinical entity, and this is now shown to be

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

tightly associated with hypocretin deficiency (see the section on the pathophysiology of the disease). Cataplexy is defined as a sudden episode of mus

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2ll of the fishing rod with a biting fish, and the perfect hit at baseball), and less frequently by negative emotions (most typically anger or frustrat

ion). All antigravity muscles can be affected leading to a progressive collapse of the subject, but respiratory and eye muscles are not affected. The Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

patient is typically awake at the onset of the attack but may experience blurred vision or ptosis. The attack is almost always bilateral and usually l

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

asts a few seconds. Neurological examination performed at the time of an attack shows a suppression of the patellar reflex and sometimes presence of a

Part VIINeurological Sleep DisordersNarcolepsy-Cataplexy Syndrome and Symptomatic Hypersomnia26Seiji Nishino, Masatoshi Sato, Mari Matsumura and Takas

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2ropping, facial muscle flickering, sagging of the jaw. or w eakness in the arms. Slurred speech or mutism is also frequently associated. It is often i

mperceptible to the observer and may even be only a subjective feeling difficult to describe, such as a feeling of warmth or that somehow time is susp Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ended [24. 25]. In other cases, it escalates to actual episodes of muscle paralysis that may last up to a few minutes. Falls and injury are rare and m

Ebook Sleep medicine - A comprehensive guide to its development, clinical milestones and advances in treatment: Part 2

ost often the patient will have time to find support or will sit down while the attack IS occurring. Long episodes occasionally blend into sleep and m

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