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The general theory is that certain individuals are predisposed to an autoimmune attack of the hypocretin neurons that is likely initiated by environmental conditions ie, toxins, hormone changes, stress and may also be infective in nature. The autoimmune attack on hypocretin neurons results in irreversible loss of hypocretin and its wake-promoting and REM-modulating effects. Pathophysiology of patients without cataplexy is less certain, although similar changes may be involved. Narcoleptic patients without cataplexy may have normal CSF hypocretin concentrations.

It is unknown if this represents heterogeneous disease or incomplete loss of hypocretin neurons following autoimmune attack. Different stimuli can trigger cataplexy within an individual patient, but the most common triggers include surprise, anger, laughter, fear, stress, and excitement. During cateplectic attacks, patients partially lose skeletal muscle control, resulting in muscle weakness and inability to respond. Patients remain conscious of themselves and their surroundings during cataplectic spells that can last a few seconds to a minute or longer.

Diagnosis Narcolepsy is diagnosed using a combination of clinical symptoms and objective sleep testing. Symptoms of daytime sleepiness can be assessed subjectively using questionnaires such as the Epworth Sleepiness Scale.

Objective testing for narcolepsy includes an overnight polysomnographic study followed by a daytime multiple sleep latency test MSLT. The overnight sleep study is performed to rule out any other sleep pathologies eg, obstructive sleep apnea, periodic limb movements of sleep that could result in EDS. The following day, subjects are given the opportunity to sleep for 20 minutes every 2 hours for 4 to 5 nap periods. Sleep paralysis is a frightening phenomenon in which an individual awakens and cannot move or respond to the environment.

Sleep paralysis may also occur in individuals without narcolepsy, so by itself, is not diagnostic. Hallucinations also occur at the sleep-wake transition either waking [hypnopompic] or falling asleep [hypnagogic] and may be an extremely vivid auditory or visual experiences.

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Lastly, individuals with narcolepsy may have disrupted nighttime sleep with frequent awakenings and a short REM sleep latency. Clinical and Social Burden of Narcolepsy Morbidity and mortality. Narcolepsy has a significantly negative effect on quality of life, and narcoleptic patients also experience increased morbidity and mortality. A factual analysis of morbidity and mortality was performed using the Danish National Patient Registry before and after a narcolepsy diagnosis. Authors found a trend hazard ratio 0.

Collectively, data suggest that narcolepsy is associated with increased mortality risk. Associated comorbidities. Studies have shown that narcolepsy is associated with increased risk of psychiatric and other medical conditions. Individuals with narcolepsy are more likely to have hypercholesterolemia, digestive diseases, heart disease, upper respiratory tract diseases, and hypertension. Narcolepsy is also associated with impairments in social functioning and health-related quality of life.

The clinical symptoms of narcolepsy seem likely to influence earning potential and healthcare expenses. Early analysis of the economic burden faced by narcoleptics found that total annual costs for narcolepsy were high, and contributed significantly to early retirement and unemployment. Compared with control subjects, patients with narcolepsy had higher health contact rates, medication use, expenses, and unemployment rates. Annual total direct and indirect costs for narcoleptics were almost 10 times higher than for control subjects.

Thus, individuals with narcolepsy suffer significant economic consequences, earning less money, having higher unemployment, and having greater health-related expenses. Healthcare utilization.


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Without a doubt, individuals with narcolepsy have significant healthcare utilization and economic costs. Narcoleptics had 2-fold higher annual rates of inpatient admissions, emergency department visits, hospital outpatient visits, other outpatient visits, and physician visits. Analysis of the Danish National Patient Registry also showed excess sum of direct and indirect healthcare costs for individuals with narcolepsy versus control subjects.

Management of Narcolepsy The treatment goals of narcolepsy are: 1 reduce EDS; 2 reduce frequency and severity of cataplexy, sleep paralysis, and hallucinations; and 3 improve and consolidate nighttime sleep disruption. Both pharmacologic and nonpharmacologic therapies are used to optimize care of patients and reduce disease burden. Nonpharmacologic therapy includes implementation of appropriate sleep hygiene and strategic nap opportunities during the day. On occasion, workplace or school accommodations may also be necessary to provide flexibility for the patient to be productive and functional.

Treatment of excessive daytime sleepiness.

The cornerstone of therapy for EDS includes traditional central nervous system stimulants methylphenidate and amphetamines and the wake-promoting agents modafinil and armodafinil. However, compared with modafinil, these agents do not have as much high-quality evidence supporting their use. Pemoline was used in the early days of narcolepsy treatment, but is associated with severe liver toxicity and is no longer available in the United States. Armodafinil is the active R -isomer, whereas modafinil is a racemic mixture of L - and R -modafinil.

Armodafinil was not available when the AASM Practice parameters were published, but has shown efficacy similar to modafinil in randomized controlled trials. Interestingly, however, the comparative effectiveness of available therapies has been estimated using combined MSLT and maintenance of wakefulness MWT data from various studies.

Mittler and Hajdukovic estimate the percent of normal wakefulness obtained during therapy with a variety of agents. Modafinil and armodafinil have been extensively investigated for the treatment of narcolepsy, and consistently reduce daytime sleepiness in randomized controlled trials. See more. James R. Doty, MD. But back then his life was at a dead end until at twelve he wandered into a magic shop looking for a plastic thumb.

Instead he met Ruth, a woman who taught him a series of exercises to ease his own suffering and manifest his greatest desires. Her final mandate was that he keep his heart open and teach these techniques to others.

Clearing The Fog Of Narcolepsy

She gave him his first glimpse of the unique relationship between the brain and the heart. Part memoir, part science, part inspiration, and part practical instruction, Into the Magic Shop shows us how we can fundamentally change our lives by first changing our brains and our hearts. Joshua Foer.

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Based on Dr. Michio Kaku. The Future of the Mind brings a topic that once belonged solely to the province of science fiction into a startling new reality. Dewey: Narcolepsy : Pathophysiology, Diagnosis, and Treatment.

Narcolepsy: pathophysiology and pharmacology.

New York: Springer, APA: Bassetti, C. L, Baumann, C. Narcolepsy : pathophysiology, diagnosis, and treatment. New York: Springer. Chicago: Bassetti, Claudio L. Narcolepsy: Pathophysiology, Diagnosis, and Treatment. AU - Baumann, Christian R. AU - Scammell, Thomas E. N 4 a I 1 4 a WL a