What is Narcolepsy with cataplexy?
In Greek:
“narco” = somnolence, stupor, numbness or stiffness;
“lepsy” = fit or seizure
Therefore, “Narcolepsy”= “Fit of stupor or stiffness” or “seized by somnolence”
Narcolepsy is a chronic sleep disorder that affects the brain's ability to regulate the normal sleep-wake cycle. This can lead to symptoms such as disturbed night-time sleep and excessive sleepiness throughout the day. Narcolepsy is a chronic neurologic disorder, for which there is no known cure.
As narcolepsy is a relatively rare sleep disorder, it is known as an ‘orphan disease ’. The term orphan disease is used to describe diseases that affect only small numbers of individuals. Currently, there is no standard or generally accepted definition of an orphan disease, and differs by region. The ratio of affected to non-affected individuals that defines an orphan disease is adjusted for the population size of each country. The WHO (World Health Organization) has suggested a frequency of less than 6.5 to 10 in 10,000.
Narcolepsy is characterized by a tetrad of hallmark features:
- Excessive daytime sleepiness (EDS)
- Cataplexy
- Hypnagogic/hypnopompic hallucinations
- Sleep paralysis
Cataplexy is a sudden episode of bilateral muscle weakness triggered by the onset of strong emotions (e.g. laughter, anger, excitement, etc.). The “Clinical Presentation” section describes each of these features in greater detail.
According to The International Classification of Sleep Disorders (2nd Ed), there are 2 types of narcolepsy:
1. Narcolepsy with cataplexy
2. Narcolepsy without cataplexy
This module will focus on narcolepsy with cataplexy.
The following video is a patient's account of her experience in living with narcolepsy.
References
1. Nishino S. Clinical and neurobiological aspects of narcolepsy. Sleep Medicine 2007; 2: 373 – 399
2. Houghton WC, Scammell TE, Thorpy M. Pharmacotherapy for cataplexy. Sleep Medicine Reviews 2004; 8: 355-366
3. Ahmed I, Thorpy M. Clinical features, diagnosis and treatment of narcolepsy. Clin Chest Med 2010; 31: 371 – 381
4. Narcolepsy UK Website http://www.narcolepsy.org.uk/AboutNarcolepsy/Whatisnarcolepsy.aspx#.T7qoR3nOxc4
5. J K Aronson. Rare diseases and orphan drugs. Br J Clin Pharmacol. 2006 March; 61(3): 243–245.
Images:
http://bouldersleeptherapy.com/disorders/narcolepsy.php/
autoimmunediseasesa2z.com
www3.unil.ch
2. Houghton WC, Scammell TE, Thorpy M. Pharmacotherapy for cataplexy. Sleep Medicine Reviews 2004; 8: 355-366
3. Ahmed I, Thorpy M. Clinical features, diagnosis and treatment of narcolepsy. Clin Chest Med 2010; 31: 371 – 381
4. Narcolepsy UK Website http://www.narcolepsy.org.uk/AboutNarcolepsy/Whatisnarcolepsy.aspx#.T7qoR3nOxc4
5. J K Aronson. Rare diseases and orphan drugs. Br J Clin Pharmacol. 2006 March; 61(3): 243–245.
Images:
http://bouldersleeptherapy.com/disorders/narcolepsy.php/
autoimmunediseasesa2z.com
www3.unil.ch